1

Principles of Cardiology

Samir M. Rafla, MD, FESC

Professor of Cardiology

Alexandria University

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Title: Principles of Cardiology. 2008

Author: Prof. Samir Rafla, MD, FACC, FESC

Head of the Cardiology Dept. Alexandria Univ. from 1 Aug 2004 to 30 Aug 2007

MBChB (Honor) June 1970

Printed by: Delta Center for Printing: 24 Delta street, Sporting, T. 03 5901923

Computer work: Mr. Haytham Abdel-Moneim

Distributed by: El-Sherok Library. T 03 484 8673

Address for correspondence: Prof. Samir Rafla

smrafla@hotmail.com 0101495577 03 5910170

First edition: 2008

Cover drawing by Dr. Marilyn Samir

National Number

2008/16131

Previous books by the author:

- Differential Diagnosis in Clinical Medicine. Vol. one: Heart and Chest. 1998

- Recent Advances in Diagnosis and Management of Arrhythmias. 2000

- Alexandria Book of Cardiology. Co-author with the staff of the cardiology dept.

Alexandria Univ., and co-editor with Prof. Tarek El-Badawy. 2004

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CONTENTS

Subject Page

Electrocardiography 1

Rheumatic Fever 3

Valvular Heart Disease

Mitral stenosis

The cardiac cycle

Mitral Regurgitation

Mitral valve prolapse

Aortic stenosis

Aortic Regurgitation

Tricuspid Stenosis

Tricuspid Regurgitation

6

7

13

15

16

19

21

22

Congenital Heart Disease

Atrial septal defect

Ventricular septal defect

Patent ductus arteriosus

Fallot’s tetralogy

Coarctation of the aorta

Pulmonary stenosis

24

25

27

28

30

33

34

Syncope and hypotension

Sudden cardiac death

36

39

Cardiac Arrhythmias Sick Sinus Syndrome

Premature beats (Extrasystoles)

Supraventricular Tachyarrhythmias

Atrial flutter

AV nodal reentrant tachycardia

Wolff-Parkinson-White syndrome

Atrial fibrillation

Treatment of Atrial Fibrillation Anticoagulation for Atrial Fibrillation

Ventricular tachycardia

Long QT syndrome

Implantable Cardioverter Defibrillator (ICD)

AV heart block

Electrophysiologic study Cardiac pacemakers

Antiarrhythmic drugs

41

42

43

44

44

46

47

48

51

53

54

55

56

56

58

59

60

Heart Failure

Right heart failure

Management

Pharmacological therapy

Ventricular Resynchronization Therapy

Acute left ventricular failure

Cardiogenic shock

62

67

69

70

74

75

77

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Refractory heart failure 80

Infective Endocarditis

Treatment

81

86

Ischemic Heart Disease

Risk factors for atherosclerosis

Stable angina pectoris

Chest pain algorithm

Unstable angina and NSTEMI

Myocardial infarction

Shock algorithm

Treatment of STEMI

Anticoagulants

87

88

89

92

95

97

103

106

110

Hypertension

Phaeochromocytoma

Investigation of the Hypertensive Patient

Treatment

Hypertensive emergencies

112

117

119

123

128

Aortic aneurysm and aortic dissection

Aortic dissection

Aortic dissection classification

129

129

132

Diseases of the peripheral arteries and veins

Deep vein thrombosis

134

135

The Lungs and Pulmonary Circulation

Pulmonary Embolism

Pulmonary Hypertension

Pulmonary Hypertension algorithm

Primary pulmonary hypertension

Cor pulmonale

Schistosomal corpulmonale

137

137

143

144

146

147

148

Diseases of The Pericardium

Acute pericarditis

Pericardial effusion

Pericardial tamponade

Constrictive pericarditis

149

149

151

152

152

Cardiomyopathy and myocarditis

Cardiomyopathy

Dilated cardiomyopathy

Hypertrophic cardiomyopathy

Restrictive cardiomyopathy

Cardiac tumors

154

155

156

156

158

158

Question of the medical rounds 159

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Principles of Cardiology pages 1-61

ELECTROCARDIOGRAPHY

Prof. Samir Rafla

The electrocardiogram (ECG) is a graphic representation of the electrical activity generated by the

heart during the cardiac cycle. The electrical activity starts from the SA node, bundle of His, right and

left bundles, Purkinje fibers to stimulate the ventricles.

Waveforms: The waveforms and intervals of the ECG are: The P wave = atrial depolarization. The

QRS complex = ventricular depolarization. The Q wave is the initial downward deflection, the R wave

is the initial upward deflection, and the S wave is the second downward deflection. The interval from

the beginning of the P wave to the beginning of the Q wave is the PR interval.

The T wave = ventricular repolarization. The interval from the end of ventricular depolarization to the

beginning of the T wave is termed the ST segment. The interval from the onset of ventricular

depolarization to end of T is the QT interval.

STANDARD APPROACH TO THE ECG: Normally, standardization is 1.0 mV per 10 mm, and

paper speed is 25 mm/s (each horizontal small box = 0.04 sec)

Heart Rate: divide 1500 by number of small boxes between each QRS.

Rhythm: Sinus rhythm is present if every P wave is followed by a QRS, PR interval > 0.12 s, and the P

wave is upright in leads I, II, and III.

Intervals: PR (0.12 - 0.20 s). QRS (0.06 - 0.10 s).

QT 0.43 s;

ST-T WAVES: ST elevation : Acute MI, coronary spasm, pericarditis (concave upward), LV

aneurysm.

ST depression: Digitalis effect, strain (due to ventricular hypertrophy), ischemia, or nontransmural MI.

Tall peaked T: Hyperkalemia; acute MI ("hyperacute T").

Inverted T: Non-Q-wave MI, ventricular "strain" pattern, drug effect (e.g., digitalis), hypokalemia,

hypocalcemia, increased intracranial pressure (e.g., subarachnoid bleeding).

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FIG: The magnified ECG wave is presented with the principal time intervals indicated.

Fig: The pathways of Conduction.

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RHEUMATIC FEVER

Introduction. Classified as a connective tissue or collagen vascular disease, rheumatic fever (RF) is

the leading cause of acquired heart disease in children and young adults.

a. In many developing countries the incidence of acute RF approaches or exceeds 100 per 100.000,

whereas in the Unites States it is estimated to be less than 2 per 100.000.

b. Rheumatic fever is more common among population at high risk for streptococcal pharyngitis, those

in close contact with school age children, and persons of low socioeconomic status. It occurs

commonly between the ages of 5 and 18 years and is rare before 5. Rheumatic fever affects both sexes

equally, except for Sydenham’s chorea, which is more prevalent in females after puberty.

The clinical manifestations of RF develop after a silent period of approximately 3 weeks following a

tonsillopharyngitis caused by a group A streptococcal infection (GAS).

Diagnostic criteria

1. The Jones criteria, are designed to aid in the diagnosis of the first episode of RF. Rheumatic

fever can be diagnosed when a previous upper airway infection with GA-Streptococci is detected in

conjunction with either two major manifestations, or one major and two minor manifestations. Major

manifestation includes arthritis, carditis, chorea, erythema marginatum, and subcutaneous nodules.

Minor manifestations include: fever, arthralgias, history of tonsillitis 1-3 weeks before the arthralgia,

history of rheumatic heart disease;

high C-reactive protein, high erythrocyte sedimentation rate, raised antistreptolysin O titer above 200

Todd’s units or prolonged PR interval on electrocardiogram (ECG).

Major manifestations:

1. Carditis: affecting 41% to 83% of patients. It can be defined as pancarditis affecting the

endocardium, myocardium, and pericardium: The main clinical manifestations include increased heart

rate, murmurs, cardiomegaly, rhythm disturbances, pericardial friction rub, and heart failure.

Congestive heart failure is rare in the acute phase; if present, it usually results from myocarditis. The

most characteristic component of rheumatic carditis is a valvulitis (endocarditis) involving the mitral

and aortic valves.

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Pericarditis may cause chest pain, friction rubs, and distant heart sounds.

2. Arthritis. This is the most common manifestation of RF. It is present in around 80% of the patients

and has been described as painful, asymmetric, migratory, and transient; it involves large joints, such as

knees, ankles, elbows, wrists, and shoulders. It improves markedly with the use of salicylates within 48

hours of treatment. Monoarthritis, oligoarthritis, and involvement of small joints of the extremities are

less common. The arthritis of RF is benign and self- limiting (Lasting 2 to 3 weeks) and does not result

in permanent sequelae.

3. Sydenham’s chorea. This extrapyramidal disorder is characterized by purposeless and involuntary

movements of face and limbs, muscular hypotonia, and emotional lability.

4. Subcutaneous nodules.

5. Erythema marginatum.

Minor manifestations:

1. Fever is encountered during the acute phase of the disease.

2. Arthralgia is defined as pain in one or more large joints without objective findings of inflammation

on physical examination.

3. Other clinical manifestations of RF include abdominal pain, epistaxis, acute glomerulonephritis.

These are not included as diagnostic criteria for the diagnosis of RF.

Laboratory examination and diagnostic testing.

1. Neither throat culture nor rapid antigen test, if positive; differentiate

between recent infection associated with RF and chronic carriage of

pharyngeal GAS.

2. Antistreptolysin O is the most commonly available test. Elevated or rising ASO titers provide solid

evidence for recent GAS infection. A greater than two-fold rise in ASO titers compared with

convalescent titers is diagnostic.

3. Increased sedimentation rate.

4. Increased C reactive protein CRP/

5. The most common finding in the electrocardiogram is the presence of P-R prolongation and sinus

tachycardia.

Therapy:

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Patient with the diagnosis of rheumatic activity should initially receive a full course of antibiotic to

ensure proper eradication of the organism.

A. Arthritis: Anti-inflammatory medications are generally recommended for 3 weeks for symptomatic

relief.

1. Pain resolves within 24 hours of starting therapy with salicylates.

2. If pain persists after salicylate treatment, the diagnosis of RF is questionable.

3. The recommended dose of salicylate is 100 mg/kg per day, given in 4 divided doses. Toxic effects

such as anorexia, nausea, vomiting, and tinnitus should be avoided.

B. Carditis

1. Strenuous physical activity should be avoided.

2. Congestive heart failure should be treated with appropriate therapy.

3. In patients with significant cardiac involvement, corticosteroids are preferred over salicylates. The

recommended dose is 1 to 2 mg/kg per day, (maximum of 60 mg/day as Prednisolone). Commonly,

therapy is needed for more than one month in patients with cardiac involvement. Therapy should be

continued until there is sufficient clinical and laboratory evidence of disease inactivity.

4. The gradual reduction in steroid doses is important to avoid relapses. Use of salicylates (75 mg/kg

per day) while tapering corticosteroids may reduce the likelihood a relapse.

Summary: Jones Criteria of Rheumatic Fever

Major Criteria Minor Criteria

Migratory polyarthritis Fever

Carditis Arthralgia

Chorea High sedimentation rate

Subcutaneous nodules Positive C reactive protein

Erythema Marginatum Prolonged PR interval

Prevention:

The most important step in the treatment of RF is the eradication of GAS infection.

Penicillin is the agent of choice. A. best results are achieved with a single intramuscular dose of

penicillin G benzathine. b. The oral antibiotic of choice is penicillin V (phenoxymethyl penicillin) (see

Table for dosage information). Patients allergic to penicillin: oral erythromycin can be used. The

recommended dosage is erythromycin for 10 days. The maximal dose of erythromycin is 1 g/day.

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Table: Duration of therapy for secondary prevention of rheumatic fever

Disease state Duration of therapy

RF + carditis + residual valvular

disease

At least 10 years post episode and at least

until age 40. Lifelong prophylaxis may be

required

RF + carditis without valvular

disease

10 years or beyond adulthood, whichever

is longer.

RF without carditis 5 years or until age of 21, whichever is

longer.

RF, rheumatic fever.

VALVULAR HEART DISEASE

MITRAL STENOSIS

ETIOLOGY AND PATHOLOGY: Two-thirds of all patients with mitral stenosis (MS) are females.

MS is generally rheumatic in origin. Pure or predominant MS occurs in approximately 40% of all

patients with rheumatic heart disease. The valve leaflets are diffusely thickened by fibrous tissue and/or

calcific deposits. The mitral commissures fuse, the chordae tendineae fuse and shorten. The valvular

cusps become rigid, and these changes in turn, lead to narrowing at the apex of the funnel-shaped

valve.

Other rare causes of mitral stenosis: Atrial myxoma, ball valve thrombus, congenital and calcific-

atherosclerortic disease.

PATHOPHYSIOLOGY: In normal adults the mitral valve orifice is 4 to 6 cm2. When the mitral valve

opening is reduced to 1 cm2, a left atrial pressure of approximately 25 mmHg is required to maintain a

normal cardiac output. The elevated left atrial pressure, in turn, raises pulmonary venous and capillary

pressures, reducing pulmonary compliance and causing exertional dyspnea.

Pulmonary hypertension results from (1) the passive backward transmission of the elevated left atrial

pressure, (2) pulmonary arteriolar constriction, (reactive pulmonary hypertension), and (3) organic

obliterative changes in the pulmonary vascular bed. In time, the resultant severe pulmonary

hypertension results in tricuspid and pulmonary incompetence as well as right-sided heart failure.

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SYMPTOMS AND COMPLICATIONS: - Dyspnea, hemoptysis. - Orthopnea and paroxysmal

nocturnal dyspnea. Pulmonary edema develops when there is a sudden surge in flow across a markedly

narrowed mitral orifice.

The cardiac cycle: Simultaneous electrocardiogram and pressure obtained from the left atrium, left

ventricle, and aorta, and the jugular pulse during one cardiac cycle.

When moderately severe MS has existed for several years, atrial arrhythmias as flutter and fibrillation

occur.

Hemoptysis results from rupture of pulmonary-bronchial venous connections (apoplexy) secondary to

pulmonary venous hypertension. Frank hemoptysis must be distinguished from the bloody sputum that

occurs with pulmonary edema, pulmonary infarction, and bronchitis, three conditions that occur with

increased frequency in the presence of MS.

Recurrent pulmonary emboli, sometimes with infarction are an important cause of morbidity and

mortality late in the course of MS, occurring most frequently in patients with right ventricular failure.

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Pulmonary infections, i.e., bronchitis, broncho-pneumonia, and lobar pneumonia, commonly

complicate untreated MS. Infective endocarditis is rare in pure MS but is not uncommon in patients

with combined stenosis and regurgitation.

Summary: Causes of hemoptysis in mitral stenosis:

- Bronchitis

- Congestion

- Pulmonary edema

- Pulmonary embolism, infarction

- Pulmonary apoplexy

Thrombi and emboli: Thrombi may form in the left atrium, particularly in the enlarged atrial

appendage of patients with MS. If they embolize, they do so most commonly to the brain, kidneys,

spleen, and extremities. Embolization occurs much more frequently in patients with atrial fibrillation.

Rarely, a large pedunculated thrombus or a free-floating clot may suddenly obstruct the stenotic mitral

orifice. Such “ball valve” thrombi produce syncope, angina, and changing auscultatory signs with

alterations in position, findings that resemble those produced by a left atrial myxoma.

PHYSICAL FINDINGS: Inspection: In advanced cases there is a malar flush. When fibrillation is

present, the jugular pulse reveals only a single expansion during systole (c-v wave) (systolic venous

pulse).

Palpation: Left parasternal lift along the left sternal border signifies an enlarged right ventricle. In

patients with pulmonary hypertension, the impact of pulmonary valve closure can usually be felt in the

second and third left intercostal spaces just left of the sternum (Diastolic shock). A diastolic thrill is

frequently present at the cardiac apex, particularly if the patient is turned into the left lateral position.

Auscultation: The first heart sound (S1) is generally accentuated and snapping. In patients with

pulmonary hypertension, the pulmonary component of the second heart sound (P2) is often accentuated,

and the two components of the second heart sound are closely split. The opening snap (OS) of the

mitral valve is most readily audible in expiration at, or just medial to, the cardiac apex but also may be

easily heard along the left sternal edge. This sound generally follows the sound of aortic valve closure

(A2) by 0.05 to 0.12; that is, it follows P2; the time interval between A2 closure and OS varies inversely

with the severity of the MS. It tends to be short (0.05 to 0.07 s) in patients with severe obstruction, and

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long, (0.10 to 0.12 s) in patients with mild MS. The intensities of the OS and S1 correlate with mobility

of the anterior mitral leaflet.

The OS usually precedes a low-pitched, rumbling, diastolic murmur, heard best at the apex with the

patient in the left lateral recumbent position. In general, the duration of the murmur correlates with the

severity of the stenosis. In patients with sinus rhythm, murmur often reappears or becomes accentuated

during atrial systole, as atrial contraction elevates the rate of blood flow across the narrowed orifice

(presystolic accentuation).

Associated lesion: With severe pulmonary hypertension, a pansystolic murmur produced by

functional tricuspid regurgitation may be audible along the left sternal border. Characteristically, this

murmur is accentuated by inspiration, and should not be confused with the apical pansystolic murmur

of mitral regurgitation.

In the presence of severe pulmonary hypertension and right ventricular failure, a third heart sound

may originate from the right ventricle. The enlarged right ventricle may rotate the heart in a clockwise

direction and form the cardiac apex, giving the examiner the erroneous impression of left ventricular

enlargement. Under these circumstances, the rumbling diastolic murmur and the other auscultatory

features of MS become less prominent or may even disappear and be replaced by the systolic murmur

of functional tricuspid regurgitation which is mistaken for mitral regurgitation. When cardiac output is

markedly reduced in a patient with MS, the typical auscultatory findings, including the diastolic

rumbling murmur, may not be detectable (silent MS).

ECG findings: The P wave is wide and may be notched which suggests left atrial enlargement. It

becomes tall and peaked in lead II and upright in lead V1 when severe pulmonary hypertension.

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Echocardiogram: Two-dimensional echo-Doppler echocardiography for estimation of the

transvalvular gradient and of mitral orifice size, the presence and severity of accompanying mitral

regurgitation, the extent of restriction of valve leaflets, their thickness, and the subvalvular changes.

Transthoracic and transesophageal echo are needed to verify presence of atrial thrombi.

X-Ray chest: Straightening of the left border of the cardiac silhouette, prominence of the main

pulmonary arteries, dilatation of the upper lobe pulmonary veins, and backward displacement of the

esophagus by an enlarged left atrium.

Summary of signs of mitral stenosis:

- Mid-diastolic rumbling murmur with presystolic accentuation;

- Snappy first sound;

- Opening snap;

- Diastolic thrill.

DIFFERENTIAL DIAGNOSIS: The apical middiastolic murmur associated with aortic regurgitation

(Austin Flint murmur) may be mistaken for MS. However, in a patient with aortic regurgitation, the

absence of an opening snap or presystolic accentuation if sinus rhythm is present points to the absence

of MS.

Tricuspid stenosis, a valvular lesion that occurs very rarely in the absence of MS, may mask many of

the clinical features of MS.

MANAGEMENT: Penicillin prophylaxis of beta-hemolytic streptococcal infections and prophylaxis

for infective endocarditis are important. In symptomatic patients, some improvement usually occurs

with restriction of sodium intake and maintenance doses of oral diuretics. Digitalis glycosides usually

do not benefit patients with pure stenosis and sinus rhythm, but they are necessary for slowing the

ventricular rate of patients with atrial fibrillation and for reducing the manifestations of right-sided

heart failure in the advanced stages of the disease.

Small doses of beta-blockers (e.g., atenolol 25 mg/d) may be added when cardiac glycosides fail to

control ventricular rate in patients with atrial fibrillation. Particular attention should be directed toward

detecting and treating any accompanying anemia and infections. Hemoptysis is treated by measures

designed to diminish pulmonary venous pressure, including bed rest, the sitting position, salt

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restriction, and diuresis. Anticoagulants should be administered continuously in those with atrial

fibrillation.

If atrial fibrillation is of relatively recent origin in a patient who’s MS is not severe enough to warrant

surgical treatment, reversion to sinus rhythm pharmacologically or by means of electrical countershock

is indicated. Usually this should be undertaken following 3 weeks of anticoagulant treatment.

Conversion to sinus rhythm is rarely helpful in patients with severe MS, particularly those in whom the

left atrium is especially enlarged or in whom atrial fibrillation is chronic.

Mitral valvotomy by balloon or surgical mitral valvotomy, is indicated in the symptomatic patient with

pure MS whose effective orifice is less than approximately 1.3 cm2 (or 0.8 cm

2 / m

2 of body surface

area). Mitral valve replacement by prosthetic valve is resorted to only if the valve is heavily calcified

and associated with incompetence.

Percutaneous balloon valvuloplasty is an alternative to surgical mitral valvuloplasty in patients with

pure or predominant rheumatic stenosis (it is now the first choice). Young patients without extensive

valvular calcification or thickening or subvalvular deformity are the best candidates for this procedure.

Contraindications of balloon mitral valvotomy:

1. presence of left atrial thrombi,

2. presence of combined mitral incompetence and stenosis, and

3. heavily calcified mitral cusps.

MITRAL REGURGITATION

ETIOLOGY:

1- Chronic rheumatic heart disease is the cause of severe mitral regurgitation (MR).

2- MR also may occur as a congenital anomaly.

3- MR may occur in patients with infarction involving the base of a papillary muscle.

4- MR may occur with marked left ventricular dilatation.

5- Massive calcification of the mitral annulus of unknown cause, presumably degenerative, which

occurs most commonly in elderly women.

6- Systemic lupus erythematosus, rheumatoid arthritis, are less common cause.

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7- Mitral prolapse.

Acute MR occur 1- secondary to infective endocarditis involving the cusps or chordae tendineae, 2- in

acute myocardial infarction with rupture of a papillary muscle or one of its heads, 3- as a consequence

of trauma, 4- or following apparently spontaneous chordal rupture.

MITRAL REGURGITATION: SYMPTOMS: Fatigue, exertional dyspnea, and orthopnea are the

most prominent complaints in patients with chronic, severe MR. Hemoptysis and systemic

embolism also occur less frequently in MR than in MS. Right-sided heart failure, with painful

hepatic congestion, ankle edema, distended neck veins, ascites, and tricuspid regurgitation,

may be observed in patients with MR who have associated pulmonary vascular disease and

marked pulmonary hypertension. In patients with acute, severe MR, left ventricular failure

with acute pulmonary edema and /or cardiovascular collapse is common.

PHYSICAL FINDINGS: Palpation: A systolic thrill is often palpable at the cardiac apex, the left

ventricle is hyperdynamic, and the apex beat is often displaced laterally. Auscultation: The first heart

sound is generally absent, soft (muffled), or buried in the systolic murmur. A low-pitched third heart

sound (S3) occurring 0.12 to 0.17 sec after aortic valve closure, i.e. at the completion of the rapid-filling

phase of the left ventricle, is an important auscultatory feature of severe MR.

A fourth heart sound is often audible in patients with acute, severe MR of recent onset who are in sinus

rhythm. A systolic murmur of at least grade III/VI intensity is the most characteristic auscultatory

finding in severe MR. It is usually holosystolic (pansystolic). In MR due to papillary muscle

dysfunction or mitral valve prolapse, the systolic murmur commences in midsystole. In patients with

ruptured chordae tendineae the systolic murmur may have a cooing or “sea gull” quality; in patients

with a flail leaflet the murmur may have a musical quality.

Summary: Signs of mitral incompetence:

- Harsh pansystolic murmur over apex propagated to axilla.

- Muffled first heart sound.

- Systolic thrill over apex.

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Electrocardiogram: In patients with sinus rhythm there is evidence of left atrial enlargement (P

mitrale), but right atrial enlargement also may be present when pulmonary hypertension is severe.

Chronic, severe MR with left atrial enlargement is generally associated with atrial fibrillation.

Echocardiogram: Doppler echocardiography and color Doppler flow echocardiography imaging are the

most accurate noninvasive techniques for the detection and estimation of MR. The left atrium is usually

enlarged. Findings which help to determine the etiology of MR can often be identified; these include

vegetations associated with infective endocarditis, incomplete coaptation of the anterior and posterior

mitral leaflets, and annular calcification, as well as left ventricular dilation, aneurysm, or dyskinesia.

The echocardiogram in patients with mitral valve prolapse is described below.

Roentgenogram: The left atrium and left ventricle are the dominant chambers; in chronic cases, the

former may be massively enlarged and forms the right border of the cardiac silhouette. Pulmonary

venous congestion, interstitial edema, and Kerly B lines are sometimes noted.

TREATMENT: Medical: The non surgical management of MR is directed toward restricting those

physical activities that regularly produce dyspnea and excessive fatigue, reducing sodium intake, and

enhancing sodium excretion with the appropriate use of diuretics. Vasodilators and digitalis glycosides

increase the forward output of the failing left ventricle. Angiotensin-converting enzyme inhibitors are

given in chronic MR. The same considerations as in patients with MS apply to the reversion of atrial

fibrillation to sinus rhythm. Surgical treatment should be offered to patients with severe MR whose

limitations do not allow them to perform normal household activities despite optimal medical

management. Surgery is indicated when the end systolic diameter of the left ventricle by echo exceeds

50 mm.

MITRAL VALVE PROLAPSE

Mitral valve prolapse (MVP), also termed the systolic click-murmur syndrome, is a common, but

highly variable, clinical syndrome. It is a frequent finding in patients who have the typical features of

the Marfan syndrome. The posterior leaflet is usually more affected than the anterior, and the mitral

valve annulus is often greatly dilated.

MVP may be associated with thoracic skeletal deformities.

MVP is common in females between the ages of 6 and 30 years. Most patients are asymptomatic and

remain so for their entire lives. Arrhythmia, most commonly ventricular premature contractions and

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paroxysmal supraventricular and ventricular tachycardia, have been reported and may cause

palpitations, light-headedness, and syncope. Many patients have chest pain which is difficult to

evaluate.

PHYSICAL EXAMINATION: Auscultation: the most important finding is the mid-or late

(nonejection) systolic click, which occurs 0.14 s or more after the first heart sound. Systolic clicks may

be followed by a high-pitched late systolic murmur, heard best at the apex. A useful echocardiographic

definition of MVP is systolic displacement (in the parasternal view) of the mitral valve leaflets into the

left atrium > 3 mm. Thickening of the mitral valve leaflets is present. Doppler studies are helpful in

revealing and evaluating accompanying MR.

Treatment: The management of patients with MVP consists of reassurance of the asymptomatic

patient without severe MR or arrhythmias; prevention of infective endocarditis with antibiotic

prophylaxis in patients with a systolic murmur and the relief of the atypical chest pain by beta blockers.

AORTIC STENOSIS

Aortic stenosis (AS) occurs in one-fourth of all patients with chronic valvular heart disease;

approximately 80 percent of adult patients with symptomatic valvular AS are male.

Etiology: 1. AS may be congenital in origin, 2. secondary to rheumatic inflammation of the valve, 3.

degenerative calcification of the aortic cusps of unknown cause.

PATHOPHYSIOLOGY: A peak systolic pressure gradient exceeding 50 mmHg or an effective aortic

orifice less than approximately 0.5 cm2/m

2 of body surface area i.e., less than approximately one-third

of the normal orifice, is generally considered to represent critical obstruction to left ventricular outflow.

SYMPTOMS: AS is rarely of hemodynamic or clinical importance until the valve orifice has narrowed

to approximately one-third of normal, i.e., to 1 cm2 in adults.

Exertional dyspnea, angina pectoris, and syncope are the three cardinal symptoms. Angina pectoris

reflects an imbalance between the augmented myocardial oxygen requirement by the hypertrophied

myocardium and the un-accompanying increase in coronary blood flow. Orthopnea, paroxysmal

nocturnal dyspnea, and pulmonary edema, i.e., symptoms of left ventricular failure, also occur only in

the advanced stages of the disease.

PHYSICAL FINDINGS: A palpable double systolic arterial pulse the so-called bisferiens pulse,

excludes pure or predominant AS and signifies dominant or pure aortic regurgitation or obstructive

hypertrophic cardiomyopathy.

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Palpation: The apex beat is usually sustained and displaced laterally, reflecting the presence of left

ventricular hypertrophy. A systolic thrill is generally present at the base of the heart in the suprasternal

notch, and along the carotid arteries.

Auscultation: Harsh ejection systolic murmur over aortic area propagated to carotids. The sound of

aortic valve closure, the second sound is very weak or even absent with tight aortic stenosis.

Frequently, a fourth heart sound is audible at the apex in many patients with severe AS and reflects

the presence of left ventricular hypertrophy and an elevated left ventricular enddiastolic pressure; a

third heart sound generally occurs when the left ventricle dilates and fails.

The murmur of AS is characteristically an ejection systolic murmur loudest at the base of the

heart, most commonly in the second right intercostal space. It is transmitted along the carotid arteries.

Occasionally, it is transmitted downward and to the apex and may be confused with the systolic

murmur of MR.

Summary: Signs of aortic stenosis:

1. Harsh ejection systolic murmur over aortic area propagated to carotids.

2. Weak or absent second heart sound (aortic component)

3. Systolic thrill over aortic area, suprasternal notch and carotids.

4. Strong sustained apex,

Electrocardiogram: This reveals left ventricular hypertrophy in the majority of patients with severs

AS.

Echocardiogram: The key findings are left ventricular hypertrophy. The transaortic valvular gradient

can be estimated by Doppler echocardiography.

Congestive heart failure was considered to be the cause of death in one-half to two-thirds of patients.

Among adults dying with valvular AS sudden death, which presumably results from an arrhythmia

(ventricular tachycardia or fibrillation) occurred in 10 to 20 percent and at an average age of 60 years.

TREATMENT: All patients with moderate or severe AS require careful periodic follow-up. In

patients with severe AS, strenuous physical activity should be avoided even in the asymptomatic stage.

Digitalis glycosides, sodium restriction, and the cautious administration of diuretics are indicated in the

treatment of congestive heart failure, but care must be taken to avoid volume depletion.

In the majority of adults with calcific AS and critical obstruction, replacement of the valve is necessary.

Percutaneous balloon aortic valvuloplasty is an alternative to surgery in children and young adults with

21

congenital aortic stenosis. It is not commonly employed in elderly with severe calcific aortic stenosis

because of a high restenosis rate.

Electrocardiogram (ECG), left ventricular, and aortic pressure curves in a patient with aortic stenosis.

There is a pressure gradient across the aortic valve during systole

22

Fig. Abnormal sounds and murmurs associated with valvular dysfunction displayed simultaneously

with left atrial (LA), left ventricular (LV), and aortic pressure tracings. AVO, aortic valve opening; E,

ejection click; MVO, mitral valve opening; OS, opening snap of the mitral valve.

.

AORTIC REGURGITATION

ETIOLOGY: Approximately three-fourths of patients with pure or predominant aortic regurgitation

(AR) are males; females predominate among patients with AR who have associated mitral valve

disease.

Causes:

1- In approximately two-thirds of patients with AR the disease is rheumatic in origin, resulting in

thickening, deformation and shortening of the individual aortic valve cusps, changes which prevent

their proper opening during systole and closure during diastole.

2- Acute AR also may result from infective endocarditis, which may attack a valve previously affected

by rheumatic disease, a congenitally deformed valve, or rarely a normal aortic valve, and perforate or

erode one or more of the leaflets.

3- Patients with discrete membranous subaortic stenosis often develop thickening of the aortic valve

leaflets, which in turn leads to mild or moderate degrees of AR.

4- AR also may occur in patients with congenital bicuspid aortic valves.

5- Aortic dilatation, i.e., aortic root disease, widening of the aortic annulus and separation of the aortic

leaflets are responsible for the AR.

6- Syphilis and ankylosing rheumatoid spondylitis may lead to aortic dilatation, aneurysm formation,

and severe regurgitation.

7- Cystic medial necrosis of the ascending aorta, associated with other manifestations of the Marfan

syndrome, idiopathic dilatation of the aorta, and severe hypertension all may widen the aortic annulus

and lead to progressive AR.

8- Occasionally, AR is caused by retrograde dissection of the aorta involving the aortic annulus.

23

History: Patients with severe AR may remain asymptomatic for 10 to 15 years.

Sinus tachycardia during exertion may produce particularly uncomfortable palpitations. Exertional

dyspnea is the first symptom of diminished cardiac reserve. This is followed by orthopnea, paroxysmal

nocturnal dyspnea, and excessive diaphoresis. Chest pain occurs frequently, even in younger patients,

due to diminished coronary filling during diastole.

Nocturnal angina may be a particularly troublesome symptom. The anginal episodes can be prolonged

and often do not respond satisfactorily to sublingual nitroglycerin. Late in the course of the disease,

evidence of systemic fluid accumulation, including congestive hepatomegaly, ankle edema, and ascites,

may develop.

PHYSICAL FINDINGS: Peripheral signs: Arterial pulse: A rapidly rising “water-hammer” pulse,

which collapses suddenly as arterial pressure falls rapidly during late systole and diastole, and capillary

pulsations, an alternate flushing and paling of the root of the nail while pressure is applied to the tip of

nail, are characteristic of free AR. A booming, “pistol-shot” sound can be heard over the femoral or

brachial arteries, and a to - fro murmur is audible if the femoral artery is lightly compressed with a

stethoscope.

The arterial pulse pressure is widened, with an elevation of the systolic pressure and a depression of the

diastolic pressure. The severity of AR does not always correlate directly with the arterial pulse

pressure, and severe regurgitation may exist in patients with arterial pressures in the range of 140/60.

Palpation: The apex beat is strong and displaced laterally and inferiorly. The systolic expansion and

diastolic retraction of the apex are prominent and contrast sharply with the sustained systolic thrust

characteristic of severe AS. In many patients with pure AR or with combined AS and AR, palpation or

recording of the carotid arterial pulse reveals it to be bisferiens, i.e., with two systolic waves separated

by trough.

Auscultation: A third heart sound is common, and occasionally, a fourth heart sound also may be heard.

The murmur of AR is typically a high-pitched, blowing, decrescendo early diastolic murmur which is

usually heard best in the third left intercostal space. Unless it is trivial in magnitude, the AR is usually

accompanied by peripheral signs such as a widened pulse pressure or a collapsing pulse. On the other

hand, with the Graham steel murmur of pulmonary regurgitation, there is usually clinical evidence of

severe pulmonary hypertension, including a loud and palpable pulmonary component to the second

heart sound.

24

A midsystolic ejection murmur is frequently audible in AR. It is generally heard best at the base of

the heart and is transmitted to the carotid vessels. This murmur may be quite loud without signifying

organic obstruction; it is often higher pitched, shorter, than the ejection systolic murmur heard in

patients with predominant AS.

A third murmur which is frequently heard in patients with AR is the Austin Flint murmur, a soft,

low-pitched, rumbling middiastolic or presystolic bruit. It is probably produced by the displacement of

the anterior leaflet of the mitral valve by the aortic regurgitant stream. Both the Austin Flint murmur

and the rumbling diastolic murmur of MS are loudest at the apex, but the murmur of MS is usually

accompanied by a loud first heart sound and immediately follows the opening snap of the mitral valve,

while the Austin Flint murmur is often shorter in duration than the murmur of MS, and in patients with

sinus rhythm the latter exhibits presystolic accentuation.

Summary: Signs of aortic incompetence over the heart:

- Soft blowing early diastolic murmur over aortic area propagated to apex.

- Austin-Flint murmur (diastolic murmur over mitral area).

Echocardiogram: Essential for detection of severity and cause of AR.

TREATMENT: Although operation constitutes the principal treatment of aortic regurgitation, and

should be carried out before the development of heart failure, the latter usually respond initially to

treatment with digitalis, salt restriction, diuretics, and vasodilators, especially angiotensin-converting

enzyme inhibitors.

In patients with severe AR, careful clinical follow-up and noninvasive testing with echocardiography at

approximately 6-month intervals are necessary. Operation is to be undertaken at the optimal time, i.e.,

after the onset of left ventricular dysfunction but prior to the development of severe symptoms. Valve

replacement is indicated if the LV dilates to 50 mm in systole and 65 to 70 mm in diastole.

ACUTE AORTIC REGURGITATION: Infective endocarditis, aortic dissection, and trauma are the

most common causes of severe, acute AR.

TRICUSPID STENOSIS

25

It is generally rheumatic in origin and is more common in women than in men. It does not usually

occur as an isolated lesion or in patients with pure MR but is usually observed in association with MS.

Hemodynamically significant TS occurs in 5 to 10 percent of patients with severe MS; rheumatic TS is

commonly associated with some degree of regurgitation.

SYMPTOMS: Since the development of MS generally precedes that of TS, many patients initially have

symptoms of pulmonary congestion. Amelioration of the latter should raise the possibility that TS may

be developing. Fatigue secondary to a low cardiac output and discomfort due to refractory edema,

ascites, and marked hepatomegaly are common in patients with TS and / or regurgitation.

Severe TS is associated with marked hepatic congestion, often resulting in cirrhosis, jaundice,

serious malnutrition, anasarca, and ascites. The jugular veins are distended, and in patients with sinus

rhythm there may be giant “a” waves.

On auscultation, the pulmonic closure sound is not accentuated, and occasionally, an OS of the

tricuspid valve may be heard approximately 0.06 s after pulmonic valve closure. The diastolic murmur

of TS has many of the quality of the diastolic murmur of MS, and since TS almost always occurs in the

presence of MS, the less common valvular lesion may be missed. The murmur is augmented during

inspiration, and it is reduced during expiration.

Surgical treatment of the tricuspid valve is not ordinarily indicated at the time of mitral valve

surgery in patients with mild TS. On the other hand, definitive surgical relief of the TS should be

carried out, preferable a the time of mitral valvotomy, in patients with moderate or severe TS who have

mean diastolic pressure gradients exceeding 4 to 5 mmHg and tricuspid orifices less than 1.5 to 2.0

cm2. TS is almost always accompanied by significant tricuspid regurgitation.

TRICUSPID REGURITATION

Most commonly, tricuspid regurgitation (TR) is functional and secondary to marked dilatation of

the right ventricle and the tricuspid annulus. Functional TR may complicate right ventricular

enlargement of any cause, including inferior wall infarcts that involve the right ventricle, and is

commonly seen in the late stages of heart failure due to rheumatic or congenital heart disease with

severe pulmonary hypertension, as well as in ischemic heart disease, cardiomyopathy, and cor

pulmonale. It is in part reversible if pulmonary hypertension is relieved. Rheumatic fever may produce

organic TR, often associated with TS. Endomyocardial fibrosis, infective endocarditis may produce

TR.

26

The clinical features of TR result primarily from systemic venous congestion and reduction of

cardiac output. The neck veins are distended with prominent V waves, and marked hepatomegaly,

ascites, pleural effusions, edema, systolic pulsations of the liver and positive hepato-jugular reflux are

common. A prominent right ventricular pulsation along the let parasternal region and a blowing

holosystolic murmur along the lower left sternal margin which may be intensified during inspiration

and reduced during expiration or the Valsalva maneuver are characteristic findings; AF is usually

present.

Summary: Signs of tricuspid regurgitation

- Pansystolic murmur over tricuspid area increases with inspiration.

- Systolic neck vein pulsations

Echocardiography and Doppler: for detection of severity of TR, estimation of pulmonary pressure and

search for vegetations of infective endocarditis.

Treatment of the underlying cause of heart failure usually reduces the severity of functional TR. In

patients with mitral valve disease and TR due to pulmonary hypertension and massive RV enlargement,

effective surgical correction of the mitral valve abnormality results in lowering of the pulmonary

vascular pressure and gradual reduction or disappearance of the TR. Tricuspid valvuloplasy by De

Vega procedure and Carpentier ring can be done.

Pulmonary Stenosis: See congenital pulmonary stenosis

Pulmonary Regurgitation

Dilatation of the pulmonary artery in cases of pulmonary hypertension may produce pulmonary

regurgitation. This is called Graham Steel murmur. It is differentiated from the early diastolic

murmur of aortic regurgitation by the associated signs of pulmonary hypertension, and by Doppler

study.

27

CONGENITAL HEART DISEASE

Congenital heart malformations remain one of the most frequent birth defects, with a live-born

prevalence of about 8 per 1000 live-born infants in western countries.

Etiology of congenital heart disease:

It is generally an abnormal form of cardiac development in the first 6-8 weeks of intrauterine life. It is

either due to exposure of the fetus in this period to injurious teratogenic factor or to abnormal

chromosomal structure.

Some causes could be identified as:

1- Drugs e.g. thalidomide, excess alcohol intake, anticonvulsant drugs.

2- Exposure to radiation e.g. X-rays and gamma rays.

3- Hereditary diseases: Diseases caused by chromosomal abnormalities eg Turner syndrome, Down

syndrome or mongolism.

4- Maternal infections e.g. German measles in the first trimester of pregnancy.

Congenital heart diseases in the adults could be classified into:

I- Left or right ventricular outflow obstruction: Aortic stenosis, pulmonary stenosis, coarctation of

aorta.

II- Left to right shunts: ASD, VSD and PDA.

III- Cyanotic heart disease: Fallot’s tetralogy and other cyanotic congenital diseases.

LEET TO RIGHT SHUNT

When there is a congenital communication between both sides of the heart, e.g. atrial or ventricular

septal defects or patent ductus arteriosus the blood always flows from the left side (left atrium, left

ventricle or aorta) to right side (right atrium, right ventricle or pulmonary artery). This is because the

pressure in all left-sided chambers is higher than in right-sided chambers.

EFFECTS:

1- Left to right shunt results in pulmonary plethora (increased vascularity in the lung). If the shunt is

very big heart failure may occur but this is rare.

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2- In mild to moderate cases the pulmonary vessels dilate to accommodate the excessive blood flow.

Mild cases are well tolerated but if the shunt is excessive the pulmonary vessels react by

vasoconstriction. Pulmonary arteriolar vasoconstriction causes pulmonary hypertension which results

in right ventricular hypertrophy.

3- Pulmonary hypertension causes rise of pressure in the chambers of the right side of heart. Ultimately

the pressure in the right side exceeds that of the left side and the blood starts to flow across the defect

in the reverse direction, i.e. right to left shunt (reversed shunt). The patient becomes cyanosed. Emboli

originating in the venous side may be shunted across the defect to the arterial side and settle in organs

such as the brain or limbs. This is paradoxical embolism.

Closure of the defect at this stage is useless and dangerous. This situation of a congenital defect +

reversed shunt is called Eisenmenger’s syndrome. Eisenmenger’s syndrome is not an independent

congenital heart disease. It is the end result of big left to right shunt. At this stage the clinical picture is

that of central cyanosis with severe pulmonary hypertension.

ATRIAL SEPTAL DEFECT

In the presence of a defect in the atrial septum the right atrium receives blood both from the normal

venous return and the left atrium, the right atrium dilates. This results in: Dilatation and hypertrophy of

the right ventricle (volume overload), dilatation of the pulmonary artery, and pulmonary plethora. If the

defect is big and uncorrected pulmonary arteriolar vasoconstriction progressively occurs and results in

pulmonary hypertension usually at age 20-30 years. When the pressure in the right atrium exceeds that

in the atrium the shunt becomes reversed (Eisenmenger’s syndrome) and the patient becomes cyanosed.

Clinical features:

1- Atrial septal defect is more common in females. When the left to right shunt is very big pulmonary

plethora may predispose to repeated chest infections in infancy. Otherwise there are no symptoms for

many years. Ultimately heart failure occurs.

2- Atrial fibrillation occurs in late cases.

3- Right ventricular dilatation and hypertrophy cause a hyperdynamic impulse in the third and fourth

spaces to the left of the sternum and precordial bulge.

4- Excessive flow across the tricuspid valve may produce a third heart sound and short mid-diastolic

murmur at the tricuspid area.

29

5- Excessive blood flow at the pulmonary valve may produce pulsations, dullness and an ejection

systolic murmur in the pulmonary area.

6- The specific auscultatory sign of atrial septal defect is wide fixed splitting of the second heart at the

pulmonary area. The pulmonary component of the second sound is delayed because the right ventricle

takes a long time o empty the excessive volume of blood it receives. The splitting dose not vary with

respiration because: although inspiration causes increase in venous return, yet the resulting rise in right

a trial pressure causes proportionate decrease in the left to right shunt so that the right ventricular

output is constant and the time relation between aortic and pulmonary components of the second sound

remains constant.

7- Progressive pulmonary hypertension occurs in big defects and result in Eisenmenger syndrome. At

this stage the clinical picture consists of: Central cyanosis, signs of pulmonary hypertension, and signs

or right ventricular hypertrophy.

X-RAY PICTURE:

1- Plethoric lung fields. 2- Dilatation of the right atrium, right ventricle and pulmonary artery. 3-

Marked pulsation of the pulmonary artery and its branches seen during screening (hilar dance).

ELECTROCARDIOGRAPHIC FEATURES: The characteristic sign is incomplete right bundle branch

block with rSr' pattern in V1 lead. Signs of right ventricular hypertrophy also appear when pulmonary

hypertension develops. Atrial fibrillation occurs in late cases.

ECHOCARDIOGRAPHY WITH DOPPLER: Must be done for every patient with suspected

congenital heart disease. In A.S.D. it shows the septal defect and dilated right ventricle and abnormal

movement of the interventricular septum characteristic of volume overload on the right ventricle.

Cardiac catheterization may be done in some cases.

COMPLICATIONS:

1- Pulmonary hypertension and reversal of shunt.

2- Right ventricular failure. 3- A trial fibrillation.

TREATMENT: Small defects can be left alone. Large defects should be closed surgically or by

percutaneous insertion of occluder (device that occludes the ASD) .

VENTRICULAR SEPTAL DEFECT

1- In the presence of a defect in the septum, the right ventricle receives both the normal venous and the

shunted blood. If the defect is big right ventricular hypertrophy occurs.

30

2- This excessive blood flows in the pulmonary artery and the pulmonary circulation and then returns

to the left atrium and the left ventricle. This causes: Dilatation of the pulmonary artery, pulmonary

plethora, dilatation of the left atrium, dilatation and hypertrophy of the left ventricle.

3- If the shunt is very big excessive flow may cause heart failure in infancy.

4- If the shunt is large the pulmonary vessels react by vasoconstriction causing pulmonary hypertension

and reversal of shunt (Eisenmenger syndrome).

5- Small V.S.D. does not cause pulmonary hypertension and may close spontaneously. Clinically, the

murmur is very loud (Roger’s disease).

CLINICAL PICTURE: The specific signs of V.S.D. are: 1- A characteristic pansystolic murmur best

heart in the third and fourth left intercostal spaces just lateral to the sternum, usually accompanied by a

thrill. 2- With large shunts the increased flow across the mitral valve may cause a third sound and a

mid-diastolic flow murmur at the apex.

The clinical course depends upon the size of the defect:

1- Small ventricular septal defect: many defects close spontaneously.

2- Moderately large defect:

1st- Progressive pulmonary hypertension and low cardiac output e.g. fatigue, syncope on exercise,

pulsations and palpable loud second heart sound in the pulmonary area, right ventricular hypertrophy,

etc.

2nd- When the pressure in the right ventricle equals that in the left ventricle no blood will flow

across the defect and the murmur diminishes disappears. The patient becomes cyanosed on crying.

3rd- When the shunt is reversed the patient becomes cyanosed.

X-RAY PICTURE: Is normal in cases with small defects. Large defects result in: pulmonary plethora

(overfilled large and tortuous pulmonary arteries), large main pulmonary artery, left and right

ventricular enlargement, left atrial enlargement.

ECHOCARDIOGRAPHY WITH DOPPLER: Can show the size of cardiac chambers. The defect can

sometimes be shown by two-dimensional echo. Color Doppler is very helpful in showing the blood

flow through the defect. Detection of the site of the defect, the magnitude of the shunt and the degree of

pulmonary hypertension can be assessed by this non-invasive method.

CARDIAC CATHETERISATION AND ANGIOGRAPHY: Is done in some cases.

COMPLICATIONS: Infective endocarditis, pulmonary hypertension, and heart failure.

DIFFERENTIAL DIAGNOSIS: A pansystolic murmur at the sternal border can be caused by tricuspid

or mitral incompetence in addition to the ventricular septal defect. Sometimes the murmur of

31

pulmonary stenosis is heard at the third intercostal space but it is usually ejection in type and its

maximal intensity is in the second space. Other causes of systolic murmur at left sternal border are

hypertrophic obstructive cardiomyopathy, subaortic membrane and aortic stenosis.

TREATMENT:

1- To prevent infective endocarditis all patients must receive an antibiotic prophylaxis before

performing minor procedures that may causes bacteremia, e.g. dental extraction, delivery, etc.

2- Small ventricular septal defects should be left alone. Many of them close spontaneously.

3- Surgical closure is indicated if the defect is moderate or large in size, provided that the pulmonary

pressure is normal or moderately elevated. Surgical closure is contraindicated if pulmonary pressure is

severe (Eisenmenger’s syndrome).

PATENT DUCTUS ARTERIOSUS

The ductus arteriosus is normally present in the fetus. It connects the aorta (at the junction of the arch

with the descending aorta) with the pulmonary artery (at the junction of the main pulmonary artery with

its left branch). It normally closes. During the first month after birth:

Effects:

1- The blood flows through the duct from the aorta to the pulmonary artery, i.e. left to right shunt.

2- As the pulmonary artery receives blood both from the shunt and the right ventricle, pulmonary artery

dilatation and pulmonary plethora occur.

3- If the shunt is big pulmonary vasoconstriction and hypertension occurs. When the pressure in the

pulmonary artery equals that of the aorta the shunt will first become confined to the systole only and

then ceases altogether. The murmur, accordingly, will first become only systolic and finally will be

completely inaudible.

5- When the pressure in the pulmonary artery exceeds that of the aorta, the shunt will be reversed and

cyanosis occurs (Eisenmenger’s syndrome).

CLINICAL FEATURES: Patent ductus arteriosus is commoner in females. Its characteristic signs are:

1- A continuous (machinery) murmur that occupies both systole and diastole because the pressure in

the aorta exceeds that of the pulmonary artery all through the cardiac cycle. It is best heard in the first

and second left intercostal spaces. There may be continuous thrill in the same area.

2- With large ductus, the increased flow across the mitral may cause a mid-diastolic murmur.

32

When the pressure in the pulmonary artery exceeds that of the aorta, right to left shunt occurs and

cyanosis appears (Eisenmenger’s syndrome). The deoxygenated blood will flow from the pulmonary

artery across the ductus down the descending aorta. The lower limbs will be cyanosed while the upper

limbs remain pink (differential cyanosis).

X-RAY PICTURE: X-ray is normal in cases with small ductus. In moderate to large ductus the

following signs appear: Pulmonary plethora, enlargement of the left atrium, left ventricle and the aorta.

Hilar dance seen in the hilum by screening.

Differential diagnosis: Other causes of continuous murmur as aorto-pulmonary window, in coarctation

of the aorta, mammary softle, rupture sinus of Valsalva, venous hum...

TREATMENT: Prophylaxis against endocarditis. Closure either surgical or with a device introduced

with percutaneous, transvenous catheter.

CYANOTIC HEART DISEASE

- Tetralogy of Fallot.

- Ebstein anomaly.

- Transposition of the great arteries.

- Total anomalous pulmonary venous drainage.

- Truncus arteriosus.

- Pulmonary arterio-venous malformation.

Acquired cyanotic disease: Eisenmenger Syndrome.

FALLOT’S TETRALOGY

33

PATHOLOGY AND EFFECTS: Fallot’s tetralogy consists of:

1- Severe pulmonary stenosis which causes right ventricular hypertrophy. The pulmonary stenosis is

usually infundibular but sometimes it is both valvular and infundibular.

2- Large ventricular septal defect which makes the pressure equal in both ventricles.

3- The origin of the aorta is abnormally deviated to the right (dextroposed, dextro = right) so that it

lies partly over the right ventricle (the aorta overrides both ventricles).

4- Due to the severe pulmonary stenosis and the large ventricular septal defect, the pressure in both

ventricles is equal. There is rush of blood across the defect and the ventricular septal defect produces

no murmur.

5- Part of the blood pumped by the right ventricle passes in the aorta (right to left shunt) causing

central cyanosis.

In summary Fallot’s tetralogy consists of four components (tetra =4).

1- Pulmonary stenosis.

2- Ventricular septal defect.

3- Dextroposed and overriding aorta.

4- Right ventricular hypertrophy.

34

CLINICAL FEATURES:

1- The patient is cyanosed since birth, (usually after birth by few weeks); the degree of cyanosis

depends on the severity of the pulmonary stenosis.

2- When the patient exercises, cyanosis is increased. In order to increase the blood flow to the head and

brain, the child usually squats to compress the lower limbs against the abdomen and to deviate the

blood from the lower to the upper half of the body. It also increases the systemic arterial resistance. As

the pressure in the aorta rises, more blood will be deviated across the pulmonary stenosis to the lungs.

Thus more oxygenated blood returns to the heart.

3- Chronic cyanosis and tissue anoxia results in: Dyspnea, fatigue, angina, retarded growth,

polycythemia, clubbing of fingers.

4- Sometimes the muscle surrounding the outflow tract of the right ventricle goes into spasm,

especially after excitement and exercise. The blood flow to the lungs decreases markedly and the

oxygenation decreases resulting in attacks of severe cyanosis: cyanotic spells. If prolonged they may

lead to death.

5- The characteristic cardiac signs are:

A- Murmur of pulmonary stenosis (ejection systolic murmur in second left space, usually

accompanied by a thrill.

B- The second heart sound is single and consists only of the aortic component. C- Right

ventricular hypertrophy.

X-RAY PICTURE:

4. Right ventricular hypertrophy causes the apex to be displaced outwards and becomes separated

from the diaphragm.

5. Right-sided aortic arch in some cases.

6. Pulmonary oligemia (the pulmonary artery and its branches are diminished in size due to the

pulmonary stenosis. All the above factors result in a characteristic cardiac shadow: Coeur en sabot

(sabot = wooden shoe).

ELECTROACARDIOGRAPHIC FEATURES: Show moderate right ventricular hypertrophy.

ECHOCARDIOGRAPHY WITH DOPPLER: Delineates the abnormal anatomy. Cardiac

catheterization and angiography is needed for differential diagnosis.

COMPLICATIONS:

1- Polycythemia causes increased viscosity of blood resulting in a tendency towards thrombosis, e.g.

cerebral thrombosis.

35

2- Infective endocarditis

3- Brain abscess results when bacterial emboli are shunted from the venous to the arterial side and

lodge in the brain (paradoxical embolism).

TREATMENT:

1- Surgical correction is indicated in all cases by: Resection of the excessive stenotic infundibular

muscle splitting of the fused pulmonary valve leaflets, and closure of the ventricular septal defect.

2- If he patient is too young, or the condition is too severe, an anastomosis is performed to allow blood

to reach the lungs by: implanting the subclavian artery in the corresponding pulmonary artery (Blalock-

Taussig operation).

3- Cyanotic attacks result from infundibular spasm and constitute an emergency. The are treated by:

Put the patient in the squatting position or compress the flexed lower limbs against the abdomen,

sedation, propranolol (inderal) intravenously. Propranolol is a beta-adrenergic blocker. It depresses the

contractility of the infundibular muscle.

LEFT VENTRICULAR OUTFLOW TRACT OBSTRUCTION

- Valvular aortic stenosis: 70% of patients with valvular AS a malformation of the valve (usually a

bicuspid valve).

- Discrete subvalvular aortic membrane:

Represents 8-10% of congenital AS. The magnitude of obstruction is variable. Most membranes are

eventually associated with progressive aortic regurgitation and their presence may be an absolute

indication for excision. There is a high recurrence rate after excision (approximately 30% and septal

myotomy is often performed).

COARCTATION OF THE AORTA

Narrowing of the aorta usually just distal to the left subclavian artery. Coarctation may affect other

parts of the aorta or the renal arteries.

EFFECTS:

1- Because of the narrowing, pressure rises in the ascending aorta and the aortic arch and its branches.

This results in hypertension in the upper limbs.

2- Pressure and flow decreases in the descending aorta and its branches producing ischemia in the

abdominal organs and the limbs.

3- Ischemia of the kidneys results in release of renin which raises the blood pressure.

36

4- Hypertension results in left ventricular hypertrophy and it severe results in left ventricular failure.

5- Anastomosis form between the branches of the aorta proximal and distal to the obstruction. The

most important of these connect the subclavian artery through its internal mammary branch to the

intercostal arteries which arise from descending aorta. The intercostal arteries become enlarged and

tortuous and erode the lower border of the ribs causing rib notching. Appreciable anastomosis

develops gradually by time. That is why rib notching is not detectable except after the age of 10. Other

anastomosis develops around the scapula and another connects the superior and inferior epigastric

arteries.

CLINICAL FEATURES:

1- In the majority of cases there are no symptoms and the essential diagnostic feature of coarctation is

that the blood pressure in the upper limbs exceeds that in the lower limbs.

2- The pulse in the upper limbs, neck and suprasternal notch is strong. Pulse in the lower limbs is weak

and delayed or absent.

3- Hypertension in the upper half of the body may produce headache, epistaxis while ischemia of the

lower half may produce thin, underdeveloped lower limbs and claudication in the calf.

4- Visible and palpable pulsations of dilated collateral may be felt in the intercostal areas.

5- A late systolic murmur may be heard on the back due to blood flow in the collaterals. The murmur is

sometimes continuous.

6- The cardiac signs are nonspecific and include: left ventricular hypertrophy, an ejection systolic

murmur heard at the aortic area.

X-RAY PICTURE:

1- Signs of left ventricular hypertrophy.

2- Rib notching is the most specific sign.

ELECTROCARDIOGRAPHIC SIGNS: Left ventricular hypertrophy and strain.

COMPLICATIONS:

1- Hypertension in the upper half of the body may result in: cerebral or subarachnoid hemorrhage, left

ventricular failure, dissection of the aorta.

2- Infective endocarditis.

TREATMENT: surgical resection of the narrowed segment is indicated in moderate and severe cases

preferably during childhood. Balloon dilation with expandable stent is a feasible method of treatment.

All patients must have prophylaxis against endocarditis.

37

PULMONARY STENOSIS

Pulmonary stenosis may be caused by: Congenital fusion of pulmonary valve cusps (congenital

valvular pulmonary stenosis).

EFFECTS:

1- In both valvular and infundibular stenosis the pressure in the right ventricle rises, this causes

hypertrophy of the right ventricle (pressure over-load). Consequently the right atrium hypertrophies.

When the stenosis is severe the output of the right ventricle and the cardiac output are reduced. The

pulmonary blood flow is reduced, i.e. pulmonary oligemia.

CLINICAL FEATURES:

1. Mild cases are as asymptomatic, in severe cases low cardiac output occurs and results in

fatigability, syncope on effort, small volume pulse, cold extremities, etc.

2. An ejection systolic murmur is caused by passage of blood through the stenosed valve. It is best

heard over the pulmonary area. It may be preceded by an ejection click.

3. The pulmonary component of the second heart sound is faint and delayed due to prolonged

contraction of the right ventricle.

4. There is usually a systolic thrill over the pulmonary area.

5. Right ventricular hypertrophy produces a sustained impulse in the third and fourth intercostal

spaces just to the left of the sternum and pulsation in the epigastrium. Forceful right atrial contraction

causes a large wave in the neck veins (the a wave).

X-RAY PICTURE: 1. Pulmonary oligemia occurs in moderate to severe cases and results in reduced

pulmonary vascular markings). 2- Right ventricular enlargement is proportional to the severity of the

stenosis. Right atrial enlargement may also occur. 3. Post-stenosis dilatation of the pulmonary artery

is seen.

ECG FEATURES: Right ventricular hypertrophy.

ECHO FEATURES: Right ventricular hypertrophy, the stenosed pulmonary valve.

TREATMENT: Either percutaneous transvenous balloon dilatation (the standard treatment, first

option) or surgical removal of the valve by open-heart surgery.

Interventions In Congenital Heart Diseases (therapeutic procedures that are used in treatment

without surgery but through catheterization):

38

- Pulmonary stenosis balloon dilatation.

- Aortic stenosis balloon dilatation.

- Coarctation of the aorta balloon dilatation and stent insertion.

- Atrial septal defect insertion of Amplatzer occluder through catheter.

- Patent ductus arteriosus occlusion by insertion of coil.

- Other procedures.

DIAGNOSIS AND MANAGEMENT OF SYNCOPE AND HYPOTENSION

Syncope is a sudden and transient loss of consciousness with associated loss of postural tone. The

occurrence of syncope is 3% in men ad 3.5% in women in the general population. As a general role, the

incidence of syncope increases with age.

Hypotension: When systolic blood pressure (SBP) is less than 90 mmHg or reduction of SBP of 30

mmHg or more from baseline.

Patients with transient episode of altered consciousness (presyncope) and those with complete loss of

consciousness (syncope) are classified into 3 broad categories: cardiac syncope, noncardiac syncope,

and syncope of undetermined etiology. Among all patients with syncope associated with cardiac

disease, sudden cardiac death is extremely high.

Table: Causes of Syncope

Circulatory (reduced cerebral blood flow)

A. Inadequate vasoconstrictor mechanisms

1. Vasovagal (vasodepressor)

2. Postural hypotension

3. Primary autonomic insufficiency

4. Sympathectomy (pharmacologic, due to antihypertensive medications such as methyldopa and

hydralazine, or surgical )

5. Carotid sinus syncope

6. Diseases of the central and peripheral nervous system, including autonomic nerves)

B. Hypovolemia

1. Blood loss – gastrointestinal hemorrhage.

2. Addison’s disease

C. Mechanical reduction of venous return

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1. Valsalva maneuver. 2. Cough; Micturition.

3. Atrial myxoma, ball valve thrombus.

D. Reduced cardiac output

1. Obstruction to left ventricular outflow: aortic stenosis, hypertrophic subaortic stenosis.

2. Obstruction to pulmonary flow: pulmonary stenosis, primary pulmonary hypertension, pulmonary

embolism.

3. Myocardial: massive myocardial infarction with pump failure.

4. Pericardial: cardiac tamponade

E. Arrhythmias

1. Bradyarrhythmias

a. Atrioventricular (AV) block (second and third degree), with Stokes-Adams attacks

b. Ventricular asystole

c. Sinus bradycardia, sinoatrial block, sinus arrest, sick sinus syndrome

d. Carotid sinus syncope

a. Tachyarrhythmias: Supraventricular tachycardia. Episodic ventricular tachycardia

Other causes of disturbances of consciousness

A. Hypoglycemia

B. Hypoxia

C. Hypoventilation

D. Transient cerebral ischemic attack

E. Emotional disturbances, anxiety attack, hysterical seizures.

Noncardiac Syncope

Neurocardiogenic syncope:

The syndrome of neurocardiogenic syncope, the common faint (also referred to as neurally mediated

hypotension, vasovagal syncope, and vasodepressor syncope), is one of the most common causes of

syncope.

This disorder is due to abnormality in the neuro-cardiovascular interactions responsible for maintaining

systemic and cerebral perfusion.

Diagnostic evaluation:

Head-up tilt (HUT) is essential for the diagnosis of neurocardiogenic syncope. Here we change the

position of the patient from the horizontal to the vertical position. HUT at an angle of 60º to 90º for a

time period of 20 to 60 min is the usual protocol.

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Management of syncope:

First-line therapy includes counseling the patient to avoid dehydration, prolonged period of standing

motionless, and situations known to trigger syncope. Volume expansion, fludrocortisone may be

helpful in augmenting salt retention and volume expansion.

Alpha-Agonists: Medodrine may prevent neurocardiogenic syncope due to vasoconstrictor effect that

may reduce venous pooling.

Orthostatic Syncope (orthostatic Hypotension):

Orthostatic hypotension is a disorder in which assumption of the upright posture is associated with a

fall in blood pressure. Therapy: is based on treatment of causes.

Management of hypotension: 1- Treatment of the etiology. 2- Avoid dehydration. 3- Medodrine. 4.

Mineralocorticoids as Astonin H.

Cardiac Syncope

It is due to severe diminution of the cardiac output Either due to severe obstructive lesion as tight mitral

stenosis, atrial myxoma, aortic stenosis, obstructive cardiomyopathy or due to arrhythmia whether

tachy or brady. Obstructive lesions and arrhythmias frequently coexist; indeed, one abnormality may

accentuate the other. Common disorders associated with cardiac syncope are listed in table.

Diagnostic evaluation of syncope associated with cardiac disease:

- History & physical examination

- Echocardiography & Doppler

- Standard ECG

- Holter monitor ( 24 h. ECG continuous recording )

- Electrophysiologic study.

- Cardiac catheterization.

Treatment of cardiac syncope: Obstructive Heart Disease, for patients with syncope caused by

obstructive heart disease, cardiac surgery is often the treatment of choice.

Arrhythmic syncope, detailed discussion of therapy for cardiac arrhythmias presented earlier.

Antiarrhythmic drugs, pacemakers and ablation are available tools of management of arrhythmia.

Syncope of undetermined cause: Despite careful diagnostic evaluation, the cause of syncope often

cannot be defined.

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Sudden Cardiac Death

Definition: Sudden cardiac death describes the unexpected natural death due to cardiac cause within a

short period from the onset of symptoms.

More recent definition focused on time interval of one hour from the symptoms leading to collapse and

then to death.

Incidence: SCD accounts for 300.000 to 400.000 deaths yearly in the United States. SCD is the most

common and often the first manifestation of coronary heart disease (CHD) and is responsible for half

the deaths from cardiovascular disease.

Sudden Cardiac Death in the young: The most common underlying pathological conditions in people

who die of SCD in the first three decades of life are myocarditis, hypertrophic cardiomyopathy,

congenital coronary artery anomalies, atherosclerotic coronary heart disease, conduction system

abnormalities (e.g. long QT), congenital arrhythmogenic disorders, arrhythmias associated with

mitral valve prolapse and aortic dissection. About 40% of SCD in the pediatric population occur in

patients with surgically treated congenital cardiac abnormalities.

Risk factors for Sudden Cardiac Death (SCD):

1- Left ventricular hypertrophy (by ECG)

2- Cholesterol.

3- Hypertension.

4- Cigarette smoking.

5- Diabetes.

6- Alcohol.

7- Obesity.

8- History of coronary heart disease.

9- Age.

10- Positive family history of SCD.

11- Frequent PVCs (Premature ventricular contractions, unsustained ventricular tachycardia).

Cardiac Abnormalities Associated with Sudden Cardiac Death

I. Ischemic heart disease

A) Coronary Atherosclerosis:

- Acute myocardial infarction, - Chronic ischemic cardiomyopathy

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B) Anomalous origin of coronary arteries.

II. Cardiomyopathies

A. Idiopathic dilated cardiomyopathy

B. Hypertrophic cardiomyopathy

C. Hypertensive cardiomyopathy

D. Arrhythmogenic right ventricular dysplasia

III. Valvular heart disease: Aortic stenosis

IV. Inflammatory and Infiltrative myocardial disease

V. Congenital heart disease.

VI. Primary Electrical Abnormality.

A. Long Q-T syndrome

B. Wolf Parkinson White syndrome (WPW).

C. Idiopathic ventricular tachycardia

D. Idiopathic ventricular fibrillation

E. Brugada syndrome (right bundle block with raised ST in V1 to V3)

VII. Drug and other toxic agents

A. Proarrhythmia (Drug induced arrhythmia)

B. Cocaine and Alcohol. C. Electrolyte abnormalities

Treatment Options for Patients at Risk of Sudden Cardiac Death (SCD)

I. Pharmacologic therapy

1- Beta blockers , Angiotensin-converting enzyme inhibitors

2- Class I antiarrhythmic drugs,

3- Class III antiarrhythmic drugs: Amiodarone, sotalol

II. Device therapy

1- Automatic implantable cardioverter Defibrillator (ICD)

2- External automatic defibrillator

III. Role of surgery: Revascularization

IV. Catheter Ablation therapy.

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CARDIAC ARRHYTHMIAS

An arrhythmia is any disturbance in the normal sequence of impulse generation and conduction in the

heart.

Anatomy of the conduction system: The conduction system of the heart consists of the sinus node,

internodal tracts, atrioventricular node (AVN), bundle of His, bundle branches (right and left), and

Purkinje fibers.

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Fig: The pathways of Conduction.

General considerations: Normal cardiac impulses arise from the automatic (pacemaking) cells of the

sinus node and are conducted through the atria to the AV junction then the His-Purkinje system to the

ventricular muscle. Normally the sinus node discharges at a rate of 60-100/min.

Mechanisms of arrhythmias

A- Disturbance of impulse formation: may result from either:

1- Disturbed normal automaticity:

2- Triggered activity: Hyper-excitable focus which discharges ectopic impulses.

B- Disturbance of Impulse conduction: e.g. heart block

Classification of arrhythmia:

Clinical classification:

- Rapid, regular. Sinus tachycardia, supraventricular tachycardia, atrial flutter, ventricular

tachycardia.

- Rapid, irregular. Sinus arrhythmia, multiple ectopic beats whether atrial or ventricular, atrial

fibrillation.

- Slow, regular. Sinus bradycardia, nodal rhythm, complete heart block.

- Slow, irregular. Slow atrial fibrillation.

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Disturbances in Sinus Rhythm

Sinus tachycardia

Cardiac impulses arise in the sinus node at a rate more than 100/min.

Etiology:

A- Physiological: Infancy, childhood, exercise and excitement.

B- Pharmacological: Sympathomimetic drugs such as epinephrine and isoproterenol.

Parasympatholytic drugs such as atropine. Thyroid hormones, nicotine, caffeine, alcohol.

C- Pathological: Fever, hypotension, heart failure, pulmonary embolism, hyperkinetic circulatory

states as anemia.

Treatment: 1- Treatment of the underlying etiology. 2- Propranolol.

Sinus Bradycardia

Cardiac impulses arise in the sinus node at a rate less than 60/min.

Etiology:

A- Physiologic: Athletes, sleep, and carotid sinus compression.

B- Pharmacologic: Digitalis, propranolol, verapamil and diltiazem.

C- Pathologic: Convalescence from infections, hypothyroidism, obstructive jaundice, rapid rise of the

intracranial tension, hypothermia and myocardial infarction (particularly inferior wall infarction).

Treatment:

1- Treatment of the underlying etiology is usually all that is needed.

2- If the patient is hemodynamically compromised, Atropine 0.6 – 1.0 mg IV may be given and

repeated every 3 hours (maximum 2.5 mg in two hours).

SICK SINUS SYNDROME: This term is applied to a syndrome encompassing a number of sinus

nodal abnormalities that include: 1- persistent spontaneous sinus bradycardia not caused by drugs, and

inappropriate for the physiological circumstance, 2- apparent sinus arrest or exit block, 3- combinations

of SA and AV conduction disturbances, or 4- alternation of paroxysms of rapid and slow atrial and

ventricular rates (bradycardia-tachycardia syndrome).

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FIG. Normal intracardiac electrograms.

PREMATURE BEATS (EXTRASYSTOLES)

These are cardiac impulses of ectopic origin occurring earlier than expected in the prevailing rhythm.

The ectopic focus may be: 1- Atrial resulting in atrial premature beat. 2- AV junctional (arising from

bundle of His) resulting in AV junctional premature beat. 3- Ventricular resulting in ventricular

premature beat.

Etiology:

A- Physiological: Emotions, exercise and fatigue.

B- Pharmacological: Coffee, alcohol, tobacco, catecholamines, digitalis and hypoxia.

C- Pathological: Various infections, digestive disturbances, hyperthyroidism and all cardiovascular

disorders.

SUPRAVENTRICULAR TACHYARRHYTHMIAS

All tachyarrhythmias that originate above the bifurcation of the bundle of His are classified as

supraventricular arrhythmias (SVT). The atrial rate must be 100 or more beats per minute for a

diagnosis.

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SVTs may be separated into three groups based on duration: brief paroxysms, persistent, and chronic

(permanent).

Arrhythmias that are paroxysmal in onset and offset (e.g., paroxysmal SVT due to AV nodal reentry or

WPW syndrome, paroxysmal atrial fibrillation, paroxysmal atrial flutter) tend to be recurrent and of

short duration; i.e., seconds to hours.

Persistent tachycardias (e.g., sinus tachycardia, ectopic atrial tachycardia (nonparoxysmal), multifocal

atrial tachycardia, longer episodes of PSVT or atrial flutter or fibrillation) may persist for days or

weeks.

Longstanding or chronic SVTs (chronic atrial flutter, chronic atrial fibrillation) do not revert if

untreated, often fail to revert even with attempted treatment, and if reverted will frequently recur

despite therapy.

Supraventricular tachyarrhythmias include; atrial tachycardia, atrial flutter, atrial fibrillation and AV

tachycardias.

ATRIAL FLUTTER

Atrial flutter is a rapid regular atrial tachyarrhythmia that is less common than the PSVTs or atrial

fibrillation. It is observed in the presence of underlying atrial abnormalities such as those secondary to

mitral valve disease, congenital heart disease, cardiomyopathies, and, less frequently, coronary artery

disease.

Untreated atrial flutter usually has atrial rates between 240 and 340 per minute, commonly very close

to 300 per minute. The ventricular rate in atrial flutter is usually a defined fraction of the atrial rate 2: 1

conduction generating a ventricular rate of 150 per minute and 4:1 conduction at 75 per minute.

Clinically, atrial flutter may occur in brief, persistent, or chronic forms, and therapeutic approaches are

influenced by the clinical pattern.

Electrocardiographic Features

Atrial flutter generates a defined pattern of atrial activity in the ECG. Classically, a saw-tooth pattern is

identifiable in leads II, 111, and aVF. A narrow QRS complex tachycardia at a rate of 150 per minute

should always lead to the consideration of atrial flutter. Carotid sinus massage will not interrupt atrial

flutter but nonetheless may be very helpful in distinguishing flutter from other mechanisms,

impairment of AV nodal conduction causes an abrupt change from a rate of 150 per minute to 75 per

minute or less.

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Management of atrial flutter: - If the patient is hemodynamically compromised, D.C. cardioversion

using low energies (around 50 joules) should be instituted.

- Administering a Class IA antiarrhythmic agent (i.e., quinidine, procainamide, or disopyramide). IC

antiarrhythmic drugs, flecainide and propafenone, are as effective, if not more effective than Class IA

drugs. Class III antiarrhythmic agents (i.e., amiodarone, sotalol) may also be quite effective. In general,

atrial flutter is difficult to suppress completely with drug therapy. - The ventricular rate is slowed by

digitalis and/or propranolol or verapamil before antiarrhythmics are instituted to avoid very rapid rates

associated with drug induced 1:1 AV conduction.

- At present, catheter ablation provides the best hope of cure.

FIG. A 12-lead ECG of a typical case of type 1 atrial flutter.

FIG. A 12-lead ECG of a typical case of type 1 atrial flutter.

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FIG: Atrial flutter with AV block varying between 2: 1 and 4: 1.

AV Nodal Reentrant Tachycardia

Electrocardiographic Features: Paroxysmal SVT due to AV nodal reentry is characterized by an

abrupt onset and termination and usually has a narrow QRS complex without clearly discernable P

waves. The rate is commonly in the range of 150 to 250 per minute (commonly 180 to 200 bpm in

adults) and with a regular rhythm.

Management of PSVT Due to AV Nodal Reentry

The acute attack: Vagal maneuvers serve as the first line of therapy. Simple procedures to terminate

paroxysmal SVT

- Carotid sinus massage: If effective the rhythm is abruptly stopped; occasionally only moderate

slowing occurs

- Cold water splash on face.

- Performance of Valsalva's maneuver (often effective).

Intravenous adenosine, Ca channel blockers (verapamil), digoxin or B-blockers are the choices for

managing the acute episodes.

Adenosine, 6 mg given intravenously, followed by one or two 6-mg boluses if necessary, is effective

and safe for acute treatment.

A 5-mg bolus of verapamil (isoptin) , followed by one or two additional 5-mg boluses 10 min apart if

the initial dose does not convert the arrhythmia, has been an effective regimen in up to 90 percent of

patients with PSVT due to AV node reentry. Intravenous digoxin, 0.5 mg infused over 10 min and

repeated if necessary may convert the arrhythmia.

DC cardioversion: Consider DC cardioversion before digitalis or a beta blocker is administered.

Radiofrequency catheter ablation: Should be considered early in the management of patients with

symptomatic recurrent episodes of AV node reentry.

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AV Reentrant Tachycardia

PSVT Due to Accessory Pathways (The Wolff-Parkinson-White Syndrome)

ELECTROCARDIOGRAPHIC RECOGNITION: Three basic features in the ECG of patients with the

usual form of WPW syndrome caused by an AV connection:

(1) Short P-R interval less than 120 msec during sinus rhythm;

(2) QRS complex duration exceeding 120 msec

(3) Slowly rising onset of the QRS in some leads (delta wave).

The most common tachycardia is characterized by a normal QRS, by ventricular rates of 150 to 250

beats/min and by sudden onset and termination.

Termination of the acute episode should be approached as for AV nodal reentry. In many patients,

particularly those with a very rapid ventricular response, electrical cardioversion is the initial treatment

of choice.

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The Wolff-Parkinson-White Syndrome

ELECTRICAL ABLATION: Ablation of the accessory pathway is advisable for patients with frequent

symptomatic arrhythmias that are not fully controlled by drugs.

Atrial Fibrillation

The arrhythmia is characterized by multiple electric foci in the atrium causing disorganized atrial

depolarizations without effective atrial contraction. Electrical activity of the atrium can be detected on

ECG as small irregular baseline undulations, called f waves, at a rate of 350 to 600 beats/min. The

ventricular response is grossly irregular (irregular irregularity) and is usually between 100 and 160

beats/min.

It is a common arrhythmia, occurring in 5 – 10 % of individuals over 65 years of age. It also occurs in

a paroxysmal form in younger patients.

The hemodynamic consequences of atrial fibrillation are due to two factors:

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(1) The loss of atrial systole may impair ventricular function in the noncompliant ventricle [e.g., aortic

stenosis, left ventricular hypertrophy (LVH)] or the dilated ventricle with systolic dysfunction, and

(2) A rapid ventricular rate will encroach upon the diastolic filling period of the left ventricle and the

diastolic flow time of the coronary arteries.

(3) The risk of embolism and stroke is a long-term concern of special importance. Atrial fibrillation

may occur in paroxysmal, persistent, and chronic patterns.

Clinical expression of atrial fibrillation:

Definition Duration

- Paroxysmal Minutes/hours

- Short-lasting Seconds --<1 hour

- Long-lasting >1 hour; -- < 48 hours

- Persistent Two days -- weeks

- Permanent (Chronic) Months / years

Table: Causes of atrial fibrillation

With structural heart disease

- Rheumatic mitral valve disease

- Ischemic heart disease

- Hypertension

- Cardiomyopathy: Dilated, Hypertrophic

- Atrial septal defect, - Constrictive pericarditis, Myocarditis

Without structural heart disease

- Alcohol. Thyrotoxicosis

- Acute pericarditis. Pulmonary embolism

- Sick sinus syndrome, Lone atrial fibrillation

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Atrial Fibrillation

Clinical picture

Onset and offset are sudden in paroxysmal cases.

Symptoms: Paroxysmal AF produces symptoms similar to those of supraventricular tachycardia.

Established AF (persisting for more than two weeks) is better tolerated than the paroxysmal variety.

Congestive heart failure may occur if the attack is prolonged, the ventricular rate is very rapid, or the

underlying heart disease is severe.

Signs:

1- Arterial pulse:

a- Rate is usually 100-150/min. Slower rates may be encountered in old age and in patients receiving

digitalis or beta-blockers.

b- Rhythm shows marked (irregular) irregularity. c- Force is irregular. d- Pulsus deficit: The radial

pulse rate is less than the cardiac rate counted at the apex beat. This is due to inability of the week

ventricular contractions following short diastolic periods to open the aortic valve.

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2- Neck veins show systolic expansion; no “a” waves are seen.

3- Auscultation reveals varying intensity of S1.

4- Exercise increases the pulse irregularity and deficit.

Electrocardiogram: The P waves are replaced by irregular f waves. The QRS complexes are normal in

shape but irregularly spaced.

Complications: 1- Atrial thrombosis due to stagnation of blood in the fibrillating atria. The formed

thrombi may embolize in the systemic and pulmonary circulations. 2- Heart failure due to loss of the

atrial contribution to contractility and the cardiac output.

Atrial fibrillation (AF) progressed to ventricular fibrillation (VF)

Treatment of Atrial Fibrillation

Pharmacologic Management of Patients with Recurrent Persistent or Permanent AF:

- Recurrent Persistent AF:

A) Minimal or no symptoms: Anticoagulation and rate control as needed.

B) Disabling symptoms in AF:

1- Anticoagulation and rate control

2- Antiarrhythmic drug therapy

3- Electrical cardioversion as needed, continue anticoagulation as needed and therapy to maintain

sinus rhythm

- Permanent AF: Anticoagulation and rate control as needed.

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AF management

Antiarrhythmic Drug Therapy to Maintain Sinus Rhythm in Patients with Recurrent

Paroxysmal or Persistent AF:

A) No or minimal heart disease:

1- Flecainide, propafenone, sotalol

2- Amiodarone, dronedarone, dofetilide, Disopyramide, procainamide, quinidine

3- Consider non-pharmacological options (ablation).

B) Heart disease present:

a- Heart failure: Amiodarone, dofetilide

1- Coronary artery disease: Sotalol, Amiodarone, dofetilide

2- Dronedarone is allowed only in HF class I or II with precaution.

3- Vernakalant I.V. for aute AF of less than 7 days duration, with many precautions and

contraindications.

C) Hypertension: With

1- With LVH (septum greater than or equal to 1.4 cm): Amiodarone

2- Without this degree of LVH: - Flecainide, propafenone.

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Drugs for Pharmacologic Cardioversion of AF (Rhythm

control)

Drug Route of Admin. And Dosage

Amiodarone Oral: 1.2 to 1.8 g /day then 200 to 400 mg /d maintenance.

IV: 1.2 g /d IV continuous or in divided doses, then 200 to 400

mg /d maintenance

Dofetilide Oral: Creatinine clearance > 60 ml/min: 500 mcg BID

Flecainide Oral 200 to 300 mg

IV: 1.5 to 3 mg /kg over 10 to 20 min

Propafenone Oral: 450 to 600 mg

IV: 1.5 to 2 mg per kg over 10 to 20 min

Orally Administered Pharmacological Agents for Heart

Rate Control in Patients with AF

Drug Maintenance dose

Digoxin 0.125 to 0.375 mg daily

Metoprolol* 25 to 100 BID

Propranolol 80 to 360 mg daily in divided doses

Verapamil 120 to 360 mg daily in divided doses

Diltiazem 120 to 360 mg daily in divided doses

Anticoagulation of Patients with Atrial Fibrillation: Indications

Rheumatic mitral valve disease with recurrent or chronic atrial fibrillation.

Dilated cardiomyopathy with recurrent persistent or chronic atrial fibrillation.

Prosthetic valves.

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Prior to (>3 weeks) elective cardioversion of persistent or chronic atrial fibrillation, and also for 3

weeks after cardioversion (because of atrial stunning).

Coronary heart disease or hypertensive heart disease with recurrent persistent or chronic atrial

fibrillation

Atrial fibrillation in thyrotoxicosis (while awaiting long-term control; elective cardioversion)

Chronic or persistent lone atrial fibrillation, age >60 years

Controversial; or limited data

Coronary or hypertensive heart disease with normal left atrial size, after first episode of paroxysmal

atrial fibrillation

Elective cardioversion of atrial fibrillation of short duration (2-3 days) with normal left atrial size

Chronic or persistent lone atrial fibrillation, age <60 years

Not indicated

Lone atrial fibrillation, short paroxysms (<48 h)

Most clinical settings associated with short paroxysms (minutes to hours)

Relative contraindications

Difficulty controlling prothrombin times. Dementia

Malignancies, especially associated with bleeding risk

Prior major bleeding events. Uncontrolled hypertension

Treatment of Cardiac Arrhythmias with Catheter Ablative Techniques

Radiofrequency ablation destroys tissue by controlled heat production. Catheter ablation is used to treat

patients with four major tachyarrhythmias: atrial flutter/fibrillation, AV nodal reentry, accessory

pathways and ventricular tachycardia.

VENTRICULAR TACHYCARDIA

Specific Forms of Ventricular Tachycardia

Duration: Salvo (3-5 impulses)

Nonsustained VT: (6 impulses, up to 29 seconds)

Sustained VT: (>30 seconds)

The electrocardiographic diagnosis of ventricular tachycardia is suggested by the occurrence of a series

of three or more bizarrely shaped premature ventricular complexes whose duration exceeds 120 msec,

with the ST-T pointing opposite to the major QRS deflection.

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The rates range from 70 to 250 beats/min. Ventricular tachycardia can be sustained, defined arbitrarily

as lasting longer than 30 sec or requiring termination because of hemodynamic collapse, or

nonsustained (Unsustained), when it stops spontaneously in less than 30 sec.

Ventricular tachycardia (Wide QRS tachycardia)

Management: Intravenous lidocaine or amiodarone, followed by an infusion of the successful drug. If

the arrhythmia does not respond to medical therapy, electrical DC cardioversion can be employed.

Ventricular tachycardia in a patient with right ventricular dysplasia.

CONGENITAL LONG QT INTERVAL SYNDROME

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The normal QT interval is .43 sec. The congenital long QT interval syndrome, which is present

persistently from childhood, is characterized by the presence of long QT intervals on the standard 12-

lead ECG. The affected patients are prone to episodes of torsade de pointes (ventricular tachycardia

with special polymorphic configuration), which may cause transient light-headedness or syncope or

sudden cardiac death. Arrhythmias may occur at rest, under emotional stress, or with exercise.

ACQUIRED LONG QT INTERVAL SYNDROME

Causes: Antiarrhythmic drugs as quinidine. There is a growing list of other drugs that may prolong the

QT interval, and establish susceptibility to torsade de pointes. These include the phenothiazines, certain

antibiotics, pentamidine, cocaine, and terfenadine, among others.

Management of Congenital Long QT Interval Syndrome: Long-term therapy includes B-adrenergic

blockade. Placement of an ICD should be considered for patients with resistant arrhythmias.

CARDIOVERSION AND DEFIBRILLATION

Differences between cardioversion and defibrillation:

Cardioversion Defibrillation

Elective Emergency

Synchronized Non-synchronized

For AF, A. flutter, SVT, VT For V. fibrillation

50, 100, 150, 200 Joules Start by 200 Joules

Need sedative first Patient is unconscious

VENTRICULAR FLUTTER AND FIBRILLATION

MANAGEMENT: Immediate nonsynchronized DC electrical shock using 200 to 360 joules is

mandatory treatment for ventricular fibrillation. Cardiopulmonary resuscitation is employed only until

defibrillation equipment is ready. Time should not be wasted with cardiopulmonary resuscitation

maneuvers if electrical defibrillation can be done promptly.

The Implantable Cardioverter Defibrillator (ICD)

Apparatus (pacemaker) that gives electric shock if the patient develops ventricular fibrillation. The

pacemaker is inserted in the sub-pectoral area.

ICD indications

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A. Cardiac arrest not due to acute ischemia or infarction or reversible causes.

B. Documented sustained VT with hemodynamic compromise.

C. Syncope of unknown origin in structural heart disease patients with inducible sustained VT.

D. Cardiomyopathy ischemic or non-ischemic with ejection fraction 30% or lower (MADIT II results).

AV HEART BLOCK

Heart block is a disturbance of impulse conduction that can be permanent or transient, owing to

anatomical or functional impairment.

The conduction disturbance is classified by severity in three categories.

During first degree heart block, conduction time is prolonged but all impulses are conducted (P-R

interval > 0.2 sec.).

Second degree heart block occurs in three forms:

Mobitz type I (Wenckebach) and type II; and persistent 2:1 block.

Mobitz Type I heart block is characterized by a progressive lengthening of the conduction time until an

impulse is not conducted (Fig).

Mobitz Type II heart block denotes occasional (Mobitz II) or repetitive sudden block of conduction of

an impulse without prior measurable lengthening of conduction time. When no impulses are conducted,

complete or third degree block is present.

Mobitz type I (Wenckebach) block

Mobitz Type II second degree heart block

COMPLETE AV BLOCK

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ELECTROCARDIOGRAPHIC RECOGNITION: Complete AV block occurs when no atrial activity

conducts to the ventricles and therefore the atria and ventricles are controlled by independent

pacemakers. Thus, complete AV block is one type of complete AV dissociation.

The ventricular focus is usually located just below the region of block, which can be above or below

the His bundle bifurcation. The ventricular rate of acquired complete heart block is less than 40

beats/min but may be faster in congenital complete AV block.

CLINICAL FEATURES. Block proximal to the His bundle generally exhibits normal QRS complexes

and rates of 40-60 beats/min because the escape focus that controls the ventricle arises in or near the

His bundle.

Causes: Surgery, electrolyte disturbances, endocarditis, tumors, Chagas' disease, rheumatoid nodules,

calcific aortic stenosis, myxedema, polymyositis, infiltrative processes (such as amyloid, sarcoid, or

scleroderma). In the adult, drug toxicity, coronary disease, and degenerative processes appear to be the

most common causes of AV heart block.

COMPLETE AV BLOCK

MANAGEMENT: Temporary or permanent pacemaker insertion is indicated in patients with

symptomatic bradyarrhythmias. Vagolytic agents such as atropine (novatropine 15 drops every 8 hours)

are useful, while catecholamines such as isoproterenol (Allupent syrup 5 ml every 8 hours) can be used

transiently to treat patients who have heart block. The use of transcutaneous pacing is preferable.

ELECTROPHYSIOLOGIC STUDY

EP study is an invasive procedure in which intracardiac electrode catheters are used to evaluate cardiac

arrhythmias and to select various therapeutic options.

Indications of EPS:

Diagnostic:

Aborted SCD (sudden cardiac death). - Syncope of undetermined cause.

Recurrent WCT (wide complex tachycardia). - Ventricular tachycardia.

Recurrent tachycardia with WPW syndrome.

Symptomatic refractory NCT (narrow complex tachycardia).

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Therapeutic:

Catheter ablation for AVNRT (AV nodal reentrant tachycardia), WPW (Wolff Parkinson White

syndrome), VT (Ventricular Tachycardia), Atrial fibrillation.

Acute termination of hemodynamically unstable tachycardias.

CARDIAC PACEMAKERS

Cardiac pacemakers are devices either implanted permanently or inserted temporarily, consisting of a

pulse generator and an electrode catheter that is placed transvenously into the right ventricle and/or

atrium. Small electrical impulses, generated by the pulse generator and delivered via the electrode

catheter depolarize the heart. Pacemakers are widely used for treating bradyarrhythmias but can also be

useful for treatment of some tachyarrhythmias.

Temporary pacing is indicated for symptomatic second or third degree heart block caused by transient

drug intoxication or electrolyte imbalance in the setting of an acute MI, CHB, or Mobitz II second

degree AV Block. Symptomatic sinus bradycardia, AF with a slow ventricular response.

Indications for permanent pacemaker implantation:

Symptomatic bradycardia, due to either sinus node dysfunction or AV nodal block, in absence of a

reversible cause, constitutes a class I indications for permanent pacing. Asymptomatic conditions that

are also considered class I indications for permanent pacing include:

1- 3rd degree AV Block .

2- Persistent advanced 2nd degree or 3rd degree AVB after acute MI with demonstrated block in His-

Purkinje system (BBB).

3- Chronic bifascicular or trifascicular block with intermittent type II second or third degree AV

Block.

Pacing modalities: a four-letter alphabetic code is used to identify pacing modalities. The first initial

defines the chamber that is paced (V: ventricle, A: atrium, D: dual chamber). The second identifies the

chamber that is sensed (V, A, D), the third indicates the response to sensed event (I: inhibited, T:

triggered, D: dual function), and the fourth when present, denotes, R: rate responsive node. VVI &

DDD modes are used most commonly. VVI units pace and sense ventricle and a sensed (native) event

inhibits the ventricular stimulus. DDD units, pace and sense both chambers, events sensed in the

atrium inhibit the atrial stimulus and trigger a ventricular response after an appropriate interval,

where as ventricle-sensed events inhibit ventricular and atrial outputs.

63

Antiarrhythmic Drugs

Class Mode of

Action

Drugs Indication Dose Side Effects

Class

IA

Reduces

rate of

entry of

sodium

into the

cell

Quinidine

(Quinidine)

For

supraventricular

and ventricular

arrhythmias

including

conversion of AF or

A flutter, SVT, VT

600 – 1000

mg/day

Prolongation of QT

interval, risk of

Torsade de pointes.

Quinidine syncope,

quinidine induced

sudden death.

Diarrhea, vomiting

Procainamide

(Pronestyl)

Is effective

against

supraventricular

and ventricular

arrhythmias

2-6

mg/min

IV. 350-

1000 mg q

6 h PO

SLE like

syndrome,

prolonged QT,

nausea, rash,

myalgia,

Disopyramid

e (Norpace)

Is effective

against

supraventricular

and ventricular

arrhythmias

100-400

mg q 8 h

Worsening of

heart failure,

anticholinergic

actions as urine

retention. Avoid

in pts with

glaucoma

Class

IB

Lidocaine

(Zylocain)

Ventricular

arrhythmias only

1-4 mg/min

IV (50-150

mg IV

loading

dose)

Confusion,

convulsions

Mexiletine

(Mexitil)

Ventricular

arrhythmias only

150-300

mg q 6-8 h

Confusion,

tremor,

bradycardia,

hypotension

Class

IC

Flecainide

(Tambocor)

Is very effective

for ventricular

and

supraventricular

tachycardias

100-200

mg q 12 h

PO

Aggravation of

arrhythmia

(proarrhythmia),

negative inotropic

effect, depression

of sinus node

64

Propafenone

(Rytmonorm)

Has a rule in

treatment of many

types of

arrhythmias

including

supraventricular

arrhythmias

150-300 mg

q 8-12 h

Negative inotropic

effect

Class

II

Beta

adrenergi

c

blockers

e.g.

Propranolol

(Inderal),

Atenolol,

Bisoprolol,

Carvedilol

For premature

beats atrial and

ventricular, for

torsade de

pointes,

10-200 mg

q 8 h PO

Bradycardia,

hypotension, heart

failure, intermittent

claudication,

worsening of

asthma, impotence

Class

III

Prolong

action

potential

duration

Amiodarone

(Cordarone)

Life-threatening

ventricular

arrhythmias,

conversion and

slowing of atrial

fibrillation,

AVNRT,

tachycardias

associated with

WPWs

200-400

mg q 6-8 h

Corneal deposits,

photosensitivity,

skin pigmentation,

thyroid

disturbances (hypo

& hyperfunction),

alveolitis, liver

enzyme elevation

Sotalol

(Betacor)

Effective in

supraventricular

and ventricular

arrhythmias

80-160 mg

x 2-3 PO

Torsade de

pointes,

bronchospasm in

asthmatic patients

Class

IV

Calcium

antagonis

ts

Verapamil

(Isoptin)

Diltiazem

Slow the

ventricular rate in

AF or flutter, treat

and prevent

AVNRT

0.1 Mg/kg

IV 40-160

mg q 6-8 h

PO

60-120 mg

q 6-8 h PO

Constipation,

edema of LL,

negative inotropic

effect

Unclas

sified

Activates

K+

channels

Adenosine

(Adenocore)

Is very effective

for the acute

conversion of

paroxysmal SVT

6-18 mg

IV rapidly

Contraindicated

in sick sinus s., or

2nd

or 3rd˚

heart

block. Antidote is

theophylline

Enhances

central

and

Digoxin

(Lanoxin)

Slow ventricular

rate in AF, flutter

0.5 – 1 mg

IV or

0.125 –

Bradycardias and

tachycardias

(atrial, junctional,

65

periphera

l vagal

tone

0.25 mg /d

PO

vent.

tachycardia),

nausea, vomiting

Note: there are new two important antiarrhythmic drugs: Dronedarone (Multaq), and Vernakalant.

Principles of Cardiology pages 1-61

ELECTROCARDIOGRAPHY

Prof. Samir Rafla

The electrocardiogram (ECG) is a graphic representation of the electrical activity generated by the

heart during the cardiac cycle. The electrical activity starts from the SA node, bundle of His, right and

left bundles, Purkinje fibers to stimulate the ventricles.

Waveforms: The waveforms and intervals of the ECG are: The P wave = atrial depolarization. The

QRS complex = ventricular depolarization. The Q wave is the initial downward deflection, the R wave

is the initial upward deflection, and the S wave is the second downward deflection. The interval from

the beginning of the P wave to the beginning of the Q wave is the PR interval.

The T wave = ventricular repolarization. The interval from the end of ventricular depolarization to the

beginning of the T wave is termed the ST segment. The interval from the onset of ventricular

depolarization to end of T is the QT interval.

STANDARD APPROACH TO THE ECG: Normally, standardization is 1.0 mV per 10 mm, and

paper speed is 25 mm/s (each horizontal small box = 0.04 sec)

Heart Rate: divide 1500 by number of small boxes between each QRS.

Rhythm: Sinus rhythm is present if every P wave is followed by a QRS, PR interval > 0.12 s, and the P

wave is upright in leads I, II, and III.

Intervals: PR (0.12 - 0.20 s). QRS (0.06 - 0.10 s).

QT 0.43 s;

ST-T WAVES: ST elevation : Acute MI, coronary spasm, pericarditis (concave upward), LV

aneurysm.

ST depression: Digitalis effect, strain (due to ventricular hypertrophy), ischemia, or nontransmural MI.

Tall peaked T: Hyperkalemia; acute MI ("hyperacute T").

66

Inverted T: Non-Q-wave MI, ventricular "strain" pattern, drug effect (e.g., digitalis), hypokalemia,

hypocalcemia, increased intracranial pressure (e.g., subarachnoid bleeding).

FIG: The magnified ECG wave is presented with the principal time intervals indicated.

67

Fig: The pathways of Conduction.

RHEUMATIC FEVER

Introduction. Classified as a connective tissue or collagen vascular disease, rheumatic fever (RF) is

the leading cause of acquired heart disease in children and young adults.

a. In many developing countries the incidence of acute RF approaches or exceeds 100 per 100.000,

whereas in the Unites States it is estimated to be less than 2 per 100.000.

b. Rheumatic fever is more common among population at high risk for streptococcal pharyngitis, those

in close contact with school age children, and persons of low socioeconomic status. It occurs

commonly between the ages of 5 and 18 years and is rare before 5. Rheumatic fever affects both sexes

equally, except for Sydenham’s chorea, which is more prevalent in females after puberty.

The clinical manifestations of RF develop after a silent period of approximately 3 weeks following a

tonsillopharyngitis caused by a group A streptococcal infection (GAS).

Diagnostic criteria

2. The Jones criteria, are designed to aid in the diagnosis of the first episode of RF. Rheumatic

fever can be diagnosed when a previous upper airway infection with GA-Streptococci is detected in

conjunction with either two major manifestations, or one major and two minor manifestations. Major

manifestation includes arthritis, carditis, chorea, erythema marginatum, and subcutaneous nodules.

68

Minor manifestations include: fever, arthralgias, history of tonsillitis 1-3 weeks before the arthralgia,

history of rheumatic heart disease;

high C-reactive protein, high erythrocyte sedimentation rate, raised antistreptolysin O titer above 200

Todd’s units or prolonged PR interval on electrocardiogram (ECG).

Major manifestations:

1. Carditis: affecting 41% to 83% of patients. It can be defined as pancarditis affecting the

endocardium, myocardium, and pericardium: The main clinical manifestations include increased heart

rate, murmurs, cardiomegaly, rhythm disturbances, pericardial friction rub, and heart failure.

Congestive heart failure is rare in the acute phase; if present, it usually results from myocarditis. The

most characteristic component of rheumatic carditis is a valvulitis (endocarditis) involving the mitral

and aortic valves.

Pericarditis may cause chest pain, friction rubs, and distant heart sounds.

2. Arthritis. This is the most common manifestation of RF. It is present in around 80% of the patients

and has been described as painful, asymmetric, migratory, and transient; it involves large joints, such as

knees, ankles, elbows, wrists, and shoulders. It improves markedly with the use of salicylates within 48

hours of treatment. Monoarthritis, oligoarthritis, and involvement of small joints of the extremities are

less common. The arthritis of RF is benign and self- limiting (Lasting 2 to 3 weeks) and does not result

in permanent sequelae.

3. Sydenham’s chorea. This extrapyramidal disorder is characterized by purposeless and involuntary

movements of face and limbs, muscular hypotonia, and emotional lability.

4. Subcutaneous nodules.

5. Erythema marginatum.

Minor manifestations:

1. Fever is encountered during the acute phase of the disease.

2. Arthralgia is defined as pain in one or more large joints without objective findings of inflammation

on physical examination.

3. Other clinical manifestations of RF include abdominal pain, epistaxis, acute glomerulonephritis.

These are not included as diagnostic criteria for the diagnosis of RF.

Laboratory examination and diagnostic testing.

69

1. Neither throat culture nor rapid antigen test, if positive; differentiate

between recent infection associated with RF and chronic carriage of

pharyngeal GAS.

2. Antistreptolysin O is the most commonly available test. Elevated or rising ASO titers provide solid

evidence for recent GAS infection. A greater than two-fold rise in ASO titers compared with

convalescent titers is diagnostic.

3. Increased sedimentation rate.

4. Increased C reactive protein CRP/

5. The most common finding in the electrocardiogram is the presence of P-R prolongation and sinus

tachycardia.

Therapy:

Patient with the diagnosis of rheumatic activity should initially receive a full course of antibiotic to

ensure proper eradication of the organism.

A. Arthritis: Anti-inflammatory medications are generally recommended for 3 weeks for symptomatic

relief.

4. Pain resolves within 24 hours of starting therapy with salicylates.

5. If pain persists after salicylate treatment, the diagnosis of RF is questionable.

6. The recommended dose of salicylate is 100 mg/kg per day, given in 4 divided doses. Toxic effects

such as anorexia, nausea, vomiting, and tinnitus should be avoided.

B. Carditis

5. Strenuous physical activity should be avoided.

6. Congestive heart failure should be treated with appropriate therapy.

7. In patients with significant cardiac involvement, corticosteroids are preferred over salicylates. The

recommended dose is 1 to 2 mg/kg per day, (maximum of 60 mg/day as Prednisolone). Commonly,

therapy is needed for more than one month in patients with cardiac involvement. Therapy should be

continued until there is sufficient clinical and laboratory evidence of disease inactivity.

8. The gradual reduction in steroid doses is important to avoid relapses. Use of salicylates (75 mg/kg

per day) while tapering corticosteroids may reduce the likelihood a relapse.

Summary: Jones Criteria of Rheumatic Fever

Major Criteria Minor Criteria

Migratory polyarthritis Fever

70

Carditis Arthralgia

Chorea High sedimentation rate

Subcutaneous nodules Positive C reactive protein

Erythema Marginatum Prolonged PR interval

Prevention:

The most important step in the treatment of RF is the eradication of GAS infection.

Penicillin is the agent of choice. A. best results are achieved with a single intramuscular dose of

penicillin G benzathine. b. The oral antibiotic of choice is penicillin V (phenoxymethyl penicillin) (see

Table for dosage information). Patients allergic to penicillin: oral erythromycin can be used. The

recommended dosage is erythromycin for 10 days. The maximal dose of erythromycin is 1 g/day.

Table: Duration of therapy for secondary prevention of rheumatic fever

Disease state Duration of therapy

RF + carditis + residual valvular

disease

At least 10 years post episode and at least

until age 40. Lifelong prophylaxis may be

required

RF + carditis without valvular

disease

10 years or beyond adulthood, whichever

is longer.

RF without carditis 5 years or until age of 21, whichever is

longer.

RF, rheumatic fever.

VALVULAR HEART DISEASE

MITRAL STENOSIS

ETIOLOGY AND PATHOLOGY: Two-thirds of all patients with mitral stenosis (MS) are females.

MS is generally rheumatic in origin. Pure or predominant MS occurs in approximately 40% of all

patients with rheumatic heart disease. The valve leaflets are diffusely thickened by fibrous tissue and/or

calcific deposits. The mitral commissures fuse, the chordae tendineae fuse and shorten. The valvular

71

cusps become rigid, and these changes in turn, lead to narrowing at the apex of the funnel-shaped

valve.

Other rare causes of mitral stenosis: Atrial myxoma, ball valve thrombus, congenital and calcific-

atherosclerortic disease.

PATHOPHYSIOLOGY: In normal adults the mitral valve orifice is 4 to 6 cm2. When the mitral valve

opening is reduced to 1 cm2, a left atrial pressure of approximately 25 mmHg is required to maintain a

normal cardiac output. The elevated left atrial pressure, in turn, raises pulmonary venous and capillary

pressures, reducing pulmonary compliance and causing exertional dyspnea.

Pulmonary hypertension results from (1) the passive backward transmission of the elevated left atrial

pressure, (2) pulmonary arteriolar constriction, (reactive pulmonary hypertension), and (3) organic

obliterative changes in the pulmonary vascular bed. In time, the resultant severe pulmonary

hypertension results in tricuspid and pulmonary incompetence as well as right-sided heart failure.

SYMPTOMS AND COMPLICATIONS: - Dyspnea, hemoptysis. - Orthopnea and paroxysmal

nocturnal dyspnea. Pulmonary edema develops when there is a sudden surge in flow across a markedly

narrowed mitral orifice.

72

The cardiac cycle: Simultaneous electrocardiogram and pressure obtained from the left atrium, left

ventricle, and aorta, and the jugular pulse during one cardiac cycle.

When moderately severe MS has existed for several years, atrial arrhythmias as flutter and fibrillation

occur.

Hemoptysis results from rupture of pulmonary-bronchial venous connections (apoplexy) secondary to

pulmonary venous hypertension. Frank hemoptysis must be distinguished from the bloody sputum that

occurs with pulmonary edema, pulmonary infarction, and bronchitis, three conditions that occur with

increased frequency in the presence of MS.

Recurrent pulmonary emboli, sometimes with infarction are an important cause of morbidity and

mortality late in the course of MS, occurring most frequently in patients with right ventricular failure.

Pulmonary infections, i.e., bronchitis, broncho-pneumonia, and lobar pneumonia, commonly

complicate untreated MS. Infective endocarditis is rare in pure MS but is not uncommon in patients

with combined stenosis and regurgitation.

Summary: Causes of hemoptysis in mitral stenosis:

- Bronchitis

- Congestion

- Pulmonary edema

- Pulmonary embolism, infarction

- Pulmonary apoplexy

Thrombi and emboli: Thrombi may form in the left atrium, particularly in the enlarged atrial

appendage of patients with MS. If they embolize, they do so most commonly to the brain, kidneys,

spleen, and extremities. Embolization occurs much more frequently in patients with atrial fibrillation.

Rarely, a large pedunculated thrombus or a free-floating clot may suddenly obstruct the stenotic mitral

orifice. Such “ball valve” thrombi produce syncope, angina, and changing auscultatory signs with

alterations in position, findings that resemble those produced by a left atrial myxoma.

73

PHYSICAL FINDINGS: Inspection: In advanced cases there is a malar flush. When fibrillation is

present, the jugular pulse reveals only a single expansion during systole (c-v wave) (systolic venous

pulse).

Palpation: Left parasternal lift along the left sternal border signifies an enlarged right ventricle. In

patients with pulmonary hypertension, the impact of pulmonary valve closure can usually be felt in the

second and third left intercostal spaces just left of the sternum (Diastolic shock). A diastolic thrill is

frequently present at the cardiac apex, particularly if the patient is turned into the left lateral position.

Auscultation: The first heart sound (S1) is generally accentuated and snapping. In patients with

pulmonary hypertension, the pulmonary component of the second heart sound (P2) is often accentuated,

and the two components of the second heart sound are closely split. The opening snap (OS) of the

mitral valve is most readily audible in expiration at, or just medial to, the cardiac apex but also may be

easily heard along the left sternal edge. This sound generally follows the sound of aortic valve closure

(A2) by 0.05 to 0.12; that is, it follows P2; the time interval between A2 closure and OS varies inversely

with the severity of the MS. It tends to be short (0.05 to 0.07 s) in patients with severe obstruction, and

long, (0.10 to 0.12 s) in patients with mild MS. The intensities of the OS and S1 correlate with mobility

of the anterior mitral leaflet.

The OS usually precedes a low-pitched, rumbling, diastolic murmur, heard best at the apex with the

patient in the left lateral recumbent position. In general, the duration of the murmur correlates with the

severity of the stenosis. In patients with sinus rhythm, murmur often reappears or becomes accentuated

during atrial systole, as atrial contraction elevates the rate of blood flow across the narrowed orifice

(presystolic accentuation).

Associated lesion: With severe pulmonary hypertension, a pansystolic murmur produced by

functional tricuspid regurgitation may be audible along the left sternal border. Characteristically, this

murmur is accentuated by inspiration, and should not be confused with the apical pansystolic murmur

of mitral regurgitation.

In the presence of severe pulmonary hypertension and right ventricular failure, a third heart sound

may originate from the right ventricle. The enlarged right ventricle may rotate the heart in a clockwise

direction and form the cardiac apex, giving the examiner the erroneous impression of left ventricular

enlargement. Under these circumstances, the rumbling diastolic murmur and the other auscultatory

features of MS become less prominent or may even disappear and be replaced by the systolic murmur

74

of functional tricuspid regurgitation which is mistaken for mitral regurgitation. When cardiac output is

markedly reduced in a patient with MS, the typical auscultatory findings, including the diastolic

rumbling murmur, may not be detectable (silent MS).

ECG findings: The P wave is wide and may be notched which suggests left atrial enlargement. It

becomes tall and peaked in lead II and upright in lead V1 when severe pulmonary hypertension.

75

Echocardiogram: Two-dimensional echo-Doppler echocardiography for estimation of the

transvalvular gradient and of mitral orifice size, the presence and severity of accompanying mitral

regurgitation, the extent of restriction of valve leaflets, their thickness, and the subvalvular changes.

Transthoracic and transesophageal echo are needed to verify presence of atrial thrombi.

X-Ray chest: Straightening of the left border of the cardiac silhouette, prominence of the main

pulmonary arteries, dilatation of the upper lobe pulmonary veins, and backward displacement of the

esophagus by an enlarged left atrium.

Summary of signs of mitral stenosis:

- Mid-diastolic rumbling murmur with presystolic accentuation;

- Snappy first sound;

- Opening snap;

- Diastolic thrill.

DIFFERENTIAL DIAGNOSIS: The apical middiastolic murmur associated with aortic regurgitation

(Austin Flint murmur) may be mistaken for MS. However, in a patient with aortic regurgitation, the

absence of an opening snap or presystolic accentuation if sinus rhythm is present points to the absence

of MS.

Tricuspid stenosis, a valvular lesion that occurs very rarely in the absence of MS, may mask many of

the clinical features of MS.

MANAGEMENT: Penicillin prophylaxis of beta-hemolytic streptococcal infections and prophylaxis

for infective endocarditis are important. In symptomatic patients, some improvement usually occurs

with restriction of sodium intake and maintenance doses of oral diuretics. Digitalis glycosides usually

do not benefit patients with pure stenosis and sinus rhythm, but they are necessary for slowing the

ventricular rate of patients with atrial fibrillation and for reducing the manifestations of right-sided

heart failure in the advanced stages of the disease.

Small doses of beta-blockers (e.g., atenolol 25 mg/d) may be added when cardiac glycosides fail to

control ventricular rate in patients with atrial fibrillation. Particular attention should be directed toward

detecting and treating any accompanying anemia and infections. Hemoptysis is treated by measures

designed to diminish pulmonary venous pressure, including bed rest, the sitting position, salt

76

restriction, and diuresis. Anticoagulants should be administered continuously in those with atrial

fibrillation.

If atrial fibrillation is of relatively recent origin in a patient who’s MS is not severe enough to warrant

surgical treatment, reversion to sinus rhythm pharmacologically or by means of electrical countershock

is indicated. Usually this should be undertaken following 3 weeks of anticoagulant treatment.

Conversion to sinus rhythm is rarely helpful in patients with severe MS, particularly those in whom the

left atrium is especially enlarged or in whom atrial fibrillation is chronic.

Mitral valvotomy by balloon or surgical mitral valvotomy, is indicated in the symptomatic patient with

pure MS whose effective orifice is less than approximately 1.3 cm2 (or 0.8 cm

2 / m

2 of body surface

area). Mitral valve replacement by prosthetic valve is resorted to only if the valve is heavily calcified

and associated with incompetence.

Percutaneous balloon valvuloplasty is an alternative to surgical mitral valvuloplasty in patients with

pure or predominant rheumatic stenosis (it is now the first choice). Young patients without extensive

valvular calcification or thickening or subvalvular deformity are the best candidates for this procedure.

Contraindications of balloon mitral valvotomy:

1. presence of left atrial thrombi,

2. presence of combined mitral incompetence and stenosis, and

3. heavily calcified mitral cusps.

MITRAL REGURGITATION

ETIOLOGY:

8- Chronic rheumatic heart disease is the cause of severe mitral regurgitation (MR).

9- MR also may occur as a congenital anomaly.

10- MR may occur in patients with infarction involving the base of a papillary muscle.

11- MR may occur with marked left ventricular dilatation.

12- Massive calcification of the mitral annulus of unknown cause, presumably degenerative, which

occurs most commonly in elderly women.

77

13- Systemic lupus erythematosus, rheumatoid arthritis, are less common cause.

14- Mitral prolapse.

Acute MR occur 1- secondary to infective endocarditis involving the cusps or chordae tendineae, 2- in

acute myocardial infarction with rupture of a papillary muscle or one of its heads, 3- as a consequence

of trauma, 4- or following apparently spontaneous chordal rupture.

MITRAL REGURGITATION: SYMPTOMS: Fatigue, exertional dyspnea, and orthopnea are the

most prominent complaints in patients with chronic, severe MR. Hemoptysis and systemic

embolism also occur less frequently in MR than in MS. Right-sided heart failure, with painful

hepatic congestion, ankle edema, distended neck veins, ascites, and tricuspid regurgitation,

may be observed in patients with MR who have associated pulmonary vascular disease and

marked pulmonary hypertension. In patients with acute, severe MR, left ventricular failure

with acute pulmonary edema and /or cardiovascular collapse is common.

PHYSICAL FINDINGS: Palpation: A systolic thrill is often palpable at the cardiac apex, the left

ventricle is hyperdynamic, and the apex beat is often displaced laterally. Auscultation: The first heart

sound is generally absent, soft (muffled), or buried in the systolic murmur. A low-pitched third heart

sound (S3) occurring 0.12 to 0.17 sec after aortic valve closure, i.e. at the completion of the rapid-filling

phase of the left ventricle, is an important auscultatory feature of severe MR.

A fourth heart sound is often audible in patients with acute, severe MR of recent onset who are in sinus

rhythm. A systolic murmur of at least grade III/VI intensity is the most characteristic auscultatory

finding in severe MR. It is usually holosystolic (pansystolic). In MR due to papillary muscle

dysfunction or mitral valve prolapse, the systolic murmur commences in midsystole. In patients with

ruptured chordae tendineae the systolic murmur may have a cooing or “sea gull” quality; in patients

with a flail leaflet the murmur may have a musical quality.

Summary: Signs of mitral incompetence:

- Harsh pansystolic murmur over apex propagated to axilla.

- Muffled first heart sound.

- Systolic thrill over apex.

78

Electrocardiogram: In patients with sinus rhythm there is evidence of left atrial enlargement (P

mitrale), but right atrial enlargement also may be present when pulmonary hypertension is severe.

Chronic, severe MR with left atrial enlargement is generally associated with atrial fibrillation.

Echocardiogram: Doppler echocardiography and color Doppler flow echocardiography imaging are the

most accurate noninvasive techniques for the detection and estimation of MR. The left atrium is usually

enlarged. Findings which help to determine the etiology of MR can often be identified; these include

vegetations associated with infective endocarditis, incomplete coaptation of the anterior and posterior

mitral leaflets, and annular calcification, as well as left ventricular dilation, aneurysm, or dyskinesia.

The echocardiogram in patients with mitral valve prolapse is described below.

Roentgenogram: The left atrium and left ventricle are the dominant chambers; in chronic cases, the

former may be massively enlarged and forms the right border of the cardiac silhouette. Pulmonary

venous congestion, interstitial edema, and Kerly B lines are sometimes noted.

TREATMENT: Medical: The non surgical management of MR is directed toward restricting those

physical activities that regularly produce dyspnea and excessive fatigue, reducing sodium intake, and

enhancing sodium excretion with the appropriate use of diuretics. Vasodilators and digitalis glycosides

increase the forward output of the failing left ventricle. Angiotensin-converting enzyme inhibitors are

given in chronic MR. The same considerations as in patients with MS apply to the reversion of atrial

fibrillation to sinus rhythm. Surgical treatment should be offered to patients with severe MR whose

limitations do not allow them to perform normal household activities despite optimal medical

management. Surgery is indicated when the end systolic diameter of the left ventricle by echo exceeds

50 mm.

MITRAL VALVE PROLAPSE

Mitral valve prolapse (MVP), also termed the systolic click-murmur syndrome, is a common, but

highly variable, clinical syndrome. It is a frequent finding in patients who have the typical features of

the Marfan syndrome. The posterior leaflet is usually more affected than the anterior, and the mitral

valve annulus is often greatly dilated.

MVP may be associated with thoracic skeletal deformities.

79

MVP is common in females between the ages of 6 and 30 years. Most patients are asymptomatic and

remain so for their entire lives. Arrhythmia, most commonly ventricular premature contractions and

paroxysmal supraventricular and ventricular tachycardia, have been reported and may cause

palpitations, light-headedness, and syncope. Many patients have chest pain which is difficult to

evaluate.

PHYSICAL EXAMINATION: Auscultation: the most important finding is the mid-or late

(nonejection) systolic click, which occurs 0.14 s or more after the first heart sound. Systolic clicks may

be followed by a high-pitched late systolic murmur, heard best at the apex. A useful echocardiographic

definition of MVP is systolic displacement (in the parasternal view) of the mitral valve leaflets into the

left atrium > 3 mm. Thickening of the mitral valve leaflets is present. Doppler studies are helpful in

revealing and evaluating accompanying MR.

Treatment: The management of patients with MVP consists of reassurance of the asymptomatic

patient without severe MR or arrhythmias; prevention of infective endocarditis with antibiotic

prophylaxis in patients with a systolic murmur and the relief of the atypical chest pain by beta blockers.

AORTIC STENOSIS

Aortic stenosis (AS) occurs in one-fourth of all patients with chronic valvular heart disease;

approximately 80 percent of adult patients with symptomatic valvular AS are male.

Etiology: 1. AS may be congenital in origin, 2. secondary to rheumatic inflammation of the valve, 3.

degenerative calcification of the aortic cusps of unknown cause.

PATHOPHYSIOLOGY: A peak systolic pressure gradient exceeding 50 mmHg or an effective aortic

orifice less than approximately 0.5 cm2/m

2 of body surface area i.e., less than approximately one-third

of the normal orifice, is generally considered to represent critical obstruction to left ventricular outflow.

SYMPTOMS: AS is rarely of hemodynamic or clinical importance until the valve orifice has narrowed

to approximately one-third of normal, i.e., to 1 cm2 in adults.

Exertional dyspnea, angina pectoris, and syncope are the three cardinal symptoms. Angina pectoris

reflects an imbalance between the augmented myocardial oxygen requirement by the hypertrophied

myocardium and the un-accompanying increase in coronary blood flow. Orthopnea, paroxysmal

nocturnal dyspnea, and pulmonary edema, i.e., symptoms of left ventricular failure, also occur only in

the advanced stages of the disease.

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PHYSICAL FINDINGS: A palpable double systolic arterial pulse the so-called bisferiens pulse,

excludes pure or predominant AS and signifies dominant or pure aortic regurgitation or obstructive

hypertrophic cardiomyopathy.

Palpation: The apex beat is usually sustained and displaced laterally, reflecting the presence of left

ventricular hypertrophy. A systolic thrill is generally present at the base of the heart in the suprasternal

notch, and along the carotid arteries.

Auscultation: Harsh ejection systolic murmur over aortic area propagated to carotids. The sound of

aortic valve closure, the second sound is very weak or even absent with tight aortic stenosis.

Frequently, a fourth heart sound is audible at the apex in many patients with severe AS and reflects

the presence of left ventricular hypertrophy and an elevated left ventricular enddiastolic pressure; a

third heart sound generally occurs when the left ventricle dilates and fails.

The murmur of AS is characteristically an ejection systolic murmur loudest at the base of the

heart, most commonly in the second right intercostal space. It is transmitted along the carotid arteries.

Occasionally, it is transmitted downward and to the apex and may be confused with the systolic

murmur of MR.

Summary: Signs of aortic stenosis:

5. Harsh ejection systolic murmur over aortic area propagated to carotids.

6. Weak or absent second heart sound (aortic component)

7. Systolic thrill over aortic area, suprasternal notch and carotids.

8. Strong sustained apex,

Electrocardiogram: This reveals left ventricular hypertrophy in the majority of patients with severs

AS.

Echocardiogram: The key findings are left ventricular hypertrophy. The transaortic valvular gradient

can be estimated by Doppler echocardiography.

Congestive heart failure was considered to be the cause of death in one-half to two-thirds of patients.

Among adults dying with valvular AS sudden death, which presumably results from an arrhythmia

(ventricular tachycardia or fibrillation) occurred in 10 to 20 percent and at an average age of 60 years.

TREATMENT: All patients with moderate or severe AS require careful periodic follow-up. In

patients with severe AS, strenuous physical activity should be avoided even in the asymptomatic stage.

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Digitalis glycosides, sodium restriction, and the cautious administration of diuretics are indicated in the

treatment of congestive heart failure, but care must be taken to avoid volume depletion.

In the majority of adults with calcific AS and critical obstruction, replacement of the valve is necessary.

Percutaneous balloon aortic valvuloplasty is an alternative to surgery in children and young adults with

congenital aortic stenosis. It is not commonly employed in elderly with severe calcific aortic stenosis

because of a high restenosis rate.

Electrocardiogram (ECG), left ventricular, and aortic pressure curves in a patient with aortic stenosis.

There is a pressure gradient across the aortic valve during systole

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Fig. Abnormal sounds and murmurs associated with valvular dysfunction displayed simultaneously

with left atrial (LA), left ventricular (LV), and aortic pressure tracings. AVO, aortic valve opening; E,

ejection click; MVO, mitral valve opening; OS, opening snap of the mitral valve.

.

AORTIC REGURGITATION

ETIOLOGY: Approximately three-fourths of patients with pure or predominant aortic regurgitation

(AR) are males; females predominate among patients with AR who have associated mitral valve

disease.

Causes:

1- In approximately two-thirds of patients with AR the disease is rheumatic in origin, resulting in

thickening, deformation and shortening of the individual aortic valve cusps, changes which prevent

their proper opening during systole and closure during diastole.

2- Acute AR also may result from infective endocarditis, which may attack a valve previously affected

by rheumatic disease, a congenitally deformed valve, or rarely a normal aortic valve, and perforate or

erode one or more of the leaflets.

3- Patients with discrete membranous subaortic stenosis often develop thickening of the aortic valve

leaflets, which in turn leads to mild or moderate degrees of AR.

4- AR also may occur in patients with congenital bicuspid aortic valves.

5- Aortic dilatation, i.e., aortic root disease, widening of the aortic annulus and separation of the aortic

leaflets are responsible for the AR.

6- Syphilis and ankylosing rheumatoid spondylitis may lead to aortic dilatation, aneurysm formation,

and severe regurgitation.

7- Cystic medial necrosis of the ascending aorta, associated with other manifestations of the Marfan

syndrome, idiopathic dilatation of the aorta, and severe hypertension all may widen the aortic annulus

and lead to progressive AR.

8- Occasionally, AR is caused by retrograde dissection of the aorta involving the aortic annulus.

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History: Patients with severe AR may remain asymptomatic for 10 to 15 years.

Sinus tachycardia during exertion may produce particularly uncomfortable palpitations. Exertional

dyspnea is the first symptom of diminished cardiac reserve. This is followed by orthopnea, paroxysmal

nocturnal dyspnea, and excessive diaphoresis. Chest pain occurs frequently, even in younger patients,

due to diminished coronary filling during diastole.

Nocturnal angina may be a particularly troublesome symptom. The anginal episodes can be prolonged

and often do not respond satisfactorily to sublingual nitroglycerin. Late in the course of the disease,

evidence of systemic fluid accumulation, including congestive hepatomegaly, ankle edema, and ascites,

may develop.

PHYSICAL FINDINGS: Peripheral signs: Arterial pulse: A rapidly rising “water-hammer” pulse,

which collapses suddenly as arterial pressure falls rapidly during late systole and diastole, and capillary

pulsations, an alternate flushing and paling of the root of the nail while pressure is applied to the tip of

nail, are characteristic of free AR. A booming, “pistol-shot” sound can be heard over the femoral or

brachial arteries, and a to - fro murmur is audible if the femoral artery is lightly compressed with a

stethoscope.

The arterial pulse pressure is widened, with an elevation of the systolic pressure and a depression of the

diastolic pressure. The severity of AR does not always correlate directly with the arterial pulse

pressure, and severe regurgitation may exist in patients with arterial pressures in the range of 140/60.

Palpation: The apex beat is strong and displaced laterally and inferiorly. The systolic expansion and

diastolic retraction of the apex are prominent and contrast sharply with the sustained systolic thrust

characteristic of severe AS. In many patients with pure AR or with combined AS and AR, palpation or

recording of the carotid arterial pulse reveals it to be bisferiens, i.e., with two systolic waves separated

by trough.

Auscultation: A third heart sound is common, and occasionally, a fourth heart sound also may be heard.

The murmur of AR is typically a high-pitched, blowing, decrescendo early diastolic murmur which is

usually heard best in the third left intercostal space. Unless it is trivial in magnitude, the AR is usually

accompanied by peripheral signs such as a widened pulse pressure or a collapsing pulse. On the other

hand, with the Graham steel murmur of pulmonary regurgitation, there is usually clinical evidence of

severe pulmonary hypertension, including a loud and palpable pulmonary component to the second

heart sound.

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A midsystolic ejection murmur is frequently audible in AR. It is generally heard best at the base of

the heart and is transmitted to the carotid vessels. This murmur may be quite loud without signifying

organic obstruction; it is often higher pitched, shorter, than the ejection systolic murmur heard in

patients with predominant AS.

A third murmur which is frequently heard in patients with AR is the Austin Flint murmur, a soft,

low-pitched, rumbling middiastolic or presystolic bruit. It is probably produced by the displacement of

the anterior leaflet of the mitral valve by the aortic regurgitant stream. Both the Austin Flint murmur

and the rumbling diastolic murmur of MS are loudest at the apex, but the murmur of MS is usually

accompanied by a loud first heart sound and immediately follows the opening snap of the mitral valve,

while the Austin Flint murmur is often shorter in duration than the murmur of MS, and in patients with

sinus rhythm the latter exhibits presystolic accentuation.

Summary: Signs of aortic incompetence over the heart:

- Soft blowing early diastolic murmur over aortic area propagated to apex.

- Austin-Flint murmur (diastolic murmur over mitral area).

Echocardiogram: Essential for detection of severity and cause of AR.

TREATMENT: Although operation constitutes the principal treatment of aortic regurgitation, and

should be carried out before the development of heart failure, the latter usually respond initially to

treatment with digitalis, salt restriction, diuretics, and vasodilators, especially angiotensin-converting

enzyme inhibitors.

In patients with severe AR, careful clinical follow-up and noninvasive testing with echocardiography at

approximately 6-month intervals are necessary. Operation is to be undertaken at the optimal time, i.e.,

after the onset of left ventricular dysfunction but prior to the development of severe symptoms. Valve

replacement is indicated if the LV dilates to 50 mm in systole and 65 to 70 mm in diastole.

ACUTE AORTIC REGURGITATION: Infective endocarditis, aortic dissection, and trauma are the

most common causes of severe, acute AR.

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TRICUSPID STENOSIS

It is generally rheumatic in origin and is more common in women than in men. It does not usually

occur as an isolated lesion or in patients with pure MR but is usually observed in association with MS.

Hemodynamically significant TS occurs in 5 to 10 percent of patients with severe MS; rheumatic TS is

commonly associated with some degree of regurgitation.

SYMPTOMS: Since the development of MS generally precedes that of TS, many patients initially have

symptoms of pulmonary congestion. Amelioration of the latter should raise the possibility that TS may

be developing. Fatigue secondary to a low cardiac output and discomfort due to refractory edema,

ascites, and marked hepatomegaly are common in patients with TS and / or regurgitation.

Severe TS is associated with marked hepatic congestion, often resulting in cirrhosis, jaundice,

serious malnutrition, anasarca, and ascites. The jugular veins are distended, and in patients with sinus

rhythm there may be giant “a” waves.

On auscultation, the pulmonic closure sound is not accentuated, and occasionally, an OS of the

tricuspid valve may be heard approximately 0.06 s after pulmonic valve closure. The diastolic murmur

of TS has many of the quality of the diastolic murmur of MS, and since TS almost always occurs in the

presence of MS, the less common valvular lesion may be missed. The murmur is augmented during

inspiration, and it is reduced during expiration.

Surgical treatment of the tricuspid valve is not ordinarily indicated at the time of mitral valve

surgery in patients with mild TS. On the other hand, definitive surgical relief of the TS should be

carried out, preferable a the time of mitral valvotomy, in patients with moderate or severe TS who have

mean diastolic pressure gradients exceeding 4 to 5 mmHg and tricuspid orifices less than 1.5 to 2.0

cm2. TS is almost always accompanied by significant tricuspid regurgitation.

TRICUSPID REGURITATION

Most commonly, tricuspid regurgitation (TR) is functional and secondary to marked dilatation of

the right ventricle and the tricuspid annulus. Functional TR may complicate right ventricular

enlargement of any cause, including inferior wall infarcts that involve the right ventricle, and is

commonly seen in the late stages of heart failure due to rheumatic or congenital heart disease with

severe pulmonary hypertension, as well as in ischemic heart disease, cardiomyopathy, and cor

pulmonale. It is in part reversible if pulmonary hypertension is relieved. Rheumatic fever may produce

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organic TR, often associated with TS. Endomyocardial fibrosis, infective endocarditis may produce

TR.

The clinical features of TR result primarily from systemic venous congestion and reduction of

cardiac output. The neck veins are distended with prominent V waves, and marked hepatomegaly,

ascites, pleural effusions, edema, systolic pulsations of the liver and positive hepato-jugular reflux are

common. A prominent right ventricular pulsation along the let parasternal region and a blowing

holosystolic murmur along the lower left sternal margin which may be intensified during inspiration

and reduced during expiration or the Valsalva maneuver are characteristic findings; AF is usually

present.

Summary: Signs of tricuspid regurgitation

- Pansystolic murmur over tricuspid area increases with inspiration.

- Systolic neck vein pulsations

Echocardiography and Doppler: for detection of severity of TR, estimation of pulmonary pressure and

search for vegetations of infective endocarditis.

Treatment of the underlying cause of heart failure usually reduces the severity of functional TR. In

patients with mitral valve disease and TR due to pulmonary hypertension and massive RV enlargement,

effective surgical correction of the mitral valve abnormality results in lowering of the pulmonary

vascular pressure and gradual reduction or disappearance of the TR. Tricuspid valvuloplasy by De

Vega procedure and Carpentier ring can be done.

Pulmonary Stenosis: See congenital pulmonary stenosis

Pulmonary Regurgitation

Dilatation of the pulmonary artery in cases of pulmonary hypertension may produce pulmonary

regurgitation. This is called Graham Steel murmur. It is differentiated from the early diastolic

murmur of aortic regurgitation by the associated signs of pulmonary hypertension, and by Doppler

study.

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CONGENITAL HEART DISEASE

Congenital heart malformations remain one of the most frequent birth defects, with a live-born

prevalence of about 8 per 1000 live-born infants in western countries.

Etiology of congenital heart disease:

It is generally an abnormal form of cardiac development in the first 6-8 weeks of intrauterine life. It is

either due to exposure of the fetus in this period to injurious teratogenic factor or to abnormal

chromosomal structure.

Some causes could be identified as:

5- Drugs e.g. thalidomide, excess alcohol intake, anticonvulsant drugs.

6- Exposure to radiation e.g. X-rays and gamma rays.

7- Hereditary diseases: Diseases caused by chromosomal abnormalities eg Turner syndrome, Down

syndrome or mongolism.

8- Maternal infections e.g. German measles in the first trimester of pregnancy.

Congenital heart diseases in the adults could be classified into:

IV- Left or right ventricular outflow obstruction: Aortic stenosis, pulmonary stenosis, coarctation of

aorta.

V- Left to right shunts: ASD, VSD and PDA.

VI- Cyanotic heart disease: Fallot’s tetralogy and other cyanotic congenital diseases.

LEET TO RIGHT SHUNT

When there is a congenital communication between both sides of the heart, e.g. atrial or ventricular

septal defects or patent ductus arteriosus the blood always flows from the left side (left atrium, left

ventricle or aorta) to right side (right atrium, right ventricle or pulmonary artery). This is because the

pressure in all left-sided chambers is higher than in right-sided chambers.

EFFECTS:

1- Left to right shunt results in pulmonary plethora (increased vascularity in the lung). If the shunt is

very big heart failure may occur but this is rare.

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2- In mild to moderate cases the pulmonary vessels dilate to accommodate the excessive blood flow.

Mild cases are well tolerated but if the shunt is excessive the pulmonary vessels react by

vasoconstriction. Pulmonary arteriolar vasoconstriction causes pulmonary hypertension which results

in right ventricular hypertrophy.

3- Pulmonary hypertension causes rise of pressure in the chambers of the right side of heart. Ultimately

the pressure in the right side exceeds that of the left side and the blood starts to flow across the defect

in the reverse direction, i.e. right to left shunt (reversed shunt). The patient becomes cyanosed. Emboli

originating in the venous side may be shunted across the defect to the arterial side and settle in organs

such as the brain or limbs. This is paradoxical embolism.

Closure of the defect at this stage is useless and dangerous. This situation of a congenital defect +

reversed shunt is called Eisenmenger’s syndrome. Eisenmenger’s syndrome is not an independent

congenital heart disease. It is the end result of big left to right shunt. At this stage the clinical picture is

that of central cyanosis with severe pulmonary hypertension.

ATRIAL SEPTAL DEFECT

In the presence of a defect in the atrial septum the right atrium receives blood both from the normal

venous return and the left atrium, the right atrium dilates. This results in: Dilatation and hypertrophy of

the right ventricle (volume overload), dilatation of the pulmonary artery, and pulmonary plethora. If the

defect is big and uncorrected pulmonary arteriolar vasoconstriction progressively occurs and results in

pulmonary hypertension usually at age 20-30 years. When the pressure in the right atrium exceeds that

in the atrium the shunt becomes reversed (Eisenmenger’s syndrome) and the patient becomes cyanosed.

Clinical features:

1- Atrial septal defect is more common in females. When the left to right shunt is very big pulmonary

plethora may predispose to repeated chest infections in infancy. Otherwise there are no symptoms for

many years. Ultimately heart failure occurs.

2- Atrial fibrillation occurs in late cases.

3- Right ventricular dilatation and hypertrophy cause a hyperdynamic impulse in the third and fourth

spaces to the left of the sternum and precordial bulge.

4- Excessive flow across the tricuspid valve may produce a third heart sound and short mid-diastolic

murmur at the tricuspid area.

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5- Excessive blood flow at the pulmonary valve may produce pulsations, dullness and an ejection

systolic murmur in the pulmonary area.

6- The specific auscultatory sign of atrial septal defect is wide fixed splitting of the second heart at the

pulmonary area. The pulmonary component of the second sound is delayed because the right ventricle

takes a long time o empty the excessive volume of blood it receives. The splitting dose not vary with

respiration because: although inspiration causes increase in venous return, yet the resulting rise in right

a trial pressure causes proportionate decrease in the left to right shunt so that the right ventricular

output is constant and the time relation between aortic and pulmonary components of the second sound

remains constant.

7- Progressive pulmonary hypertension occurs in big defects and result in Eisenmenger syndrome. At

this stage the clinical picture consists of: Central cyanosis, signs of pulmonary hypertension, and signs

or right ventricular hypertrophy.

X-RAY PICTURE:

2- Plethoric lung fields. 2- Dilatation of the right atrium, right ventricle and pulmonary artery. 3-

Marked pulsation of the pulmonary artery and its branches seen during screening (hilar dance).

ELECTROCARDIOGRAPHIC FEATURES: The characteristic sign is incomplete right bundle branch

block with rSr' pattern in V1 lead. Signs of right ventricular hypertrophy also appear when pulmonary

hypertension develops. Atrial fibrillation occurs in late cases.

ECHOCARDIOGRAPHY WITH DOPPLER: Must be done for every patient with suspected

congenital heart disease. In A.S.D. it shows the septal defect and dilated right ventricle and abnormal

movement of the interventricular septum characteristic of volume overload on the right ventricle.

Cardiac catheterization may be done in some cases.

COMPLICATIONS:

3- Pulmonary hypertension and reversal of shunt.

4- Right ventricular failure. 3- A trial fibrillation.

TREATMENT: Small defects can be left alone. Large defects should be closed surgically or by

percutaneous insertion of occluder (device that occludes the ASD) .

VENTRICULAR SEPTAL DEFECT

1- In the presence of a defect in the septum, the right ventricle receives both the normal venous and the

shunted blood. If the defect is big right ventricular hypertrophy occurs.

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2- This excessive blood flows in the pulmonary artery and the pulmonary circulation and then returns

to the left atrium and the left ventricle. This causes: Dilatation of the pulmonary artery, pulmonary

plethora, dilatation of the left atrium, dilatation and hypertrophy of the left ventricle.

3- If the shunt is very big excessive flow may cause heart failure in infancy.

4- If the shunt is large the pulmonary vessels react by vasoconstriction causing pulmonary hypertension

and reversal of shunt (Eisenmenger syndrome).

5- Small V.S.D. does not cause pulmonary hypertension and may close spontaneously. Clinically, the

murmur is very loud (Roger’s disease).

CLINICAL PICTURE: The specific signs of V.S.D. are: 1- A characteristic pansystolic murmur best

heart in the third and fourth left intercostal spaces just lateral to the sternum, usually accompanied by a

thrill. 2- With large shunts the increased flow across the mitral valve may cause a third sound and a

mid-diastolic flow murmur at the apex.

The clinical course depends upon the size of the defect:

3- Small ventricular septal defect: many defects close spontaneously.

4- Moderately large defect:

4th- Progressive pulmonary hypertension and low cardiac output e.g. fatigue, syncope on exercise,

pulsations and palpable loud second heart sound in the pulmonary area, right ventricular hypertrophy,

etc.

5th- When the pressure in the right ventricle equals that in the left ventricle no blood will flow

across the defect and the murmur diminishes disappears. The patient becomes cyanosed on crying.

6th- When the shunt is reversed the patient becomes cyanosed.

X-RAY PICTURE: Is normal in cases with small defects. Large defects result in: pulmonary plethora

(overfilled large and tortuous pulmonary arteries), large main pulmonary artery, left and right

ventricular enlargement, left atrial enlargement.

ECHOCARDIOGRAPHY WITH DOPPLER: Can show the size of cardiac chambers. The defect can

sometimes be shown by two-dimensional echo. Color Doppler is very helpful in showing the blood

flow through the defect. Detection of the site of the defect, the magnitude of the shunt and the degree of

pulmonary hypertension can be assessed by this non-invasive method.

CARDIAC CATHETERISATION AND ANGIOGRAPHY: Is done in some cases.

COMPLICATIONS: Infective endocarditis, pulmonary hypertension, and heart failure.

DIFFERENTIAL DIAGNOSIS: A pansystolic murmur at the sternal border can be caused by tricuspid

or mitral incompetence in addition to the ventricular septal defect. Sometimes the murmur of

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pulmonary stenosis is heard at the third intercostal space but it is usually ejection in type and its

maximal intensity is in the second space. Other causes of systolic murmur at left sternal border are

hypertrophic obstructive cardiomyopathy, subaortic membrane and aortic stenosis.

TREATMENT:

1- To prevent infective endocarditis all patients must receive an antibiotic prophylaxis before

performing minor procedures that may causes bacteremia, e.g. dental extraction, delivery, etc.

2- Small ventricular septal defects should be left alone. Many of them close spontaneously.

3- Surgical closure is indicated if the defect is moderate or large in size, provided that the pulmonary

pressure is normal or moderately elevated. Surgical closure is contraindicated if pulmonary pressure is

severe (Eisenmenger’s syndrome).

PATENT DUCTUS ARTERIOSUS

The ductus arteriosus is normally present in the fetus. It connects the aorta (at the junction of the arch

with the descending aorta) with the pulmonary artery (at the junction of the main pulmonary artery with

its left branch). It normally closes. During the first month after birth:

Effects:

1- The blood flows through the duct from the aorta to the pulmonary artery, i.e. left to right shunt.

2- As the pulmonary artery receives blood both from the shunt and the right ventricle, pulmonary artery

dilatation and pulmonary plethora occur.

3- If the shunt is big pulmonary vasoconstriction and hypertension occurs. When the pressure in the

pulmonary artery equals that of the aorta the shunt will first become confined to the systole only and

then ceases altogether. The murmur, accordingly, will first become only systolic and finally will be

completely inaudible.

5- When the pressure in the pulmonary artery exceeds that of the aorta, the shunt will be reversed and

cyanosis occurs (Eisenmenger’s syndrome).

CLINICAL FEATURES: Patent ductus arteriosus is commoner in females. Its characteristic signs are:

1- A continuous (machinery) murmur that occupies both systole and diastole because the pressure in

the aorta exceeds that of the pulmonary artery all through the cardiac cycle. It is best heard in the first

and second left intercostal spaces. There may be continuous thrill in the same area.

2- With large ductus, the increased flow across the mitral may cause a mid-diastolic murmur.

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When the pressure in the pulmonary artery exceeds that of the aorta, right to left shunt occurs and

cyanosis appears (Eisenmenger’s syndrome). The deoxygenated blood will flow from the pulmonary

artery across the ductus down the descending aorta. The lower limbs will be cyanosed while the upper

limbs remain pink (differential cyanosis).

X-RAY PICTURE: X-ray is normal in cases with small ductus. In moderate to large ductus the

following signs appear: Pulmonary plethora, enlargement of the left atrium, left ventricle and the aorta.

Hilar dance seen in the hilum by screening.

Differential diagnosis: Other causes of continuous murmur as aorto-pulmonary window, in coarctation

of the aorta, mammary softle, rupture sinus of Valsalva, venous hum...

TREATMENT: Prophylaxis against endocarditis. Closure either surgical or with a device introduced

with percutaneous, transvenous catheter.

CYANOTIC HEART DISEASE

- Tetralogy of Fallot.

- Ebstein anomaly.

- Transposition of the great arteries.

- Total anomalous pulmonary venous drainage.

- Truncus arteriosus.

- Pulmonary arterio-venous malformation.

Acquired cyanotic disease: Eisenmenger Syndrome.

FALLOT’S TETRALOGY

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PATHOLOGY AND EFFECTS: Fallot’s tetralogy consists of:

6- Severe pulmonary stenosis which causes right ventricular hypertrophy. The pulmonary stenosis is

usually infundibular but sometimes it is both valvular and infundibular.

7- Large ventricular septal defect which makes the pressure equal in both ventricles.

8- The origin of the aorta is abnormally deviated to the right (dextroposed, dextro = right) so that it

lies partly over the right ventricle (the aorta overrides both ventricles).

9- Due to the severe pulmonary stenosis and the large ventricular septal defect, the pressure in both

ventricles is equal. There is rush of blood across the defect and the ventricular septal defect produces

no murmur.

10- Part of the blood pumped by the right ventricle passes in the aorta (right to left shunt) causing

central cyanosis.

In summary Fallot’s tetralogy consists of four components (tetra =4).

6- Pulmonary stenosis.

7- Ventricular septal defect.

8- Dextroposed and overriding aorta.

9- Right ventricular hypertrophy.

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CLINICAL FEATURES:

1- The patient is cyanosed since birth, (usually after birth by few weeks); the degree of cyanosis

depends on the severity of the pulmonary stenosis.

2- When the patient exercises, cyanosis is increased. In order to increase the blood flow to the head and

brain, the child usually squats to compress the lower limbs against the abdomen and to deviate the

blood from the lower to the upper half of the body. It also increases the systemic arterial resistance. As

the pressure in the aorta rises, more blood will be deviated across the pulmonary stenosis to the lungs.

Thus more oxygenated blood returns to the heart.

3- Chronic cyanosis and tissue anoxia results in: Dyspnea, fatigue, angina, retarded growth,

polycythemia, clubbing of fingers.

4- Sometimes the muscle surrounding the outflow tract of the right ventricle goes into spasm,

especially after excitement and exercise. The blood flow to the lungs decreases markedly and the

oxygenation decreases resulting in attacks of severe cyanosis: cyanotic spells. If prolonged they may

lead to death.

10- The characteristic cardiac signs are:

C- Murmur of pulmonary stenosis (ejection systolic murmur in second left space, usually

accompanied by a thrill.

D- The second heart sound is single and consists only of the aortic component. C- Right

ventricular hypertrophy.

X-RAY PICTURE:

4. Right ventricular hypertrophy causes the apex to be displaced outwards and becomes separated

from the diaphragm.

5. Right-sided aortic arch in some cases.

6. Pulmonary oligemia (the pulmonary artery and its branches are diminished in size due to the

pulmonary stenosis. All the above factors result in a characteristic cardiac shadow: Coeur en sabot

(sabot = wooden shoe).

ELECTROACARDIOGRAPHIC FEATURES: Show moderate right ventricular hypertrophy.

ECHOCARDIOGRAPHY WITH DOPPLER: Delineates the abnormal anatomy. Cardiac

catheterization and angiography is needed for differential diagnosis.

COMPLICATIONS:

4- Polycythemia causes increased viscosity of blood resulting in a tendency towards thrombosis, e.g.

cerebral thrombosis.

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5- Infective endocarditis

6- Brain abscess results when bacterial emboli are shunted from the venous to the arterial side and

lodge in the brain (paradoxical embolism).

TREATMENT:

1- Surgical correction is indicated in all cases by: Resection of the excessive stenotic infundibular

muscle splitting of the fused pulmonary valve leaflets, and closure of the ventricular septal defect.

2- If he patient is too young, or the condition is too severe, an anastomosis is performed to allow blood

to reach the lungs by: implanting the subclavian artery in the corresponding pulmonary artery (Blalock-

Taussig operation).

3- Cyanotic attacks result from infundibular spasm and constitute an emergency. The are treated by:

Put the patient in the squatting position or compress the flexed lower limbs against the abdomen,

sedation, propranolol (inderal) intravenously. Propranolol is a beta-adrenergic blocker. It depresses the

contractility of the infundibular muscle.

LEFT VENTRICULAR OUTFLOW TRACT OBSTRUCTION

- Valvular aortic stenosis: 70% of patients with valvular AS a malformation of the valve (usually a

bicuspid valve).

- Discrete subvalvular aortic membrane:

Represents 8-10% of congenital AS. The magnitude of obstruction is variable. Most membranes are

eventually associated with progressive aortic regurgitation and their presence may be an absolute

indication for excision. There is a high recurrence rate after excision (approximately 30% and septal

myotomy is often performed).

COARCTATION OF THE AORTA

Narrowing of the aorta usually just distal to the left subclavian artery. Coarctation may affect other

parts of the aorta or the renal arteries.

EFFECTS:

1- Because of the narrowing, pressure rises in the ascending aorta and the aortic arch and its branches.

This results in hypertension in the upper limbs.

2- Pressure and flow decreases in the descending aorta and its branches producing ischemia in the

abdominal organs and the limbs.

3- Ischemia of the kidneys results in release of renin which raises the blood pressure.

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4- Hypertension results in left ventricular hypertrophy and it severe results in left ventricular failure.

5- Anastomosis form between the branches of the aorta proximal and distal to the obstruction. The

most important of these connect the subclavian artery through its internal mammary branch to the

intercostal arteries which arise from descending aorta. The intercostal arteries become enlarged and

tortuous and erode the lower border of the ribs causing rib notching. Appreciable anastomosis

develops gradually by time. That is why rib notching is not detectable except after the age of 10. Other

anastomosis develops around the scapula and another connects the superior and inferior epigastric

arteries.

CLINICAL FEATURES:

1- In the majority of cases there are no symptoms and the essential diagnostic feature of coarctation is

that the blood pressure in the upper limbs exceeds that in the lower limbs.

2- The pulse in the upper limbs, neck and suprasternal notch is strong. Pulse in the lower limbs is weak

and delayed or absent.

3- Hypertension in the upper half of the body may produce headache, epistaxis while ischemia of the

lower half may produce thin, underdeveloped lower limbs and claudication in the calf.

4- Visible and palpable pulsations of dilated collateral may be felt in the intercostal areas.

5- A late systolic murmur may be heard on the back due to blood flow in the collaterals. The murmur is

sometimes continuous.

6- The cardiac signs are nonspecific and include: left ventricular hypertrophy, an ejection systolic

murmur heard at the aortic area.

X-RAY PICTURE:

1- Signs of left ventricular hypertrophy.

2- Rib notching is the most specific sign.

ELECTROCARDIOGRAPHIC SIGNS: Left ventricular hypertrophy and strain.

COMPLICATIONS:

1- Hypertension in the upper half of the body may result in: cerebral or subarachnoid hemorrhage, left

ventricular failure, dissection of the aorta.

2- Infective endocarditis.

TREATMENT: surgical resection of the narrowed segment is indicated in moderate and severe cases

preferably during childhood. Balloon dilation with expandable stent is a feasible method of treatment.

All patients must have prophylaxis against endocarditis.

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PULMONARY STENOSIS

Pulmonary stenosis may be caused by: Congenital fusion of pulmonary valve cusps (congenital

valvular pulmonary stenosis).

EFFECTS:

2- In both valvular and infundibular stenosis the pressure in the right ventricle rises, this causes

hypertrophy of the right ventricle (pressure over-load). Consequently the right atrium hypertrophies.

When the stenosis is severe the output of the right ventricle and the cardiac output are reduced. The

pulmonary blood flow is reduced, i.e. pulmonary oligemia.

CLINICAL FEATURES:

6. Mild cases are as asymptomatic, in severe cases low cardiac output occurs and results in

fatigability, syncope on effort, small volume pulse, cold extremities, etc.

7. An ejection systolic murmur is caused by passage of blood through the stenosed valve. It is best

heard over the pulmonary area. It may be preceded by an ejection click.

8. The pulmonary component of the second heart sound is faint and delayed due to prolonged

contraction of the right ventricle.

9. There is usually a systolic thrill over the pulmonary area.

10. Right ventricular hypertrophy produces a sustained impulse in the third and fourth intercostal

spaces just to the left of the sternum and pulsation in the epigastrium. Forceful right atrial contraction

causes a large wave in the neck veins (the a wave).

X-RAY PICTURE: 1. Pulmonary oligemia occurs in moderate to severe cases and results in reduced

pulmonary vascular markings). 2- Right ventricular enlargement is proportional to the severity of the

stenosis. Right atrial enlargement may also occur. 3. Post-stenosis dilatation of the pulmonary artery

is seen.

ECG FEATURES: Right ventricular hypertrophy.

ECHO FEATURES: Right ventricular hypertrophy, the stenosed pulmonary valve.

TREATMENT: Either percutaneous transvenous balloon dilatation (the standard treatment, first

option) or surgical removal of the valve by open-heart surgery.

Interventions In Congenital Heart Diseases (therapeutic procedures that are used in treatment

without surgery but through catheterization):

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- Pulmonary stenosis balloon dilatation.

- Aortic stenosis balloon dilatation.

- Coarctation of the aorta balloon dilatation and stent insertion.

- Atrial septal defect insertion of Amplatzer occluder through catheter.

- Patent ductus arteriosus occlusion by insertion of coil.

- Other procedures.

DIAGNOSIS AND MANAGEMENT OF SYNCOPE AND HYPOTENSION

Syncope is a sudden and transient loss of consciousness with associated loss of postural tone. The

occurrence of syncope is 3% in men ad 3.5% in women in the general population. As a general role, the

incidence of syncope increases with age.

Hypotension: When systolic blood pressure (SBP) is less than 90 mmHg or reduction of SBP of 30

mmHg or more from baseline.

Patients with transient episode of altered consciousness (presyncope) and those with complete loss of

consciousness (syncope) are classified into 3 broad categories: cardiac syncope, noncardiac syncope,

and syncope of undetermined etiology. Among all patients with syncope associated with cardiac

disease, sudden cardiac death is extremely high.

Table: Causes of Syncope

Circulatory (reduced cerebral blood flow)

F. Inadequate vasoconstrictor mechanisms

7. Vasovagal (vasodepressor)

8. Postural hypotension

9. Primary autonomic insufficiency

10. Sympathectomy (pharmacologic, due to antihypertensive medications such as methyldopa and

hydralazine, or surgical )

11. Carotid sinus syncope

12. Diseases of the central and peripheral nervous system, including autonomic nerves)

G. Hypovolemia

4. Blood loss – gastrointestinal hemorrhage.

5. Addison’s disease

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H. Mechanical reduction of venous return

2. Valsalva maneuver. 2. Cough; Micturition.

6. Atrial myxoma, ball valve thrombus.

I. Reduced cardiac output

5. Obstruction to left ventricular outflow: aortic stenosis, hypertrophic subaortic stenosis.

6. Obstruction to pulmonary flow: pulmonary stenosis, primary pulmonary hypertension, pulmonary

embolism.

7. Myocardial: massive myocardial infarction with pump failure.

8. Pericardial: cardiac tamponade

J. Arrhythmias

2. Bradyarrhythmias

e. Atrioventricular (AV) block (second and third degree), with Stokes-Adams attacks

f. Ventricular asystole

g. Sinus bradycardia, sinoatrial block, sinus arrest, sick sinus syndrome

h. Carotid sinus syncope

b. Tachyarrhythmias: Supraventricular tachycardia. Episodic ventricular tachycardia

Other causes of disturbances of consciousness

F. Hypoglycemia

G. Hypoxia

H. Hypoventilation

I. Transient cerebral ischemic attack

J. Emotional disturbances, anxiety attack, hysterical seizures.

Noncardiac Syncope

Neurocardiogenic syncope:

The syndrome of neurocardiogenic syncope, the common faint (also referred to as neurally mediated

hypotension, vasovagal syncope, and vasodepressor syncope), is one of the most common causes of

syncope.

This disorder is due to abnormality in the neuro-cardiovascular interactions responsible for maintaining

systemic and cerebral perfusion.

Diagnostic evaluation:

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Head-up tilt (HUT) is essential for the diagnosis of neurocardiogenic syncope. Here we change the

position of the patient from the horizontal to the vertical position. HUT at an angle of 60º to 90º for a

time period of 20 to 60 min is the usual protocol.

Management of syncope:

First-line therapy includes counseling the patient to avoid dehydration, prolonged period of standing

motionless, and situations known to trigger syncope. Volume expansion, fludrocortisone may be

helpful in augmenting salt retention and volume expansion.

Alpha-Agonists: Medodrine may prevent neurocardiogenic syncope due to vasoconstrictor effect that

may reduce venous pooling.

Orthostatic Syncope (orthostatic Hypotension):

Orthostatic hypotension is a disorder in which assumption of the upright posture is associated with a

fall in blood pressure. Therapy: is based on treatment of causes.

Management of hypotension: 1- Treatment of the etiology. 2- Avoid dehydration. 3- Medodrine. 4.

Mineralocorticoids as Astonin H.

Cardiac Syncope

It is due to severe diminution of the cardiac output Either due to severe obstructive lesion as tight mitral

stenosis, atrial myxoma, aortic stenosis, obstructive cardiomyopathy or due to arrhythmia whether

tachy or brady. Obstructive lesions and arrhythmias frequently coexist; indeed, one abnormality may

accentuate the other. Common disorders associated with cardiac syncope are listed in table.

Diagnostic evaluation of syncope associated with cardiac disease:

- History & physical examination

- Echocardiography & Doppler

- Standard ECG

- Holter monitor ( 24 h. ECG continuous recording )

- Electrophysiologic study.

- Cardiac catheterization.

Treatment of cardiac syncope: Obstructive Heart Disease, for patients with syncope caused by

obstructive heart disease, cardiac surgery is often the treatment of choice.

Arrhythmic syncope, detailed discussion of therapy for cardiac arrhythmias presented earlier.

Antiarrhythmic drugs, pacemakers and ablation are available tools of management of arrhythmia.

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Syncope of undetermined cause: Despite careful diagnostic evaluation, the cause of syncope often

cannot be defined.

Sudden Cardiac Death

Definition: Sudden cardiac death describes the unexpected natural death due to cardiac cause within a

short period from the onset of symptoms.

More recent definition focused on time interval of one hour from the symptoms leading to collapse and

then to death.

Incidence: SCD accounts for 300.000 to 400.000 deaths yearly in the United States. SCD is the most

common and often the first manifestation of coronary heart disease (CHD) and is responsible for half

the deaths from cardiovascular disease.

Sudden Cardiac Death in the young: The most common underlying pathological conditions in people

who die of SCD in the first three decades of life are myocarditis, hypertrophic cardiomyopathy,

congenital coronary artery anomalies, atherosclerotic coronary heart disease, conduction system

abnormalities (e.g. long QT), congenital arrhythmogenic disorders, arrhythmias associated with

mitral valve prolapse and aortic dissection. About 40% of SCD in the pediatric population occur in

patients with surgically treated congenital cardiac abnormalities.

Risk factors for Sudden Cardiac Death (SCD):

12- Left ventricular hypertrophy (by ECG)

13- Cholesterol.

14- Hypertension.

15- Cigarette smoking.

16- Diabetes.

17- Alcohol.

18- Obesity.

19- History of coronary heart disease.

20- Age.

21- Positive family history of SCD.

22- Frequent PVCs (Premature ventricular contractions, unsustained ventricular tachycardia).

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Cardiac Abnormalities Associated with Sudden Cardiac Death

I. Ischemic heart disease

A) Coronary Atherosclerosis:

- Acute myocardial infarction, - Chronic ischemic cardiomyopathy

B) Anomalous origin of coronary arteries.

II. Cardiomyopathies

E. Idiopathic dilated cardiomyopathy

F. Hypertrophic cardiomyopathy

G. Hypertensive cardiomyopathy

H. Arrhythmogenic right ventricular dysplasia

III. Valvular heart disease: Aortic stenosis

IV. Inflammatory and Infiltrative myocardial disease

V. Congenital heart disease.

VI. Primary Electrical Abnormality.

F. Long Q-T syndrome

G. Wolf Parkinson White syndrome (WPW).

H. Idiopathic ventricular tachycardia

I. Idiopathic ventricular fibrillation

J. Brugada syndrome (right bundle block with raised ST in V1 to V3)

VII. Drug and other toxic agents

C. Proarrhythmia (Drug induced arrhythmia)

D. Cocaine and Alcohol. C. Electrolyte abnormalities

Treatment Options for Patients at Risk of Sudden Cardiac Death (SCD)

I. Pharmacologic therapy

4- Beta blockers , Angiotensin-converting enzyme inhibitors

5- Class I antiarrhythmic drugs,

6- Class III antiarrhythmic drugs: Amiodarone, sotalol

II. Device therapy

3- Automatic implantable cardioverter Defibrillator (ICD)

4- External automatic defibrillator

III. Role of surgery: Revascularization

IV. Catheter Ablation therapy.

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CARDIAC ARRHYTHMIAS

An arrhythmia is any disturbance in the normal sequence of impulse generation and conduction in the

heart.

Anatomy of the conduction system: The conduction system of the heart consists of the sinus node,

internodal tracts, atrioventricular node (AVN), bundle of His, bundle branches (right and left), and

Purkinje fibers.

Fig: The pathways of Conduction.

General considerations: Normal cardiac impulses arise from the automatic (pacemaking) cells of the

sinus node and are conducted through the atria to the AV junction then the His-Purkinje system to the

ventricular muscle. Normally the sinus node discharges at a rate of 60-100/min.

Mechanisms of arrhythmias

A- Disturbance of impulse formation: may result from either:

3- Disturbed normal automaticity:

4- Triggered activity: Hyper-excitable focus which discharges ectopic impulses.

B- Disturbance of Impulse conduction: e.g. heart block

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Classification of arrhythmia:

Clinical classification:

- Rapid, regular. Sinus tachycardia, supraventricular tachycardia, atrial flutter, ventricular

tachycardia.

- Rapid, irregular. Sinus arrhythmia, multiple ectopic beats whether atrial or ventricular, atrial

fibrillation.

- Slow, regular. Sinus bradycardia, nodal rhythm, complete heart block.

- Slow, irregular. Slow atrial fibrillation.

Disturbances in Sinus Rhythm

Sinus tachycardia

Cardiac impulses arise in the sinus node at a rate more than 100/min.

Etiology:

A- Physiological: Infancy, childhood, exercise and excitement.

B- Pharmacological: Sympathomimetic drugs such as epinephrine and isoproterenol.

Parasympatholytic drugs such as atropine. Thyroid hormones, nicotine, caffeine, alcohol.

C- Pathological: Fever, hypotension, heart failure, pulmonary embolism, hyperkinetic circulatory

states as anemia.

Treatment: 1- Treatment of the underlying etiology. 2- Propranolol.

Sinus Bradycardia

Cardiac impulses arise in the sinus node at a rate less than 60/min.

Etiology:

A- Physiologic: Athletes, sleep, and carotid sinus compression.

B- Pharmacologic: Digitalis, propranolol, verapamil and diltiazem.

C- Pathologic: Convalescence from infections, hypothyroidism, obstructive jaundice, rapid rise of the

intracranial tension, hypothermia and myocardial infarction (particularly inferior wall infarction).

Treatment:

3- Treatment of the underlying etiology is usually all that is needed.

4- If the patient is hemodynamically compromised, Atropine 0.6 – 1.0 mg IV may be given and

repeated every 3 hours (maximum 2.5 mg in two hours).

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SICK SINUS SYNDROME: This term is applied to a syndrome encompassing a number of sinus

nodal abnormalities that include: 1- persistent spontaneous sinus bradycardia not caused by drugs, and

inappropriate for the physiological circumstance, 2- apparent sinus arrest or exit block, 3- combinations

of SA and AV conduction disturbances, or 4- alternation of paroxysms of rapid and slow atrial and

ventricular rates (bradycardia-tachycardia syndrome).

FIG. Normal intracardiac electrograms.

PREMATURE BEATS (EXTRASYSTOLES)

These are cardiac impulses of ectopic origin occurring earlier than expected in the prevailing rhythm.

The ectopic focus may be: 1- Atrial resulting in atrial premature beat. 2- AV junctional (arising from

bundle of His) resulting in AV junctional premature beat. 3- Ventricular resulting in ventricular

premature beat.

Etiology:

A- Physiological: Emotions, exercise and fatigue.

B- Pharmacological: Coffee, alcohol, tobacco, catecholamines, digitalis and hypoxia.

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C- Pathological: Various infections, digestive disturbances, hyperthyroidism and all cardiovascular

disorders.

SUPRAVENTRICULAR TACHYARRHYTHMIAS

All tachyarrhythmias that originate above the bifurcation of the bundle of His are classified as

supraventricular arrhythmias (SVT). The atrial rate must be 100 or more beats per minute for a

diagnosis.

SVTs may be separated into three groups based on duration: brief paroxysms, persistent, and chronic

(permanent).

Arrhythmias that are paroxysmal in onset and offset (e.g., paroxysmal SVT due to AV nodal reentry or

WPW syndrome, paroxysmal atrial fibrillation, paroxysmal atrial flutter) tend to be recurrent and of

short duration; i.e., seconds to hours.

Persistent tachycardias (e.g., sinus tachycardia, ectopic atrial tachycardia (nonparoxysmal), multifocal

atrial tachycardia, longer episodes of PSVT or atrial flutter or fibrillation) may persist for days or

weeks.

Longstanding or chronic SVTs (chronic atrial flutter, chronic atrial fibrillation) do not revert if

untreated, often fail to revert even with attempted treatment, and if reverted will frequently recur

despite therapy.

Supraventricular tachyarrhythmias include; atrial tachycardia, atrial flutter, atrial fibrillation and AV

tachycardias.

ATRIAL FLUTTER

Atrial flutter is a rapid regular atrial tachyarrhythmia that is less common than the PSVTs or atrial

fibrillation. It is observed in the presence of underlying atrial abnormalities such as those secondary to

mitral valve disease, congenital heart disease, cardiomyopathies, and, less frequently, coronary artery

disease.

Untreated atrial flutter usually has atrial rates between 240 and 340 per minute, commonly very close

to 300 per minute. The ventricular rate in atrial flutter is usually a defined fraction of the atrial rate 2: 1

conduction generating a ventricular rate of 150 per minute and 4:1 conduction at 75 per minute.

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Clinically, atrial flutter may occur in brief, persistent, or chronic forms, and therapeutic approaches are

influenced by the clinical pattern.

Electrocardiographic Features

Atrial flutter generates a defined pattern of atrial activity in the ECG. Classically, a saw-tooth pattern is

identifiable in leads II, 111, and aVF. A narrow QRS complex tachycardia at a rate of 150 per minute

should always lead to the consideration of atrial flutter. Carotid sinus massage will not interrupt atrial

flutter but nonetheless may be very helpful in distinguishing flutter from other mechanisms,

impairment of AV nodal conduction causes an abrupt change from a rate of 150 per minute to 75 per

minute or less.

Management of atrial flutter: - If the patient is hemodynamically compromised, D.C. cardioversion

using low energies (around 50 joules) should be instituted.

- Administering a Class IA antiarrhythmic agent (i.e., quinidine, procainamide, or disopyramide). IC

antiarrhythmic drugs, flecainide and propafenone, are as effective, if not more effective than Class IA

drugs. Class III antiarrhythmic agents (i.e., amiodarone, sotalol) may also be quite effective. In general,

atrial flutter is difficult to suppress completely with drug therapy. - The ventricular rate is slowed by

digitalis and/or propranolol or verapamil before antiarrhythmics are instituted to avoid very rapid rates

associated with drug induced 1:1 AV conduction.

- At present, catheter ablation provides the best hope of cure.

FIG. A 12-lead ECG of a typical case of type 1 atrial flutter.

FIG. A 12-lead ECG of a typical case of type 1 atrial flutter.

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FIG: Atrial flutter with AV block varying between 2: 1 and 4: 1.

AV Nodal Reentrant Tachycardia

Electrocardiographic Features: Paroxysmal SVT due to AV nodal reentry is characterized by an

abrupt onset and termination and usually has a narrow QRS complex without clearly discernable P

waves. The rate is commonly in the range of 150 to 250 per minute (commonly 180 to 200 bpm in

adults) and with a regular rhythm.

Management of PSVT Due to AV Nodal Reentry

The acute attack: Vagal maneuvers serve as the first line of therapy. Simple procedures to terminate

paroxysmal SVT

- Carotid sinus massage: If effective the rhythm is abruptly stopped; occasionally only moderate

slowing occurs

- Cold water splash on face.

- Performance of Valsalva's maneuver (often effective).

Intravenous adenosine, Ca channel blockers (verapamil), digoxin or B-blockers are the choices for

managing the acute episodes.

Adenosine, 6 mg given intravenously, followed by one or two 6-mg boluses if necessary, is effective

and safe for acute treatment.

A 5-mg bolus of verapamil (isoptin) , followed by one or two additional 5-mg boluses 10 min apart if

the initial dose does not convert the arrhythmia, has been an effective regimen in up to 90 percent of

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patients with PSVT due to AV node reentry. Intravenous digoxin, 0.5 mg infused over 10 min and

repeated if necessary may convert the arrhythmia.

DC cardioversion: Consider DC cardioversion before digitalis or a beta blocker is administered.

Radiofrequency catheter ablation: Should be considered early in the management of patients with

symptomatic recurrent episodes of AV node reentry.

AV Reentrant Tachycardia

PSVT Due to Accessory Pathways (The Wolff-Parkinson-White Syndrome)

ELECTROCARDIOGRAPHIC RECOGNITION: Three basic features in the ECG of patients with the

usual form of WPW syndrome caused by an AV connection:

(1) Short P-R interval less than 120 msec during sinus rhythm;

(2) QRS complex duration exceeding 120 msec

(3) Slowly rising onset of the QRS in some leads (delta wave).

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The most common tachycardia is characterized by a normal QRS, by ventricular rates of 150 to 250

beats/min and by sudden onset and termination.

Termination of the acute episode should be approached as for AV nodal reentry. In many patients,

particularly those with a very rapid ventricular response, electrical cardioversion is the initial treatment

of choice.

The Wolff-Parkinson-White Syndrome

ELECTRICAL ABLATION: Ablation of the accessory pathway is advisable for patients with frequent

symptomatic arrhythmias that are not fully controlled by drugs.

Atrial Fibrillation

The arrhythmia is characterized by multiple electric foci in the atrium causing disorganized atrial

depolarizations without effective atrial contraction. Electrical activity of the atrium can be detected on

ECG as small irregular baseline undulations, called f waves, at a rate of 350 to 600 beats/min. The

ventricular response is grossly irregular (irregular irregularity) and is usually between 100 and 160

beats/min.

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It is a common arrhythmia, occurring in 5 – 10 % of individuals over 65 years of age. It also occurs in

a paroxysmal form in younger patients.

The hemodynamic consequences of atrial fibrillation are due to two factors:

(1) The loss of atrial systole may impair ventricular function in the noncompliant ventricle [e.g., aortic

stenosis, left ventricular hypertrophy (LVH)] or the dilated ventricle with systolic dysfunction, and

(2) A rapid ventricular rate will encroach upon the diastolic filling period of the left ventricle and the

diastolic flow time of the coronary arteries.

(3) The risk of embolism and stroke is a long-term concern of special importance. Atrial fibrillation

may occur in paroxysmal, persistent, and chronic patterns.

Clinical expression of atrial fibrillation:

Definition Duration

- Paroxysmal Minutes/hours

- Short-lasting Seconds --<1 hour

- Long-lasting >1 hour; -- < 48 hours

- Persistent Two days -- weeks

- Permanent (Chronic) Months / years

Table: Causes of atrial fibrillation

With structural heart disease

- Rheumatic mitral valve disease

- Ischemic heart disease

- Hypertension

- Cardiomyopathy: Dilated, Hypertrophic

- Atrial septal defect, - Constrictive pericarditis, Myocarditis

Without structural heart disease

- Alcohol. Thyrotoxicosis

- Acute pericarditis. Pulmonary embolism

- Sick sinus syndrome, Lone atrial fibrillation

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Atrial Fibrillation

Clinical picture

Onset and offset are sudden in paroxysmal cases.

Symptoms: Paroxysmal AF produces symptoms similar to those of supraventricular tachycardia.

Established AF (persisting for more than two weeks) is better tolerated than the paroxysmal variety.

Congestive heart failure may occur if the attack is prolonged, the ventricular rate is very rapid, or the

underlying heart disease is severe.

Signs:

1- Arterial pulse:

a- Rate is usually 100-150/min. Slower rates may be encountered in old age and in patients receiving

digitalis or beta-blockers.

b- Rhythm shows marked (irregular) irregularity. c- Force is irregular. d- Pulsus deficit: The radial

pulse rate is less than the cardiac rate counted at the apex beat. This is due to inability of the week

ventricular contractions following short diastolic periods to open the aortic valve.

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2- Neck veins show systolic expansion; no “a” waves are seen.

3- Auscultation reveals varying intensity of S1.

4- Exercise increases the pulse irregularity and deficit.

Electrocardiogram: The P waves are replaced by irregular f waves. The QRS complexes are normal in

shape but irregularly spaced.

Complications: 1- Atrial thrombosis due to stagnation of blood in the fibrillating atria. The formed

thrombi may embolize in the systemic and pulmonary circulations. 2- Heart failure due to loss of the

atrial contribution to contractility and the cardiac output.

Atrial fibrillation (AF) progressed to ventricular fibrillation (VF)

Treatment of Atrial Fibrillation

Pharmacologic Management of Patients with Recurrent Persistent or Permanent AF:

- Recurrent Persistent AF:

C) Minimal or no symptoms: Anticoagulation and rate control as needed.

D) Disabling symptoms in AF:

4- Anticoagulation and rate control

5- Antiarrhythmic drug therapy

6- Electrical cardioversion as needed, continue anticoagulation as needed and therapy to maintain

sinus rhythm

- Permanent AF: Anticoagulation and rate control as needed.

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AF management

Antiarrhythmic Drug Therapy to Maintain Sinus Rhythm in Patients with Recurrent

Paroxysmal or Persistent AF:

A) No or minimal heart disease:

4- Flecainide, propafenone, sotalol

5- Amiodarone, dronedarone, dofetilide, Disopyramide, procainamide, quinidine

6- Consider non-pharmacological options (ablation).

B) Heart disease present:

a- Heart failure: Amiodarone, dofetilide

4- Coronary artery disease: Sotalol, Amiodarone, dofetilide

5- Dronedarone is allowed only in HF class I or II with precaution.

6- Vernakalant I.V. for aute AF of less than 7 days duration, with many precautions and

contraindications.

C) Hypertension: With

3- With LVH (septum greater than or equal to 1.4 cm): Amiodarone

4- Without this degree of LVH: - Flecainide, propafenone.

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Drugs for Pharmacologic Cardioversion of AF (Rhythm

control)

Drug Route of Admin. And Dosage

Amiodarone Oral: 1.2 to 1.8 g /day then 200 to 400 mg /d maintenance.

IV: 1.2 g /d IV continuous or in divided doses, then 200 to 400

mg /d maintenance

Dofetilide Oral: Creatinine clearance > 60 ml/min: 500 mcg BID

Flecainide Oral 200 to 300 mg

IV: 1.5 to 3 mg /kg over 10 to 20 min

Propafenone Oral: 450 to 600 mg

IV: 1.5 to 2 mg per kg over 10 to 20 min

Orally Administered Pharmacological Agents for Heart

Rate Control in Patients with AF

Drug Maintenance dose

Digoxin 0.125 to 0.375 mg daily

Metoprolol* 25 to 100 BID

Propranolol 80 to 360 mg daily in divided doses

Verapamil 120 to 360 mg daily in divided doses

Diltiazem 120 to 360 mg daily in divided doses

Anticoagulation of Patients with Atrial Fibrillation: Indications

Rheumatic mitral valve disease with recurrent or chronic atrial fibrillation.

Dilated cardiomyopathy with recurrent persistent or chronic atrial fibrillation.

Prosthetic valves.

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Prior to (>3 weeks) elective cardioversion of persistent or chronic atrial fibrillation, and also for 3

weeks after cardioversion (because of atrial stunning).

Coronary heart disease or hypertensive heart disease with recurrent persistent or chronic atrial

fibrillation

Atrial fibrillation in thyrotoxicosis (while awaiting long-term control; elective cardioversion)

Chronic or persistent lone atrial fibrillation, age >60 years

Controversial; or limited data

Coronary or hypertensive heart disease with normal left atrial size, after first episode of paroxysmal

atrial fibrillation

Elective cardioversion of atrial fibrillation of short duration (2-3 days) with normal left atrial size

Chronic or persistent lone atrial fibrillation, age <60 years

Not indicated

Lone atrial fibrillation, short paroxysms (<48 h)

Most clinical settings associated with short paroxysms (minutes to hours)

Relative contraindications

Difficulty controlling prothrombin times. Dementia

Malignancies, especially associated with bleeding risk

Prior major bleeding events. Uncontrolled hypertension

Treatment of Cardiac Arrhythmias with Catheter Ablative Techniques

Radiofrequency ablation destroys tissue by controlled heat production. Catheter ablation is used to treat

patients with four major tachyarrhythmias: atrial flutter/fibrillation, AV nodal reentry, accessory

pathways and ventricular tachycardia.

VENTRICULAR TACHYCARDIA

Specific Forms of Ventricular Tachycardia

Duration: Salvo (3-5 impulses)

Nonsustained VT: (6 impulses, up to 29 seconds)

Sustained VT: (>30 seconds)

The electrocardiographic diagnosis of ventricular tachycardia is suggested by the occurrence of a series

of three or more bizarrely shaped premature ventricular complexes whose duration exceeds 120 msec,

with the ST-T pointing opposite to the major QRS deflection.

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The rates range from 70 to 250 beats/min. Ventricular tachycardia can be sustained, defined arbitrarily

as lasting longer than 30 sec or requiring termination because of hemodynamic collapse, or

nonsustained (Unsustained), when it stops spontaneously in less than 30 sec.

Ventricular tachycardia (Wide QRS tachycardia)

Management: Intravenous lidocaine or amiodarone, followed by an infusion of the successful drug. If

the arrhythmia does not respond to medical therapy, electrical DC cardioversion can be employed.

Ventricular tachycardia in a patient with right ventricular dysplasia.

CONGENITAL LONG QT INTERVAL SYNDROME

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The normal QT interval is .43 sec. The congenital long QT interval syndrome, which is present

persistently from childhood, is characterized by the presence of long QT intervals on the standard 12-

lead ECG. The affected patients are prone to episodes of torsade de pointes (ventricular tachycardia

with special polymorphic configuration), which may cause transient light-headedness or syncope or

sudden cardiac death. Arrhythmias may occur at rest, under emotional stress, or with exercise.

ACQUIRED LONG QT INTERVAL SYNDROME

Causes: Antiarrhythmic drugs as quinidine. There is a growing list of other drugs that may prolong the

QT interval, and establish susceptibility to torsade de pointes. These include the phenothiazines, certain

antibiotics, pentamidine, cocaine, and terfenadine, among others.

Management of Congenital Long QT Interval Syndrome: Long-term therapy includes B-adrenergic

blockade. Placement of an ICD should be considered for patients with resistant arrhythmias.

CARDIOVERSION AND DEFIBRILLATION

Differences between cardioversion and defibrillation:

Cardioversion Defibrillation

Elective Emergency

Synchronized Non-synchronized

For AF, A. flutter, SVT, VT For V. fibrillation

50, 100, 150, 200 Joules Start by 200 Joules

Need sedative first Patient is unconscious

VENTRICULAR FLUTTER AND FIBRILLATION

MANAGEMENT: Immediate nonsynchronized DC electrical shock using 200 to 360 joules is

mandatory treatment for ventricular fibrillation. Cardiopulmonary resuscitation is employed only until

defibrillation equipment is ready. Time should not be wasted with cardiopulmonary resuscitation

maneuvers if electrical defibrillation can be done promptly.

The Implantable Cardioverter Defibrillator (ICD)

Apparatus (pacemaker) that gives electric shock if the patient develops ventricular fibrillation. The

pacemaker is inserted in the sub-pectoral area.

ICD indications

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A. Cardiac arrest not due to acute ischemia or infarction or reversible causes.

B. Documented sustained VT with hemodynamic compromise.

C. Syncope of unknown origin in structural heart disease patients with inducible sustained VT.

D. Cardiomyopathy ischemic or non-ischemic with ejection fraction 30% or lower (MADIT II results).

AV HEART BLOCK

Heart block is a disturbance of impulse conduction that can be permanent or transient, owing to

anatomical or functional impairment.

The conduction disturbance is classified by severity in three categories.

During first degree heart block, conduction time is prolonged but all impulses are conducted (P-R

interval > 0.2 sec.).

Second degree heart block occurs in three forms:

Mobitz type I (Wenckebach) and type II; and persistent 2:1 block.

Mobitz Type I heart block is characterized by a progressive lengthening of the conduction time until an

impulse is not conducted (Fig).

Mobitz Type II heart block denotes occasional (Mobitz II) or repetitive sudden block of conduction of

an impulse without prior measurable lengthening of conduction time. When no impulses are conducted,

complete or third degree block is present.

Mobitz type I (Wenckebach) block

Mobitz Type II second degree heart block

COMPLETE AV BLOCK

121

ELECTROCARDIOGRAPHIC RECOGNITION: Complete AV block occurs when no atrial activity

conducts to the ventricles and therefore the atria and ventricles are controlled by independent

pacemakers. Thus, complete AV block is one type of complete AV dissociation.

The ventricular focus is usually located just below the region of block, which can be above or below

the His bundle bifurcation. The ventricular rate of acquired complete heart block is less than 40

beats/min but may be faster in congenital complete AV block.

CLINICAL FEATURES. Block proximal to the His bundle generally exhibits normal QRS complexes

and rates of 40-60 beats/min because the escape focus that controls the ventricle arises in or near the

His bundle.

Causes: Surgery, electrolyte disturbances, endocarditis, tumors, Chagas' disease, rheumatoid nodules,

calcific aortic stenosis, myxedema, polymyositis, infiltrative processes (such as amyloid, sarcoid, or

scleroderma). In the adult, drug toxicity, coronary disease, and degenerative processes appear to be the

most common causes of AV heart block.

COMPLETE AV BLOCK

MANAGEMENT: Temporary or permanent pacemaker insertion is indicated in patients with

symptomatic bradyarrhythmias. Vagolytic agents such as atropine (novatropine 15 drops every 8 hours)

are useful, while catecholamines such as isoproterenol (Allupent syrup 5 ml every 8 hours) can be used

transiently to treat patients who have heart block. The use of transcutaneous pacing is preferable.

ELECTROPHYSIOLOGIC STUDY

EP study is an invasive procedure in which intracardiac electrode catheters are used to evaluate cardiac

arrhythmias and to select various therapeutic options.

Indications of EPS:

Diagnostic:

Aborted SCD (sudden cardiac death). - Syncope of undetermined cause.

Recurrent WCT (wide complex tachycardia). - Ventricular tachycardia.

Recurrent tachycardia with WPW syndrome.

Symptomatic refractory NCT (narrow complex tachycardia).

122

Therapeutic:

Catheter ablation for AVNRT (AV nodal reentrant tachycardia), WPW (Wolff Parkinson White

syndrome), VT (Ventricular Tachycardia), Atrial fibrillation.

Acute termination of hemodynamically unstable tachycardias.

CARDIAC PACEMAKERS

Cardiac pacemakers are devices either implanted permanently or inserted temporarily, consisting of a

pulse generator and an electrode catheter that is placed transvenously into the right ventricle and/or

atrium. Small electrical impulses, generated by the pulse generator and delivered via the electrode

catheter depolarize the heart. Pacemakers are widely used for treating bradyarrhythmias but can also be

useful for treatment of some tachyarrhythmias.

Temporary pacing is indicated for symptomatic second or third degree heart block caused by transient

drug intoxication or electrolyte imbalance in the setting of an acute MI, CHB, or Mobitz II second

degree AV Block. Symptomatic sinus bradycardia, AF with a slow ventricular response.

Indications for permanent pacemaker implantation:

Symptomatic bradycardia, due to either sinus node dysfunction or AV nodal block, in absence of a

reversible cause, constitutes a class I indications for permanent pacing. Asymptomatic conditions that

are also considered class I indications for permanent pacing include:

4- 3rd degree AV Block .

5- Persistent advanced 2nd degree or 3rd degree AVB after acute MI with demonstrated block in His-

Purkinje system (BBB).

6- Chronic bifascicular or trifascicular block with intermittent type II second or third degree AV

Block.

Pacing modalities: a four-letter alphabetic code is used to identify pacing modalities. The first initial

defines the chamber that is paced (V: ventricle, A: atrium, D: dual chamber). The second identifies the

chamber that is sensed (V, A, D), the third indicates the response to sensed event (I: inhibited, T:

triggered, D: dual function), and the fourth when present, denotes, R: rate responsive node. VVI &

DDD modes are used most commonly. VVI units pace and sense ventricle and a sensed (native) event

inhibits the ventricular stimulus. DDD units, pace and sense both chambers, events sensed in the

atrium inhibit the atrial stimulus and trigger a ventricular response after an appropriate interval,

where as ventricle-sensed events inhibit ventricular and atrial outputs.

123

Antiarrhythmic Drugs

Class Mode of

Action

Drugs Indication Dose Side Effects

Class

IA

Reduces

rate of

entry of

sodium

into the

cell

Quinidine

(Quinidine)

For

supraventricular

and ventricular

arrhythmias

including

conversion of AF or

A flutter, SVT, VT

600 – 1000

mg/day

Prolongation of QT

interval, risk of

Torsade de pointes.

Quinidine syncope,

quinidine induced

sudden death.

Diarrhea, vomiting

Procainamide

(Pronestyl)

Is effective

against

supraventricular

and ventricular

arrhythmias

2-6

mg/min

IV. 350-

1000 mg q

6 h PO

SLE like

syndrome,

prolonged QT,

nausea, rash,

myalgia,

Disopyramid

e (Norpace)

Is effective

against

supraventricular

and ventricular

arrhythmias

100-400

mg q 8 h

Worsening of

heart failure,

anticholinergic

actions as urine

retention. Avoid

in pts with

glaucoma

Class

IB

Lidocaine

(Zylocain)

Ventricular

arrhythmias only

1-4 mg/min

IV (50-150

mg IV

loading

dose)

Confusion,

convulsions

Mexiletine

(Mexitil)

Ventricular

arrhythmias only

150-300

mg q 6-8 h

Confusion,

tremor,

bradycardia,

hypotension

Class

IC

Flecainide

(Tambocor)

Is very effective

for ventricular

and

supraventricular

tachycardias

100-200

mg q 12 h

PO

Aggravation of

arrhythmia

(proarrhythmia),

negative inotropic

effect, depression

of sinus node

124

Propafenone

(Rytmonorm)

Has a rule in

treatment of many

types of

arrhythmias

including

supraventricular

arrhythmias

150-300 mg

q 8-12 h

Negative inotropic

effect

Class

II

Beta

adrenergi

c

blockers

e.g.

Propranolol

(Inderal),

Atenolol,

Bisoprolol,

Carvedilol

For premature

beats atrial and

ventricular, for

torsade de

pointes,

10-200 mg

q 8 h PO

Bradycardia,

hypotension, heart

failure, intermittent

claudication,

worsening of

asthma, impotence

Class

III

Prolong

action

potential

duration

Amiodarone

(Cordarone)

Life-threatening

ventricular

arrhythmias,

conversion and

slowing of atrial

fibrillation,

AVNRT,

tachycardias

associated with

WPWs

200-400

mg q 6-8 h

Corneal deposits,

photosensitivity,

skin pigmentation,

thyroid

disturbances (hypo

& hyperfunction),

alveolitis, liver

enzyme elevation

Sotalol

(Betacor)

Effective in

supraventricular

and ventricular

arrhythmias

80-160 mg

x 2-3 PO

Torsade de

pointes,

bronchospasm in

asthmatic patients

Class

IV

Calcium

antagonis

ts

Verapamil

(Isoptin)

Diltiazem

Slow the

ventricular rate in

AF or flutter, treat

and prevent

AVNRT

0.2 Mg/kg

IV 40-160

mg q 6-8 h

PO

60-120 mg

q 6-8 h PO

Constipation,

edema of LL,

negative inotropic

effect

Unclas

sified

Activates

K+

channels

Adenosine

(Adenocore)

Is very effective

for the acute

conversion of

paroxysmal SVT

6-18 mg

IV rapidly

Contraindicated

in sick sinus s., or

2nd

or 3rd˚

heart

block. Antidote is

theophylline

Enhances

central

and

Digoxin

(Lanoxin)

Slow ventricular

rate in AF, flutter

0.5 – 1 mg

IV or

0.125 –

Bradycardias and

tachycardias

(atrial, junctional,

125

periphera

l vagal

tone

0.25 mg /d

PO

vent.

tachycardia),

nausea, vomiting

Note: there are new two important antiarrhythmic drugs: Dronedarone (Multaq), and Vernakalant.

Samir Rafla: Principles of Cardiology pages 62-86

HEART FAILURE

Heart Failure means that the heart cannot pump sufficient blood for body needs.

Classification of heart failure:

A: 1. Left-sided heart failure.

2. Right-sided heart failure.

3. Combined left and right sided heart failure.

B: 1. Systolic heart failure.

2. Diastolic heart failure (heart failure with preserved systolic function.

C: 1. Forward failure (eg cardiogenic shock).

2. Backward failure (pulmonary congestion and pulmonary edema).

D: 1. Low output failure.

2. High output failure (eg in hyperthyroidism, anemia...)

The pathophysiology heart of heart failure

The causes of heart failure: The heart fails either because it is subjected to an overwhelming load, or

because the heart muscle is disordered:

A volume load is imposed by disorders which demand that the ventricle expels more blood per

minute than is normal. Examples include thyrotoxicosis and anemia, in which the total cardiac output is

increased; and mitral regurgitation and aortic regurgitation, in which the left ventricle has to expel not

only the normal forward flow into the aorta but also the large volume of regurgitated blood as well.

A pressure load is imposed by disorders which increase resistance to outflow from the ventricles

(eg systemic hypertension and by aortic stenosis).

Disorders of myocardial function result not only from diminished contractility but also from loss of

contractile tissue, as occurs in myocardial infarction. This is the commonest cause of heart failure. An

additional factor in this condition is a paradoxical movement of infarcted muscle which further

increases the work of the remaining myocardium.

126

Dilatation of the heart - increase in end-diastolic volume

In response to a volume load, the heart dilates, i.e. the ventricular volume is increased. Up to a point,

dilatation is a normal and efficient response.

Hypertrophy of the heart

When the ventricle has to face a chronic increase of pressure load, such as that imposed by arterial

hypertension, aortic stenosis or pulmonary hypertension, the myocardium hypertrophies, i.e. it

increases in weight as a result of an enlargement of individual muscle fibres.

Cellular changes in heart failure: Changes include:

Abnormal calcium metabolism: Heart failure results in changes in excitation contraction coupling.

• Changes in myocardial gene expression.

In some types of heart failure, a process of cell self-destruction may be initiated, resulting in further

loss of myocytes and progressive impairment of ventricular function. The process of ‘programmed cell

death’ is termed apoptosis.

Neuroendocrine response to heart failure

Cardiac failure activates several components of the neuroendocrine system, which play an important

intermediary role in its clinical manifestations:

Sympathetic nervous system. Activation of the sympathetic nervous system results in an increase in

myocardial contractility, heart rate, and vasoconstriction of arteries and veins. Although this may be

beneficial in maintaining blood pressure, it is adverse in so far as it increases preload, afterload, and

myocardial oxygen requirement. There is also an increased plasma noradrenaline (norepinephrine), but

myocardial catecholamines are reduced.

Renin-angiotensin-aldosterone systems. Both the fall in cardiac output itself and the increase in

sympathetic tone reduce effective blood flow to the kidney and, consequently, increase renin secretion.

As a result, there is a rise in angiotensin II levels, which leads directly to vasoconstriction and

indirectly, by stimulating aldosterone secretion, to sodium retention and the expansion of blood

volume. This is advantageous in so far as increasing preload helps to maintain stroke volume by the

Starling mechanism, but it does so at the expense of circulatory congestion.

127

Fig: some of the neuroendocrine and renal responses to cardiac failure

Atrial natriuretic peptide (ANP). Distension of the atria leads to the release of this peptide which

has natriuretic and vasodilator properties. Levels of ANP are elevated in patients with heart failure and

correlate with functional class. In addition, ANP has been suggested as a screening test to aid in the

diagnosis of heart failure.

Heart failure results in local vasoconstriction. This is partly a result of a reduced responsiveness to

local vasodilators (endothelial derived relaxing factor = nitric oxide) and to increased levels of the local

vasoconstrictor, endothelin. A role for endothelin antagonists in the treatment of heart failure has been

suggested.

Regional circulations in cardiac failure

There is a redistribution of blood flow to different organs and tissues in cardiac failure. Salt and water

retention

An almost invariable feature of cardiac failure is the retention of sodium and water. This leads to a

substantial increase in extracellular and plasma volume and plays a large part in the production of the

clinical features of cardiac failure.

Raised venous pressure in cardiac failure

When the left ventricle fails the pulmonary venous pressure rises, and when the right ventricle fails the

pressure rises in the systemic veins. The increased blood volume resulting from sodium and water

128

retention contributes to the venous return and is thus a factor in producing the raised venous pressure,

as is venoconstriction.

The effect of Left ventricular failure on the Lungs

When the left ventricle fails, the diastolic pressure in the left ventricle rises and with it the left atrial

pressure. Since the pulmonary veins and capillaries are in continuity with the left atrium, the pressures

in these vessels rise concomitantly. As failure advances, the left atrial pressure progressively increases

from its normal level of 5—10 mmHg to 25—30 mmHg. The hydrostatic pressure in the capillaries

may lead to an exudation of fluid from the capillaries into the alveolar walls and alveoli. The

pulmonary congestion caused by the high pulmonary venous pressure and by the changes in the

alveolar walls makes the lung more rigid (less compliant). As a result of this, more work must be done

by the respiratory muscles to move a given volume of air.

Arrhythmias in heart failure

Patients with heart failure have a high incidence of sudden death. The majority of deaths are thought to

be due to ventricular tachycardia or ventricular fibrillation.

Arrhythmia prevention in patients with heart failure is a particular problem. The efficacy of

antiarrhythmic drugs is reduced and there is, moreover, an increased incidence of proarrhythmic side-

effects. In addition, most antiarrhythmic drugs have negative inotropic effects.

CLINICAL SYNDROMES OF HEART FAILURE

Left heart failure

Aetiology

The features of left heart failure develop when there is a major obstruction to outflow from the left

atrium (e.g. mitral stenosis) or when the left ventricle can no longer cope with the demands upon it.

The common causes of left ventricular failure are:

• myocardial infarction

• systemic hypertension

• valvular heart disease

• Cardiomyopathy.

Clinical features: The clinical features of left-sided cardiac failure are largely the consequence of

pulmonary congestion. The symptoms are:

129

• dyspnoea on exertion

• orthopnoea and paroxysmal nocturnal dyspnoea

• acute pulmonary oedema.

The physical signs of left ventricular failure may include:

• pulmonary crepitations

• third heart sound

• pleural effusion

• pulsus alternans — alternate large and low volume indication of severe left ventricular failure.

Investigations

• The chest radiograph may show features of pulmonary venous congestion, particularly of the upper

lobe, interstitial oedema and alveolar oedema.

• The electrocardiogram (ECG) may be of value although it does not provide direct evidence of left

heart failure. For example, it is unusual for hypertension or aortic valve disease to lead to the symptoms

of left heart failure without producing ECG evidence of left ventricular hypertrophy first. Again, it is

unusual for coronary artery disease to lead to left heart failure if the ECG is normal. This is not,

however, true of mitral regurgitation.

• Echocardiography: To assess left ventricular end-diastolic and end-systolic dimensions; systolic

function (Ejection Fraction). Echocardiography is also important in the exclusion of other potentially

treatable causes of heart failure such as aortic stenosis or mitral regurgitation.

Atrial natriuretic peptide (ANP) levels and the closely related brain natriuretic peptide (BNP) are

elevated in patients with heart failure.

Differential diagnosis

The dyspnoea of left heart failure is more likely to be provoked by lying down flat. Patients with

dyspnoea due to pulmonary disease usually have a history of asthmatic attacks or of chronic cough and

sputum.

Paroxysmal nocturnal dyspnoea and acute pulmonary oedema may be difficult to differentiate from

acute respiratory attacks. The latter are commonly associated with bronchospasm and purulent sputum.

In contrast, the patient with acute pulmonary oedema is usually free of pulmonary infection, has fine

crepitations rather than rhonchi and is liable to cough up pink frothy sputum. Furthermore, examination

usually reveals the signs of left-sided heart disease. Correct diagnosis is of great importance because

the therapy of the two conditions is different. For example, morphine may be lethal in respiratory

130

failure, but invaluable in acute pulmonary oedema. The chest radiograph is also helpful in showing

signs of oedema or infection. In cases of doubt, estimation of the arterial CO2 tension is of value

because this is usually low in acute pulmonary oedema.

Right heart failure

Aetiology

- left ventricular failure with its consequent effects upon the pulmonary circulation.

- right ventricular infarction .

- pulmonary disease, particularly chronic bronchitis and emphysema pulmonary hypertension .

- pulmonary valve disease

- tricuspid regurgitation.

- Right sided venous congestion can result from tricuspid stenosis.

Clinical features: The characteristic features of right heart failure are:

• Elevated jugular venous pressure. In the normal individual, the venous pressure in the internal

jugular veins does not exceed 2 cm vertically above the sternal angle when the patient is reclining at

45° In right heart failure this figure is exceeded. Even if normal at rest, it rises on exercise.

• Hepatomegaly. If chronic, this may result in cirrhosis.

• Oedema. This is of the dependent type and usually most evident in the pretibial and ankle regions.

• Ascites. This may occasionally occur in patients with severe right heart failure.

• Tricuspid regurgitation. This can occur in patients with severe or long-standing right heart failure,

when right ventricular dilatation results in a functional incompetence of the tricuspid valve. A

prominent V wave may be seen in venous pulse and a pulsatile liver edge may be palpable.

Differential diagnosis: In patients presenting with isolated signs of right heart failure, the possibility of

pericardial constriction or tamponade should be considered as an alternative diagnosis.

Other clinical features of cardiac failure

There are a number of common but less specific features of cardiac failure:

• Fatigue is a frequent symptom which is difficult to evaluate.

• The nutrition of patients with cardiac failure is often good in the early stages, but cachexia sets in as

disability increases.

• In the very advanced case, cerebral symptoms may develop with dulling of consciousness,

confusion or changes in personality.

131

• Patients with cardiac failure are prone to develop venous thrombosis and pulmonary emboli are

common.

• Mild jaundice, due to hepatic congestion or cirrhosis, is quite frequent in right-sided heart failure.

• Proteinuria due to renal congestion is often present.

General management of cardiac failure

The principles of treating cardiac failure may be enumerated as follows:

• the correction or amelioration of the underlying disease

• the control of precipitating factors

• the reduction of demands on the heart by weight loss and the restriction of physical activity

• pharmacological therapy to modify the heart failure state and, particularly, to reverse the adverse

consequences of neuroendocrine and renal responses to heart failure.

The objectives of therapy are twofold: to alleviate the symptoms caused by heart failure and to improve

the prognosis.

The correction or amelioration of the underlying cause

When heart disease is due to such causes as thyrotoxicosis or hypertension, corrective treatment can be

started immediately. In congenital and rheumatic heart disease, surgical management is usually

required.

In the case of ischaemic heart disease, the cause of heart failure is generally previous myocardial

infarction rather than ongoing ischemia. Coronary revascularization procedures may be considered.

The control of complicating factors (Question: what are the causes of resistant HF or refractory

HF or acute on top of chronic HF):

Cardiac failure is often precipitated or exacerbated by factors superimposed on the underlying heart

disease. Amongst these are: - New myocardial infarction

• Arrhythmias especially atrial fibrillation. Infections, infective endocarditis.

• Uncontrolled hypertension. Pulmonary embolism. Anemia

• Excessive sodium intake. Stopped treatment or insufficient treatment.

• Over-exertion. Pregnancy. Multivalvular or Mutlivessel coronary disease.

• The recognition of precipitating factors is of great importance in management of heart failure,

because the correction of these conditions will often result in the improvement of symptoms.

Exercise

132

Rest reduces the demands on the heart and leads to a fall in venous pressure and a reduction in

pulmonary congestion. It allows a relative increase in renal blood flow and often leads to a diuresis.

However, bed rest also encourages the development of venous thrombosis and pulmonary embolism.

The degree of physical restriction necessary depends upon the severity of the cardiac failure. When

there is severe pulmonary congestion or peripheral oedema, a period of complete rest may be required.

Complete bed rest is seldom necessary for more than a few days, after which a gradual increase in

activity should be encouraged, depending upon the response.

In patients with lesser degrees of heart failure, regular exercise should be encouraged. Typical exercise

would be to recommend 20 to 30 mm walking three times per week.

Management of salt and water retention

Low salt diets effectively counteract cardiac failure. However, with the availability of potent diuretic

drugs, no extreme limitation of sodium intake is usually necessary.

PHARMACOLOGICAL THERAPY

Diuretics: The loop diuretics: furosemide (frusemide), bumetanide

These drugs prevent reabsorption at multiple sites including the proximal and distal tubules and the

ascending limb of the loop of Henle.

Thiazide diuretics

The main mechanism is the inhibition sodium reabsorption in the distal convoluted tubule. These drugs

sometimes cause hyperglycaemia and hyperuricaemia, and may precipitate diabetes and clinical gout.

Potassium-sparing diuretics:

This group comprises two classes of agent:

• Spironolactone. This drug is an aldosterone antagonist, providing a weak diuresis with a potassium-

sparing action.

• Amiloride and triamterene. These drugs inhibit sodium—potassium exchange in the distal tubule.

They have a weak diuretic effect.

Recently, spironolactone has been shown to confer an additional mortality benefit of approximately

30% in patients with severe heart failure.

Hyperkalaemia is a potential complication, particularly in patients with impaired renal function.

Particular caution is necessary when adding potassium retaining diuretics to ACE inhibitor therapy.

Spironolactone causes breast enlargement or pain in approximately 10% of men taking the drug.

Inhibitors of the renin-angiotensin system

133

Two types of pharmacological agent can be used to block the renin—angiotensin system:

• ACE inhibitors, which inhibit the conversion of angiotensin I to angiotensin II.

• Angiotensin II receptor blocking agents provide an alternative approach to inhibition of the rennin-

angiotensin system. They block the vasoconstrictor and other actions of angiotensin II.

ACE inhibitors

ACE inhibitors are indicated both for the treatment of symptoms and to improve prognosis in patients

with heart failure. In view of these benefits, ACE inhibitors should be prescribed, unless

contraindicated, in patients with symptomatic heart failure and in all patients, irrespective of symptoms,

with an ejection fraction of less than 40%.

Following myocardial infarction, ACE inhibitors are of particular value. They are indicated not only for

the treatment of failure, but also for the prevention of adverse remodeling.

ACE inhibitors are in general well tolerated. However, a number of problems may be encountered,

including hypotension, renal impairment and cough:

• First-dose hypotension can be minimized by reducing the dose of the ACE inhibitor on

commencing therapy and omitting diuretics for 1—2 days beforehand.

• ACE inhibitors occasionally cause deterioration of renal function. They are contraindicated in

patients with an initial creatinine level greater than 200 mmol/L (or creatinine > 2.6 mg/dl). Renal

function should be checked routinely 1—2 Weeks after commencing therapy.

• Cough is a potentially troublesome side-effect, occurring in up to 10% of patients.

Angiotensin II receptor blockers

In patients intolerant of ACE inhibitors there is evidence that angiotensin receptor blocking drugs do

improve prognosis.

Other vasodilators

The widespread applicability and indications for ACE inhibitors have reduced the importance of other

vasodilators in the management of heart failure.

Nitrate vasodilators remain of value in the management of acute left ventricular failure. Sublingual

glyceryl trinitrate can be administered in the acute phase and can be followed by an intravenous

infusion. Nitrates act predominantly as venodilators.

Hydralazine, by contrast, is predominantly an arterial dilator.

Beta-blockers

134

Excessive sympathetic stimulation may contribute to progression of heart failure in a number of ways,

including additional energy requirements, ventricular hypertrophy and arrhythmias. Beta-blockers

result in a reduction in mortality, of the order of 30%. The reduction in mortality relates substantially to

a reduction in sudden deaths, but beta-blockers also benefit symptoms and have been shown to reduce

hospitalizations for heart failure. Beta-blockers have been shown to benefit patients with class II and III

heart failure and to benefit selected patients with class IV heart failure. In general, the more severe the

degree of heart failure and the worse the prognosis of the patient, the greater the benefit to be gained

from beta-blockade.

• Patient selection is crucial. Beta-blockers should not be given in new onset or uncontrolled heart

failure. Patients presenting with acute heart failure or with an exacerbation of chronic heart failure

should be stabilized with diuretics and ACE inhibitors before initiating a beta-blocker. Bradycardia

(heart rate < 60) and hypotension (systolic blood pressure < 100) are relative contraindications and

require particularly careful monitoring on commencement of therapy.

• Low dose initiation of therapy is crucial.

• Slow upward dose titration with clinical monitoring. Titration should occur at intervals of not less

than 2 weeks. Dizziness, postural hypotension and worsening heart failure are all relatively common

and may require dose reduction or cessation of beta-blocker therapy.

Inotropic agents

Digitalis glycosides

Mechanisms of action

The inotropic action of digitalis is mediated through the sodium/potassium ATPase (sodium) pump, to

which it binds. The inhibition of this pump leads to an accumulation of intra-cellular sodium; because

of the sodium— calcium exchange system, this results in an increase in the amount of calcium

available to activate contraction. Digitalis also has sympathomimetic and parasympathetic (vagal)

effects. The latter is clinically important, in that it causes slowing of the sinus rate.

Indications: Digoxin is particularly indicated in patients with HF and atrial fibrillation, for its

beneficial effects to reduce ventricular response rate. In this setting it is an appropriate first line agent.

Other drugs: Ivapradine (procoralan) that reduces sinus node rate without diminishing myocardial

contractility. Trimetazidine (Vastarel) and Cardioton (Crataegus monogyna ).

VENTRICULAR RESYNCHRONIZATION THERAPY

There is growing evidence that some individuals with severe heart failure may be improved by

biventricular pacing to provide ventricular resynchronization.

135

In many patients with severe heart failure, left ventricular contraction becomes incoordinate. Delay in

the spread of the electrical impulse to different regions of the ventricle results in a dispersion of the

onset of contraction. As a consequence, the regions of the ventricle activated earliest may be relaxing

by the time later regions have started to contract. This results in an additional inefficiency of pump

function, responsible for an additional deterioration in ejection fraction and cardiac output.

Ventricular resynchronization pacing aims at pacing the septum and lateral wall of the left ventricle

simultaneously, thereby improving the synchrony of contraction. One electrode is placed in the right

ventricle, as for conventional pacing, and the other at the left free wall. Left ventricular pacing is

achieved via the coronary sinus. Simultaneous pacing at the two sites results in a narrowing of QRS

width and an improvement in cardiac output.

Criteria of selection of patients likely to benefit most from ventricular resynchronization pacing

include:

• Severe heart failure, New York Heart Association class III or IV

• Left bundle branch block

• QRS width greater than 120 ms

• Evidence of incoordinate left ventricular contraction on echocardiography.

ARRHYTHMIA MANAGEMENT

About 50% of patients with heart failure die from progressive heart failure. The other 50% die

suddenly as a result of ventricular arrhythmias.

Beta-blockade has been shown to dramatically reduce sudden deaths.

There is growing evidence to suggest a role for implantable defibrillators in this patient population.

Implantable defibrillators have been shown to significantly reduce mortality in patients with an

ejection fraction less than 30%.

As implantable defibrillators can be combined with ventricular resynchronization, there is likely to be a

growing role for device therapy in the management of patients with severe heart failure.

ACUTE LEFT VENTRICULAR FAILURE

Acute pulmonary oedema is a life-threatening emergency. Characteristically, the patient is extremely

breathless and frightened. The patient is unable to lie flat and prefers to sit bolt upright. In severe cases

they may cough up blood tinged, pink sputum.

Causes of acute left ventricular failure:

136

- Acute myocardial infarction.

- Atrial fibrillation and other tachyarrhythmias.

- Severe hypertension.

- Myocarditis

- Infective endocarditis with acute valve damage (incompetence).

- Chordal rupture.

- Cardiac tamponade

- Acute exacerbation of chronic heart failure (due to increased sodium intake, non-compliance with

medications (eg stopping digitalis), exacerbation of hypertension, acute arrhythmias, infection and/or

fever, pulmonary embolism, anemia and hemorrhage, thyrotoxicosis, pregnancy and child birth,

infective endocarditis with valve damage, rheumatic fever, physical emotion and stress, and prolonged

tachycardia or bradycardia).

Causes of non-cardiac pulmonary edema:

- Adult respiratory distress syndrome.

- Pulmonary embolism.

- Toxic gases.

- Gram negative septicemia (shock-lung).

- Diffuse pulmonary infections.

- Aspiration.

- Narcotic overdose especially parentral heroin.

- Lymphatic obstruction.

- Following cardio-pulmonary bypass.

- Hemorrhagic pancreatitis.

Clinical features

• The patient is tachypnoeic and distressed.

• Perspiring profusely.

• Systolic pressure is frequently elevated.

• A marked tachycardia is evident with a gallop rhythm on auscultation.

• Crackles and wheeze are heard throughout the chest.

Table: Differentiating Points Between Bronchial Asthma and Cardiac Asthma

Bronchial Asthma Cardiac Asthma

History of allergy usually present usually absent

137

Age usually young usually older

Cardiac lesion absent present (causing left heart failure)

Cough associated with viscid

sputum

associated with frothy pinkish

sputum

Chest examination mainly wheezes wheezes and crepitations

Eosinophilia usually present absent

Circulation time normal or short prolonged

Investigations

• Chest radiograph shows diffuse haziness due to alveolar fluid. Changes generally bilateral (Bat-

wing appearance) but occasionally may be unilateral.

• Blood gases. Arterial p02 falls. Initially pCO2 also falls due to breathing, but in the later stages

pCO2 may rise due to impaired exchange.

Management: Management of acute LVF:

• General. A venous line should be inserted and the patient should be monitored.

• Oxygen. This should be administered in high concentrations (60%) unless the patient has

concomitant airways disease.

• Diamorphine. The standard dose of diamorphine is 5 mg given intravenously.

• Diuretics. The patient should be given intravenous furosemide (frusemide). The usual dose would

be 40 mg, but this may be increased in patients already on diuretic therapy.

• Nitrates. Administration of a sublingual tablet of glyceryl trinitrate has an immediate effect of

lowering pulmonary pressures and reducing pulmonary oedema. This may be followed, if necessary, by

an infusion of the drug.

• Inotropic therapy. In cases of refractory pulmonary oedema, inotropic therapy should be

considered. Aminophylline 250 mg i.v. over 10 mm is frequently effective. Alternatively, patients may

be started on a dobutamine infusion, beginning at 5 µg/kg/min.

CARDIOGENIC SHOCK

The terms acute circulatory failure, low output state, and shock are used to describe a syndrome

comprising arterial hypotension, cold, moist and cyanosed extremities, a rapid weak pulse, a low urine

output and a diminished level of consciousness. This pattern can arise as a result of impaired cardiac

function, in which case, it is termed cardiogenic shock.

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This clinical pattern is common to a number of other disorders and cardiogenic shock must be

differentiated from other causes of shock, including:

• hypovolaemic shock, eg by haemorrhage and loss of fluid from burns, vomiting and diarrhoea

• septicaemic shock

• anaphylactic shock

• acute pancreatitis.

Shock is described as cardiogenic when it is clearly cardiac in origin. This may be due to many

different causes, including myocardial infarction, massive pulmonary embolism, dissecting aneurysm,

pericardial tamponade, rupture of a valve cusp, and arrhythmias; also pulmonary embolism. In

cardiogenic shock, the central venous pressure is usually raised, in contrast to hypovolaemic shock, in

which it is characteristically low.

Clinical features

• In the first stage of shock, there is a fall in cardiac output and blood pressure, due to either a

diminution in venous return or to an inability of the myocardium to expel an adequate stroke volume.

• As a consequence of the hypotension, there is a fall in renal blood flow, with oliguria.

• Reflex tachycardia occurs.

• Compensatory reflex arteriolar vasoconstriction further reduces blood flow to the kidneys,

abdominal viscera, muscle and skin. Vasodilatation of the cerebral and coronary vessels permits the

maintenance of a relatively good blood flow in these territories. If the vasoconstriction is sufficiently

great, the blood pressure may be kept at or close to normal levels but at the expense of producing tissue

hypoxia with consequent acidosis.

Management of cardiogenic shock

General management

If the patient is in severe pain or distress, opiates should be given intravenously (provided there is no

contraindication) and high-flow oxygen administered, preferably by a tight-fitting face mask making

use of the Venturi principle, or by mechanical ventilation. Unless there is pulmonary oedema, the

patient should be laid flat, with the legs slightly raised. A catheter should be introduced to measure

urinary output. Arterial blood gases and pH should be monitored. A Swan-Ganz balloon- tip catheter

should be used to obtain pulmonary artery and ‘pulmonary capillary wedge’ pressures if a cardiac or

pulmonary cause is known or suspected (recently this invasive monitoring was considered not

mandatory in some cases). As measurement of blood pressure by a sphygmomanometer is unreliable in

severe shock, direct arterial pressure monitoring should be undertaken, when possible.

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Correction of hypovolaemia

Although left ventricular filling pressures are most commonly elevated in patients with cardiogenic

shock, this is not always the case. Patients may have undergone a period of prior diuretic therapy

resulting in fluid depletion. Alternatively, in cases of right ventricular infarction the left ventricle may

be under filled. A Swan—Ganz catheter enables pulmonary artery wedge pressure to be estimated to

achieve an optimal pressure of between 18 and 20 mmHg. If the pressure is below this level, saline

should be administered to increase the wedge pressure and optimize cardiac output

Inotropic agents

These drugs enhance myocardial contractility but at the expense of increased oxygen consumption.

Dopamine and dobutamine are most frequently used.

The effects of dopamine, a natural precursor of noradrenaline (norepinephrine), depend upon the dose.

Administered intravenously in a dosage of 2—5 µg/kg/min, it causes dilatation of renal and mesenteric

vessels; at doses of 5—10 µg/kg/min, it increases myocardial contractility and cardiac output. At

higher doses, it causes vasoconstriction (it should not be infused directly into a peripheral vein as

leakage may cause local necrosis). Dopamine may induce nausea and vomiting, and can lead to an

excessive tachycardia and arrhythmias.

Dobutamine is a synthetic sympathomimetic agent whose predominant action is one of stimulating

activity. It is less likely to cause vasoconstriction or tachycardia than dopamine. It is given by

intravenous infusion at a rate of 2.5—10 µg/kg/min.

Mechanical support

The intra-aortic balloon pump is of value in acute myocardial infarction if shock has been caused by a

surgically correctable lesion, such as a ventricular septal defect or papillary muscle rupture.

REFRACTORY HEART FAILURE

Heart failure is termed refractory when it persists or deteriorates despite intensive therapy. Causes:

- Hyperthyroidism

- Anemia

- Recurrent pulmonary emboli

- Atrial fibrillation and other arrhythmias

- Multiple myocardial infarcts, multivessel coronary disease

- Hypertension uncontrolled

- Pneumonia, other infections, chronic obstructive pulmonary disease with exacerbations.

- Infective endocarditis

- Rheumatic activity

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- Pregnancy

- Constrictive pericarditis and endomyocardial fibrosis

- Left ventricular aneurysm

- The myocardium reached end stage with fibrosis, scars, and multiple infarcts.

Diagnosis and Treatment: according to cause.

ACUTE EXACERBATION ON TOP OF CHRONIC HEART FAILURE

Cause, diagnosis and treatment: Review above page --.

INFECTIVE ENDOCARDITIS

Infective endocarditic can occur in two ways:

1. When the heart valves and endocardium are damaged, organisms of low pathogenicity (e.g.

streptococcus viridans) can invade them and produce a slowly progressive infection, i.e. subacute

bacterial (or infective) endocarditis.

2. Normal valves and endocardium can be invaded by organisms of high pathogenicity (e.g.

staphylococcus aureus, pneumococcus, gonococcus) in the course of a fulminating septicemia

originating in another organ. In these cases the course is usually acute, i.e. acute bacterial (or infective)

endocarditis.

SITE OF INVOLVEMENT:

- Subacute infective endocarditis occur on top of a preexisting heart disease, e.g. chronic rheumatic

heart disease, congenital heart disease, etc. or on artificial (prosthetic) valve.

- It most commonly complicates mitral regurgitation, aortic stenosis, aortic regurgitation, calcific or

sclerotic aortic valve, ventricular septal defect, patent ductus arteriosus, bicuspid aortic valve or

artificial valves.

- It is less common in cases of Fallot’s tetralogy and pure mitral stenosis. It is very rare in cases of

atrial septal defect. Endocarditis of the tricuspid valve occurs in intravenous drug abusers who inject

drugs under septic conditions.

- It is more common in the left side of the heart than the right side.

ORGANISM:

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The most common causative organism is Streptococcus viridans. Less common are Staphylococcus

aureus and enterococcus. Fungal endocarditis is caused by candida or aspergillus and it is common in

patients receiving large doses of antibiotic, steroids or cytotoxic drugs. It is also common in drug

abusers and in infections of prosthetic materials placed in the circulation e.g. indwelling catheters,

prosthetic valves, etc.

SOURCE OF INFECTION:

Bacteremia, by dental extraction and other dental procedures, urinary catheterization, labor, abortion,

upper respiratory infection, etc. But often the source of infection is unknown.

CLINICAL PICTURE:

1. Onset is usually insidious with fever, sweating, arthralgia, malaise, toxic anemic look.

2. Persistent fever is usually of low grade but varies, with pallor and earthy “café au lait” facies.

3. The heart may show the following:

a. There is always evidence of pre-existing heart disease.

b. Change or increase of existing murmurs or the development of new murmurs due to destruction of

heart valves by the infection.

c. In advanced cases heart failure results from toxic myocarditis and the effects of valvular defects.

4. The spleen is moderately enlarged and tender in most cases.

5. Clubbing of the fingers occurs after 5-6 weeks.

6. Involvement of the kidney may result in

a. Hematuria, whether microscopic or macroscopic, and proteinuria occurs in most cases.

b. A picture of immune complex acute glomerulonephritis.

c. Renal failure may complicate advanced cases.

d. Renal infarction may cause pain in the loins and hematuria.

7. Embolism may involve any organ, e.g. spleen, kidney, limbs, brain, retina, mesenteries. It produces

variable signs and symptoms of infarction depending on the site and size of the embolus and may cause

mycotic aneurysm. Pulmonary embolism may complicate endocarditis involving the right cardiac

chambers. Retinal emboli or immune complexes cause areas of hemorrhage with pale center (Roth

spots).

8. Neurologic manifestations include:

a. Infective endocarditis sometimes presents as cerebral embolism. Septic infarction may result in

cerebral abscess.

b. Cerebral or subarachnoid hemorrhage may result from rupture of a mycotic aneurysm.

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c. A picture stimulating meningitis or encephalitis may occur.

9. Skin lesions include:

a. The commonest are petechial hemorrhages. They appear as crops of small brown red spots that do

not disappear on pressure. They are most commonly found in the chest, neck, palate and conjunctiva.

b. Osler nodules are tender intracutaneous nodules usually found in the pulps of finger and in the

thenar and hypothenar eminence.

c. More rarely streaks of hemorrhage under the nails (splinter hemorrhage), or flat erythematous

macules in the palms and soles (Janeway lesions) may be seen.

DIAGNOSIS:

1. Bacterial endocarditis must always be suspected in any patient with a murmur or a known heart

disease who develops an unexplained or prolonged fever.

2. When bacterial endocarditis is suspected the diagnosis is confirmed by blood culture. Culture

must be done for aerobic and anaerobic bacteria and fungi and must be incubated for up to 3 weeks to

allow slow growing organisms to emerge. When an organism is isolated its antibiotic sensitivity must

be tested. Cultures may also be negative in fungal endocarditis and in infections by fastidious or slowly

growing organisms, but most commonly in those who recently received antibiotic therapy.

3. Echocardiography is essential and may show vegetations on the valves, an abscess on the valve

ring, and the underlying heart disease. Transesophageal echocardiography is more sensitive than the

transthoracic technique.

4. Urine examination commonly shows microscopic or macroscopic

5. Hematuria. Red cell casts and heavy proteinuria indicate the presence of immune complex

glomerulonephritis.

6. Blood examination shows elevated ESR, anemia and sometimes leukocytosis.

Duke criteria for diagnosis of IE:

I- Definite IE:

A- Pathological criteria:

1- Microorganisms--------culture or histology or

2- Pathological lesions----vegetations, abscess confirmed by histology

b- Clinical criteria: (2 major, or 1 major +3 minor, or 5 minor)

*Major criteria:

1- Positive blood culture for IE.

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2- Evidence of endocardial involvement:

Positive echo---vegetations, abscess, new dehiscence of prosthetic valve, or new or worsened valvular

regurgitation.

* Minor criteria:

1- Predisposition: heart disease, or IV drug use.

2- Fever: >38 c

3- Vascular phenomena:

4- Immunologic phenomena:

5- Microbiological evidence:

6- Echo findings:

II- Possible IE:

Findings consistent with IE that fall short of (definite) but not (rejected)

III- Rejected: Firm alternative diagnosis for manifestations of endocarditis or

Resolution of manifestations of endocarditis, antibiotic for 4 days or less

COMPLICATIONS:

The most important complications are:

1. Arterial emboli may cause hemiplegia, aphasia, infarction of the bowel, kidney, lung, or ischemia

or gangrene of arm or leg.

2. Destruction or perforation of cardiac valves. Large vegetations may interfere with prosthetic valve

function.

3. Congestive cardiac failure.

4. Uremia.

DIFFERENTIAL DIAGNOSIS:

The most important differential diagnosis is:

a. Rheumatic activity

b. Intercurrent infections and fevers.

1. Sometimes, it may be very difficult to differentiate infective endocarditis from rheumatic activity.

The presence of enlarged spleen, clubbing of the fingers, petechiae, embolic manifestations and

hematuria points towards infective endocarditis. On the other hand, the appearance of fleeting arthritis,

erythema marginatum, subcutaneous nodules and evidence of previous streptococcal throat infection

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favours the diagnosis of rheumatic activity. When in doubt, multiple blood cultures should be done and

the patient should be treated as infective endocarditis.

2. Infective endocarditis must be differentiated from other causes of fever in patients with previous

heart disease, e.g. specific infections as brucella, typhoid, tuberculosis etc., connective tissue diseases,

lymphomas, etc. These diseases are diagnosed by their specific signs and tests.

PROPHYLAXIS:

1. Amoxicillin (2 gm) prophylaxis should be given orally to all patients with rheumatic or

congenital heart disease, one hour before all procedures that may result in bacteremia. These include

dental extraction, tonsillectomy, urethral catheterization, prostatic massage, delivery, abortion, etc. In

cases of penicillin sensitivity clindamycin, erythromycin or vancomycin can be used.

2. In patients at very high risk of developing endocarditis (e.g. those with prosthetic valves or

history of previous endocarditis), more intensive prophylaxis is required. Ampicillin 2 gm IV or IM

should be given 30 minutes before the procedure together with gentamycin 1.5 mg/kg. This should be

repeated 6 hours later.

TREATMENT:

- Streptococcus viridans is usually penicillin sensitive and is eradicated by giving 3 million units of

penicillin l.V. every 4 hours (18 million per day) for 3-4 weeks together with gentamycin 1 mg/kg

every 8 hours I.M. for the first two weeks of treatment. For penicillin resistant streptococci and for

penicillin allergic patient vancomycin is given 15 mg/kg/12 hours + gentamycin.

1. Enterococcus is less sensitive and needs 20-40 million units of penicillin G I.V. plus gentamycin

3 mg/kg I.M. daily. Ampicillin in a dosage of 2 gm every 4 hours I.M. may be substituted for

penicillin. The effective dose should be maintained for at least 6-8 weeks.

2. Staphylococcus: most strains secrete penicillinase and these should be treated by penicillinase-

resistant penicillins (nafcillin or oxacillin) or cefazolin or vancomycin 15 mg/kg/12 hours for 6 weeks.

An aminoglycoside antibiotic and rifampicin 300 mg twice daily may be added.

3. Culture-negative endocarditis patients should be treated as enterococcal endocarditis.

Surgery will also be needed if:

a. Infection cannot be controlled.

b. An abscess forms around the valve ring.

c. Very big vegetations that may cause major emboli.

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d. Congestive heart failure develops due to destruction or perforation of a valve.

Duke Criteria for Infective Endocarditis

Major criteria :

Positive blood culture for Infective Endocarditis

Typical microorganism consistent with IE from 2 separate blood cultures, as noted below:

• viridans streptococci, Streptococcus bovis, or HACEK group, or

• community-acquired Staphylococcus aureus or enterococci, in the absence of a primary focus

or Microorganisms consistent with IE from persistently positive blood cultures defined as:

• 2 positive cultures of blood samples drawn >12 hours apart, or

• all of 3 or a majority of 4 separate cultures of blood (with first and last

sample drawn 1 hour apart)

Evidence of endocardial involvement Positive echocardiogram for IE defined as :

• oscillating intracardiac mass on valve or supporting structures, in the path of regurgitant jets, or on implanted material in the absence of an alternative anatomic explanation, or

• abscess, or

• new partial dehiscence of prosthetic valve or

New valvular regurgitation (worsening or changing of preexisting murmur not sufficient) Minor criteria :

Predisposition: predisposing heart condition or intravenous drug use

Fever: temperature > 38.0° C (100.4° F)

Vascular phenomena: major arterial emboli, septic pulmonary infarcts, mycotic aneurysm, intracranial hemorrhage, conjunctival hemorrhages, and Janeway lesions

Immunologic phenomena: glomerulonephritis, Osler's nodes, Roth spots, and rheumatoid factor

Microbiological evidence: positive blood culture but does not meet a major criterion as noted above¹ or serological evidence of active infection with organism consistent with IE

Echocardiographic findings: consistent with IE but do not meet a major criterion as noted above

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Samir Rafla: Principles of Cardiology pages 62-86

HEART FAILURE

Heart Failure means that the heart cannot pump sufficient blood for body needs.

Classification of heart failure:

A: 1. Left-sided heart failure.

4. Right-sided heart failure.

5. Combined left and right sided heart failure.

B: 1. Systolic heart failure.

2. Diastolic heart failure (heart failure with preserved systolic function.

C: 1. Forward failure (eg cardiogenic shock).

2. Backward failure (pulmonary congestion and pulmonary edema).

D: 1. Low output failure.

2. High output failure (eg in hyperthyroidism, anemia...)

The pathophysiology heart of heart failure

The causes of heart failure: The heart fails either because it is subjected to an overwhelming load, or

because the heart muscle is disordered:

A volume load is imposed by disorders which demand that the ventricle expels more blood per

minute than is normal. Examples include thyrotoxicosis and anemia, in which the total cardiac output is

increased; and mitral regurgitation and aortic regurgitation, in which the left ventricle has to expel not

only the normal forward flow into the aorta but also the large volume of regurgitated blood as well.

A pressure load is imposed by disorders which increase resistance to outflow from the ventricles

(eg systemic hypertension and by aortic stenosis).

Disorders of myocardial function result not only from diminished contractility but also from loss of

contractile tissue, as occurs in myocardial infarction. This is the commonest cause of heart failure. An

additional factor in this condition is a paradoxical movement of infarcted muscle which further

increases the work of the remaining myocardium.

Dilatation of the heart - increase in end-diastolic volume

In response to a volume load, the heart dilates, i.e. the ventricular volume is increased. Up to a point,

dilatation is a normal and efficient response.

Hypertrophy of the heart

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When the ventricle has to face a chronic increase of pressure load, such as that imposed by arterial

hypertension, aortic stenosis or pulmonary hypertension, the myocardium hypertrophies, i.e. it

increases in weight as a result of an enlargement of individual muscle fibres.

Cellular changes in heart failure: Changes include:

Abnormal calcium metabolism: Heart failure results in changes in excitation contraction coupling.

• Changes in myocardial gene expression.

In some types of heart failure, a process of cell self-destruction may be initiated, resulting in further

loss of myocytes and progressive impairment of ventricular function. The process of ‘programmed cell

death’ is termed apoptosis.

Neuroendocrine response to heart failure

Cardiac failure activates several components of the neuroendocrine system, which play an important

intermediary role in its clinical manifestations:

Sympathetic nervous system. Activation of the sympathetic nervous system results in an increase in

myocardial contractility, heart rate, and vasoconstriction of arteries and veins. Although this may be

beneficial in maintaining blood pressure, it is adverse in so far as it increases preload, afterload, and

myocardial oxygen requirement. There is also an increased plasma noradrenaline (norepinephrine), but

myocardial catecholamines are reduced.

Renin-angiotensin-aldosterone systems. Both the fall in cardiac output itself and the increase in

sympathetic tone reduce effective blood flow to the kidney and, consequently, increase renin secretion.

As a result, there is a rise in angiotensin II levels, which leads directly to vasoconstriction and

indirectly, by stimulating aldosterone secretion, to sodium retention and the expansion of blood

volume. This is advantageous in so far as increasing preload helps to maintain stroke volume by the

Starling mechanism, but it does so at the expense of circulatory congestion.

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Fig: some of the neuroendocrine and renal responses to cardiac failure

Atrial natriuretic peptide (ANP). Distension of the atria leads to the release of this peptide which

has natriuretic and vasodilator properties. Levels of ANP are elevated in patients with heart failure and

correlate with functional class. In addition, ANP has been suggested as a screening test to aid in the

diagnosis of heart failure.

Heart failure results in local vasoconstriction. This is partly a result of a reduced responsiveness to

local vasodilators (endothelial derived relaxing factor = nitric oxide) and to increased levels of the local

vasoconstrictor, endothelin. A role for endothelin antagonists in the treatment of heart failure has been

suggested.

Regional circulations in cardiac failure

There is a redistribution of blood flow to different organs and tissues in cardiac failure. Salt and water

retention

An almost invariable feature of cardiac failure is the retention of sodium and water. This leads to a

substantial increase in extracellular and plasma volume and plays a large part in the production of the

clinical features of cardiac failure.

Raised venous pressure in cardiac failure

When the left ventricle fails the pulmonary venous pressure rises, and when the right ventricle fails the

pressure rises in the systemic veins. The increased blood volume resulting from sodium and water

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retention contributes to the venous return and is thus a factor in producing the raised venous pressure,

as is venoconstriction.

The effect of Left ventricular failure on the Lungs

When the left ventricle fails, the diastolic pressure in the left ventricle rises and with it the left atrial

pressure. Since the pulmonary veins and capillaries are in continuity with the left atrium, the pressures

in these vessels rise concomitantly. As failure advances, the left atrial pressure progressively increases

from its normal level of 5—10 mmHg to 25—30 mmHg. The hydrostatic pressure in the capillaries

may lead to an exudation of fluid from the capillaries into the alveolar walls and alveoli. The

pulmonary congestion caused by the high pulmonary venous pressure and by the changes in the

alveolar walls makes the lung more rigid (less compliant). As a result of this, more work must be done

by the respiratory muscles to move a given volume of air.

Arrhythmias in heart failure

Patients with heart failure have a high incidence of sudden death. The majority of deaths are thought to

be due to ventricular tachycardia or ventricular fibrillation.

Arrhythmia prevention in patients with heart failure is a particular problem. The efficacy of

antiarrhythmic drugs is reduced and there is, moreover, an increased incidence of proarrhythmic side-

effects. In addition, most antiarrhythmic drugs have negative inotropic effects.

CLINICAL SYNDROMES OF HEART FAILURE

Left heart failure

Aetiology

The features of left heart failure develop when there is a major obstruction to outflow from the left

atrium (e.g. mitral stenosis) or when the left ventricle can no longer cope with the demands upon it.

The common causes of left ventricular failure are:

• myocardial infarction

• systemic hypertension

• valvular heart disease

• Cardiomyopathy.

Clinical features: The clinical features of left-sided cardiac failure are largely the consequence of

pulmonary congestion. The symptoms are:

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• dyspnoea on exertion

• orthopnoea and paroxysmal nocturnal dyspnoea

• acute pulmonary oedema.

The physical signs of left ventricular failure may include:

• pulmonary crepitations

• third heart sound

• pleural effusion

• pulsus alternans — alternate large and low volume indication of severe left ventricular failure.

Investigations

• The chest radiograph may show features of pulmonary venous congestion, particularly of the upper

lobe, interstitial oedema and alveolar oedema.

• The electrocardiogram (ECG) may be of value although it does not provide direct evidence of left

heart failure. For example, it is unusual for hypertension or aortic valve disease to lead to the symptoms

of left heart failure without producing ECG evidence of left ventricular hypertrophy first. Again, it is

unusual for coronary artery disease to lead to left heart failure if the ECG is normal. This is not,

however, true of mitral regurgitation.

• Echocardiography: To assess left ventricular end-diastolic and end-systolic dimensions; systolic

function (Ejection Fraction). Echocardiography is also important in the exclusion of other potentially

treatable causes of heart failure such as aortic stenosis or mitral regurgitation.

Atrial natriuretic peptide (ANP) levels and the closely related brain natriuretic peptide (BNP) are

elevated in patients with heart failure.

Differential diagnosis

The dyspnoea of left heart failure is more likely to be provoked by lying down flat. Patients with

dyspnoea due to pulmonary disease usually have a history of asthmatic attacks or of chronic cough and

sputum.

Paroxysmal nocturnal dyspnoea and acute pulmonary oedema may be difficult to differentiate from

acute respiratory attacks. The latter are commonly associated with bronchospasm and purulent sputum.

In contrast, the patient with acute pulmonary oedema is usually free of pulmonary infection, has fine

crepitations rather than rhonchi and is liable to cough up pink frothy sputum. Furthermore, examination

usually reveals the signs of left-sided heart disease. Correct diagnosis is of great importance because

the therapy of the two conditions is different. For example, morphine may be lethal in respiratory

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failure, but invaluable in acute pulmonary oedema. The chest radiograph is also helpful in showing

signs of oedema or infection. In cases of doubt, estimation of the arterial CO2 tension is of value

because this is usually low in acute pulmonary oedema.

Right heart failure

Aetiology

- left ventricular failure with its consequent effects upon the pulmonary circulation.

- right ventricular infarction .

- pulmonary disease, particularly chronic bronchitis and emphysema pulmonary hypertension .

- pulmonary valve disease

- tricuspid regurgitation.

- Right sided venous congestion can result from tricuspid stenosis.

Clinical features: The characteristic features of right heart failure are:

• Elevated jugular venous pressure. In the normal individual, the venous pressure in the internal

jugular veins does not exceed 2 cm vertically above the sternal angle when the patient is reclining at

45° In right heart failure this figure is exceeded. Even if normal at rest, it rises on exercise.

• Hepatomegaly. If chronic, this may result in cirrhosis.

• Oedema. This is of the dependent type and usually most evident in the pretibial and ankle regions.

• Ascites. This may occasionally occur in patients with severe right heart failure.

• Tricuspid regurgitation. This can occur in patients with severe or long-standing right heart failure,

when right ventricular dilatation results in a functional incompetence of the tricuspid valve. A

prominent V wave may be seen in venous pulse and a pulsatile liver edge may be palpable.

Differential diagnosis: In patients presenting with isolated signs of right heart failure, the possibility of

pericardial constriction or tamponade should be considered as an alternative diagnosis.

Other clinical features of cardiac failure

There are a number of common but less specific features of cardiac failure:

• Fatigue is a frequent symptom which is difficult to evaluate.

• The nutrition of patients with cardiac failure is often good in the early stages, but cachexia sets in as

disability increases.

• In the very advanced case, cerebral symptoms may develop with dulling of consciousness,

confusion or changes in personality.

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• Patients with cardiac failure are prone to develop venous thrombosis and pulmonary emboli are

common.

• Mild jaundice, due to hepatic congestion or cirrhosis, is quite frequent in right-sided heart failure.

• Proteinuria due to renal congestion is often present.

General management of cardiac failure

The principles of treating cardiac failure may be enumerated as follows:

• the correction or amelioration of the underlying disease

• the control of precipitating factors

• the reduction of demands on the heart by weight loss and the restriction of physical activity

• pharmacological therapy to modify the heart failure state and, particularly, to reverse the adverse

consequences of neuroendocrine and renal responses to heart failure.

The objectives of therapy are twofold: to alleviate the symptoms caused by heart failure and to improve

the prognosis.

The correction or amelioration of the underlying cause

When heart disease is due to such causes as thyrotoxicosis or hypertension, corrective treatment can be

started immediately. In congenital and rheumatic heart disease, surgical management is usually

required.

In the case of ischaemic heart disease, the cause of heart failure is generally previous myocardial

infarction rather than ongoing ischemia. Coronary revascularization procedures may be considered.

The control of complicating factors (Question: what are the causes of resistant HF or refractory

HF or acute on top of chronic HF):

Cardiac failure is often precipitated or exacerbated by factors superimposed on the underlying heart

disease. Amongst these are: - New myocardial infarction

• Arrhythmias especially atrial fibrillation. Infections, infective endocarditis.

• Uncontrolled hypertension. Pulmonary embolism. Anemia

• Excessive sodium intake. Stopped treatment or insufficient treatment.

• Over-exertion. Pregnancy. Multivalvular or Mutlivessel coronary disease.

• The recognition of precipitating factors is of great importance in management of heart failure,

because the correction of these conditions will often result in the improvement of symptoms.

Exercise

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Rest reduces the demands on the heart and leads to a fall in venous pressure and a reduction in

pulmonary congestion. It allows a relative increase in renal blood flow and often leads to a diuresis.

However, bed rest also encourages the development of venous thrombosis and pulmonary embolism.

The degree of physical restriction necessary depends upon the severity of the cardiac failure. When

there is severe pulmonary congestion or peripheral oedema, a period of complete rest may be required.

Complete bed rest is seldom necessary for more than a few days, after which a gradual increase in

activity should be encouraged, depending upon the response.

In patients with lesser degrees of heart failure, regular exercise should be encouraged. Typical exercise

would be to recommend 20 to 30 mm walking three times per week.

Management of salt and water retention

Low salt diets effectively counteract cardiac failure. However, with the availability of potent diuretic

drugs, no extreme limitation of sodium intake is usually necessary.

PHARMACOLOGICAL THERAPY

Diuretics: The loop diuretics: furosemide (frusemide), bumetanide

These drugs prevent reabsorption at multiple sites including the proximal and distal tubules and the

ascending limb of the loop of Henle.

Thiazide diuretics

The main mechanism is the inhibition sodium reabsorption in the distal convoluted tubule. These drugs

sometimes cause hyperglycaemia and hyperuricaemia, and may precipitate diabetes and clinical gout.

Potassium-sparing diuretics:

This group comprises two classes of agent:

• Spironolactone. This drug is an aldosterone antagonist, providing a weak diuresis with a potassium-

sparing action.

• Amiloride and triamterene. These drugs inhibit sodium—potassium exchange in the distal tubule.

They have a weak diuretic effect.

Recently, spironolactone has been shown to confer an additional mortality benefit of approximately

30% in patients with severe heart failure.

Hyperkalaemia is a potential complication, particularly in patients with impaired renal function.

Particular caution is necessary when adding potassium retaining diuretics to ACE inhibitor therapy.

Spironolactone causes breast enlargement or pain in approximately 10% of men taking the drug.

Inhibitors of the renin-angiotensin system

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Two types of pharmacological agent can be used to block the renin—angiotensin system:

• ACE inhibitors, which inhibit the conversion of angiotensin I to angiotensin II.

• Angiotensin II receptor blocking agents provide an alternative approach to inhibition of the rennin-

angiotensin system. They block the vasoconstrictor and other actions of angiotensin II.

ACE inhibitors

ACE inhibitors are indicated both for the treatment of symptoms and to improve prognosis in patients

with heart failure. In view of these benefits, ACE inhibitors should be prescribed, unless

contraindicated, in patients with symptomatic heart failure and in all patients, irrespective of symptoms,

with an ejection fraction of less than 40%.

Following myocardial infarction, ACE inhibitors are of particular value. They are indicated not only for

the treatment of failure, but also for the prevention of adverse remodeling.

ACE inhibitors are in general well tolerated. However, a number of problems may be encountered,

including hypotension, renal impairment and cough:

• First-dose hypotension can be minimized by reducing the dose of the ACE inhibitor on

commencing therapy and omitting diuretics for 1—2 days beforehand.

• ACE inhibitors occasionally cause deterioration of renal function. They are contraindicated in

patients with an initial creatinine level greater than 200 mmol/L (or creatinine > 2.6 mg/dl). Renal

function should be checked routinely 1—2 Weeks after commencing therapy.

• Cough is a potentially troublesome side-effect, occurring in up to 10% of patients.

Angiotensin II receptor blockers

In patients intolerant of ACE inhibitors there is evidence that angiotensin receptor blocking drugs do

improve prognosis.

Other vasodilators

The widespread applicability and indications for ACE inhibitors have reduced the importance of other

vasodilators in the management of heart failure.

Nitrate vasodilators remain of value in the management of acute left ventricular failure. Sublingual

glyceryl trinitrate can be administered in the acute phase and can be followed by an intravenous

infusion. Nitrates act predominantly as venodilators.

Hydralazine, by contrast, is predominantly an arterial dilator.

Beta-blockers

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Excessive sympathetic stimulation may contribute to progression of heart failure in a number of ways,

including additional energy requirements, ventricular hypertrophy and arrhythmias. Beta-blockers

result in a reduction in mortality, of the order of 30%. The reduction in mortality relates substantially to

a reduction in sudden deaths, but beta-blockers also benefit symptoms and have been shown to reduce

hospitalizations for heart failure. Beta-blockers have been shown to benefit patients with class II and III

heart failure and to benefit selected patients with class IV heart failure. In general, the more severe the

degree of heart failure and the worse the prognosis of the patient, the greater the benefit to be gained

from beta-blockade.

• Patient selection is crucial. Beta-blockers should not be given in new onset or uncontrolled heart

failure. Patients presenting with acute heart failure or with an exacerbation of chronic heart failure

should be stabilized with diuretics and ACE inhibitors before initiating a beta-blocker. Bradycardia

(heart rate < 60) and hypotension (systolic blood pressure < 100) are relative contraindications and

require particularly careful monitoring on commencement of therapy.

• Low dose initiation of therapy is crucial.

• Slow upward dose titration with clinical monitoring. Titration should occur at intervals of not less

than 2 weeks. Dizziness, postural hypotension and worsening heart failure are all relatively common

and may require dose reduction or cessation of beta-blocker therapy.

Inotropic agents

Digitalis glycosides

Mechanisms of action

The inotropic action of digitalis is mediated through the sodium/potassium ATPase (sodium) pump, to

which it binds. The inhibition of this pump leads to an accumulation of intra-cellular sodium; because

of the sodium— calcium exchange system, this results in an increase in the amount of calcium

available to activate contraction. Digitalis also has sympathomimetic and parasympathetic (vagal)

effects. The latter is clinically important, in that it causes slowing of the sinus rate.

Indications: Digoxin is particularly indicated in patients with HF and atrial fibrillation, for its

beneficial effects to reduce ventricular response rate. In this setting it is an appropriate first line agent.

Other drugs: Ivapradine (procoralan) that reduces sinus node rate without diminishing myocardial

contractility. Trimetazidine (Vastarel) and Cardioton (Crataegus monogyna ).

VENTRICULAR RESYNCHRONIZATION THERAPY

There is growing evidence that some individuals with severe heart failure may be improved by

biventricular pacing to provide ventricular resynchronization.

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In many patients with severe heart failure, left ventricular contraction becomes incoordinate. Delay in

the spread of the electrical impulse to different regions of the ventricle results in a dispersion of the

onset of contraction. As a consequence, the regions of the ventricle activated earliest may be relaxing

by the time later regions have started to contract. This results in an additional inefficiency of pump

function, responsible for an additional deterioration in ejection fraction and cardiac output.

Ventricular resynchronization pacing aims at pacing the septum and lateral wall of the left ventricle

simultaneously, thereby improving the synchrony of contraction. One electrode is placed in the right

ventricle, as for conventional pacing, and the other at the left free wall. Left ventricular pacing is

achieved via the coronary sinus. Simultaneous pacing at the two sites results in a narrowing of QRS

width and an improvement in cardiac output.

Criteria of selection of patients likely to benefit most from ventricular resynchronization pacing

include:

• Severe heart failure, New York Heart Association class III or IV

• Left bundle branch block

• QRS width greater than 120 ms

• Evidence of incoordinate left ventricular contraction on echocardiography.

ARRHYTHMIA MANAGEMENT

About 50% of patients with heart failure die from progressive heart failure. The other 50% die

suddenly as a result of ventricular arrhythmias.

Beta-blockade has been shown to dramatically reduce sudden deaths.

There is growing evidence to suggest a role for implantable defibrillators in this patient population.

Implantable defibrillators have been shown to significantly reduce mortality in patients with an

ejection fraction less than 30%.

As implantable defibrillators can be combined with ventricular resynchronization, there is likely to be a

growing role for device therapy in the management of patients with severe heart failure.

ACUTE LEFT VENTRICULAR FAILURE

Acute pulmonary oedema is a life-threatening emergency. Characteristically, the patient is extremely

breathless and frightened. The patient is unable to lie flat and prefers to sit bolt upright. In severe cases

they may cough up blood tinged, pink sputum.

Causes of acute left ventricular failure:

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- Acute myocardial infarction.

- Atrial fibrillation and other tachyarrhythmias.

- Severe hypertension.

- Myocarditis

- Infective endocarditis with acute valve damage (incompetence).

- Chordal rupture.

- Cardiac tamponade

- Acute exacerbation of chronic heart failure (due to increased sodium intake, non-compliance with

medications (eg stopping digitalis), exacerbation of hypertension, acute arrhythmias, infection and/or

fever, pulmonary embolism, anemia and hemorrhage, thyrotoxicosis, pregnancy and child birth,

infective endocarditis with valve damage, rheumatic fever, physical emotion and stress, and prolonged

tachycardia or bradycardia).

Causes of non-cardiac pulmonary edema:

- Adult respiratory distress syndrome.

- Pulmonary embolism.

- Toxic gases.

- Gram negative septicemia (shock-lung).

- Diffuse pulmonary infections.

- Aspiration.

- Narcotic overdose especially parentral heroin.

- Lymphatic obstruction.

- Following cardio-pulmonary bypass.

- Hemorrhagic pancreatitis.

Clinical features

• The patient is tachypnoeic and distressed.

• Perspiring profusely.

• Systolic pressure is frequently elevated.

• A marked tachycardia is evident with a gallop rhythm on auscultation.

• Crackles and wheeze are heard throughout the chest.

Table: Differentiating Points Between Bronchial Asthma and Cardiac Asthma

Bronchial Asthma Cardiac Asthma

History of allergy usually present usually absent

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Age usually young usually older

Cardiac lesion absent present (causing left heart failure)

Cough associated with viscid

sputum

associated with frothy pinkish

sputum

Chest examination mainly wheezes wheezes and crepitations

Eosinophilia usually present absent

Circulation time normal or short prolonged

Investigations

• Chest radiograph shows diffuse haziness due to alveolar fluid. Changes generally bilateral (Bat-

wing appearance) but occasionally may be unilateral.

• Blood gases. Arterial p02 falls. Initially pCO2 also falls due to breathing, but in the later stages

pCO2 may rise due to impaired exchange.

Management: Management of acute LVF:

• General. A venous line should be inserted and the patient should be monitored.

• Oxygen. This should be administered in high concentrations (60%) unless the patient has

concomitant airways disease.

• Diamorphine. The standard dose of diamorphine is 5 mg given intravenously.

• Diuretics. The patient should be given intravenous furosemide (frusemide). The usual dose would

be 40 mg, but this may be increased in patients already on diuretic therapy.

• Nitrates. Administration of a sublingual tablet of glyceryl trinitrate has an immediate effect of

lowering pulmonary pressures and reducing pulmonary oedema. This may be followed, if necessary, by

an infusion of the drug.

• Inotropic therapy. In cases of refractory pulmonary oedema, inotropic therapy should be

considered. Aminophylline 250 mg i.v. over 10 mm is frequently effective. Alternatively, patients may

be started on a dobutamine infusion, beginning at 5 µg/kg/min.

CARDIOGENIC SHOCK

The terms acute circulatory failure, low output state, and shock are used to describe a syndrome

comprising arterial hypotension, cold, moist and cyanosed extremities, a rapid weak pulse, a low urine

output and a diminished level of consciousness. This pattern can arise as a result of impaired cardiac

function, in which case, it is termed cardiogenic shock.

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This clinical pattern is common to a number of other disorders and cardiogenic shock must be

differentiated from other causes of shock, including:

• hypovolaemic shock, eg by haemorrhage and loss of fluid from burns, vomiting and diarrhoea

• septicaemic shock

• anaphylactic shock

• acute pancreatitis.

Shock is described as cardiogenic when it is clearly cardiac in origin. This may be due to many

different causes, including myocardial infarction, massive pulmonary embolism, dissecting aneurysm,

pericardial tamponade, rupture of a valve cusp, and arrhythmias; also pulmonary embolism. In

cardiogenic shock, the central venous pressure is usually raised, in contrast to hypovolaemic shock, in

which it is characteristically low.

Clinical features

• In the first stage of shock, there is a fall in cardiac output and blood pressure, due to either a

diminution in venous return or to an inability of the myocardium to expel an adequate stroke volume.

• As a consequence of the hypotension, there is a fall in renal blood flow, with oliguria.

• Reflex tachycardia occurs.

• Compensatory reflex arteriolar vasoconstriction further reduces blood flow to the kidneys,

abdominal viscera, muscle and skin. Vasodilatation of the cerebral and coronary vessels permits the

maintenance of a relatively good blood flow in these territories. If the vasoconstriction is sufficiently

great, the blood pressure may be kept at or close to normal levels but at the expense of producing tissue

hypoxia with consequent acidosis.

Management of cardiogenic shock

General management

If the patient is in severe pain or distress, opiates should be given intravenously (provided there is no

contraindication) and high-flow oxygen administered, preferably by a tight-fitting face mask making

use of the Venturi principle, or by mechanical ventilation. Unless there is pulmonary oedema, the

patient should be laid flat, with the legs slightly raised. A catheter should be introduced to measure

urinary output. Arterial blood gases and pH should be monitored. A Swan-Ganz balloon- tip catheter

should be used to obtain pulmonary artery and ‘pulmonary capillary wedge’ pressures if a cardiac or

pulmonary cause is known or suspected (recently this invasive monitoring was considered not

mandatory in some cases). As measurement of blood pressure by a sphygmomanometer is unreliable in

severe shock, direct arterial pressure monitoring should be undertaken, when possible.

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Correction of hypovolaemia

Although left ventricular filling pressures are most commonly elevated in patients with cardiogenic

shock, this is not always the case. Patients may have undergone a period of prior diuretic therapy

resulting in fluid depletion. Alternatively, in cases of right ventricular infarction the left ventricle may

be under filled. A Swan—Ganz catheter enables pulmonary artery wedge pressure to be estimated to

achieve an optimal pressure of between 18 and 20 mmHg. If the pressure is below this level, saline

should be administered to increase the wedge pressure and optimize cardiac output

Inotropic agents

These drugs enhance myocardial contractility but at the expense of increased oxygen consumption.

Dopamine and dobutamine are most frequently used.

The effects of dopamine, a natural precursor of noradrenaline (norepinephrine), depend upon the dose.

Administered intravenously in a dosage of 2—5 µg/kg/min, it causes dilatation of renal and mesenteric

vessels; at doses of 5—10 µg/kg/min, it increases myocardial contractility and cardiac output. At

higher doses, it causes vasoconstriction (it should not be infused directly into a peripheral vein as

leakage may cause local necrosis). Dopamine may induce nausea and vomiting, and can lead to an

excessive tachycardia and arrhythmias.

Dobutamine is a synthetic sympathomimetic agent whose predominant action is one of stimulating

activity. It is less likely to cause vasoconstriction or tachycardia than dopamine. It is given by

intravenous infusion at a rate of 2.5—10 µg/kg/min.

Mechanical support

The intra-aortic balloon pump is of value in acute myocardial infarction if shock has been caused by a

surgically correctable lesion, such as a ventricular septal defect or papillary muscle rupture.

REFRACTORY HEART FAILURE

Heart failure is termed refractory when it persists or deteriorates despite intensive therapy. Causes:

- Hyperthyroidism

- Anemia

- Recurrent pulmonary emboli

- Atrial fibrillation and other arrhythmias

- Multiple myocardial infarcts, multivessel coronary disease

- Hypertension uncontrolled

- Pneumonia, other infections, chronic obstructive pulmonary disease with exacerbations.

- Infective endocarditis

- Rheumatic activity

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- Pregnancy

- Constrictive pericarditis and endomyocardial fibrosis

- Left ventricular aneurysm

- The myocardium reached end stage with fibrosis, scars, and multiple infarcts.

Diagnosis and Treatment: according to cause.

ACUTE EXACERBATION ON TOP OF CHRONIC HEART FAILURE

Cause, diagnosis and treatment: Review above page --.

INFECTIVE ENDOCARDITIS

Infective endocarditic can occur in two ways:

3. When the heart valves and endocardium are damaged, organisms of low pathogenicity (e.g.

streptococcus viridans) can invade them and produce a slowly progressive infection, i.e. subacute

bacterial (or infective) endocarditis.

4. Normal valves and endocardium can be invaded by organisms of high pathogenicity (e.g.

staphylococcus aureus, pneumococcus, gonococcus) in the course of a fulminating septicemia

originating in another organ. In these cases the course is usually acute, i.e. acute bacterial (or infective)

endocarditis.

SITE OF INVOLVEMENT:

- Subacute infective endocarditis occur on top of a preexisting heart disease, e.g. chronic rheumatic

heart disease, congenital heart disease, etc. or on artificial (prosthetic) valve.

- It most commonly complicates mitral regurgitation, aortic stenosis, aortic regurgitation, calcific or

sclerotic aortic valve, ventricular septal defect, patent ductus arteriosus, bicuspid aortic valve or

artificial valves.

- It is less common in cases of Fallot’s tetralogy and pure mitral stenosis. It is very rare in cases of

atrial septal defect. Endocarditis of the tricuspid valve occurs in intravenous drug abusers who inject

drugs under septic conditions.

- It is more common in the left side of the heart than the right side.

ORGANISM:

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The most common causative organism is Streptococcus viridans. Less common are Staphylococcus

aureus and enterococcus. Fungal endocarditis is caused by candida or aspergillus and it is common in

patients receiving large doses of antibiotic, steroids or cytotoxic drugs. It is also common in drug

abusers and in infections of prosthetic materials placed in the circulation e.g. indwelling catheters,

prosthetic valves, etc.

SOURCE OF INFECTION:

Bacteremia, by dental extraction and other dental procedures, urinary catheterization, labor, abortion,

upper respiratory infection, etc. But often the source of infection is unknown.

CLINICAL PICTURE:

4. Onset is usually insidious with fever, sweating, arthralgia, malaise, toxic anemic look.

5. Persistent fever is usually of low grade but varies, with pallor and earthy “café au lait” facies.

6. The heart may show the following:

a. There is always evidence of pre-existing heart disease.

b. Change or increase of existing murmurs or the development of new murmurs due to destruction of

heart valves by the infection.

c. In advanced cases heart failure results from toxic myocarditis and the effects of valvular defects.

7. The spleen is moderately enlarged and tender in most cases.

8. Clubbing of the fingers occurs after 5-6 weeks.

9. Involvement of the kidney may result in

e. Hematuria, whether microscopic or macroscopic, and proteinuria occurs in most cases.

f. A picture of immune complex acute glomerulonephritis.

g. Renal failure may complicate advanced cases.

h. Renal infarction may cause pain in the loins and hematuria.

10. Embolism may involve any organ, e.g. spleen, kidney, limbs, brain, retina, mesenteries. It produces

variable signs and symptoms of infarction depending on the site and size of the embolus and may cause

mycotic aneurysm. Pulmonary embolism may complicate endocarditis involving the right cardiac

chambers. Retinal emboli or immune complexes cause areas of hemorrhage with pale center (Roth

spots).

11. Neurologic manifestations include:

d. Infective endocarditis sometimes presents as cerebral embolism. Septic infarction may result in

cerebral abscess.

e. Cerebral or subarachnoid hemorrhage may result from rupture of a mycotic aneurysm.

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f. A picture stimulating meningitis or encephalitis may occur.

12. Skin lesions include:

d. The commonest are petechial hemorrhages. They appear as crops of small brown red spots that do

not disappear on pressure. They are most commonly found in the chest, neck, palate and conjunctiva.

e. Osler nodules are tender intracutaneous nodules usually found in the pulps of finger and in the

thenar and hypothenar eminence.

f. More rarely streaks of hemorrhage under the nails (splinter hemorrhage), or flat erythematous

macules in the palms and soles (Janeway lesions) may be seen.

DIAGNOSIS:

7. Bacterial endocarditis must always be suspected in any patient with a murmur or a known heart

disease who develops an unexplained or prolonged fever.

8. When bacterial endocarditis is suspected the diagnosis is confirmed by blood culture. Culture

must be done for aerobic and anaerobic bacteria and fungi and must be incubated for up to 3 weeks to

allow slow growing organisms to emerge. When an organism is isolated its antibiotic sensitivity must

be tested. Cultures may also be negative in fungal endocarditis and in infections by fastidious or slowly

growing organisms, but most commonly in those who recently received antibiotic therapy.

9. Echocardiography is essential and may show vegetations on the valves, an abscess on the valve

ring, and the underlying heart disease. Transesophageal echocardiography is more sensitive than the

transthoracic technique.

10. Urine examination commonly shows microscopic or macroscopic

11. Hematuria. Red cell casts and heavy proteinuria indicate the presence of immune complex

glomerulonephritis.

12. Blood examination shows elevated ESR, anemia and sometimes leukocytosis.

Duke criteria for diagnosis of IE:

I- Definite IE:

A- Pathological criteria:

3- Microorganisms--------culture or histology or

4- Pathological lesions----vegetations, abscess confirmed by histology

b- Clinical criteria: (2 major, or 1 major +3 minor, or 5 minor)

*Major criteria:

1- Positive blood culture for IE.

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2- Evidence of endocardial involvement:

Positive echo---vegetations, abscess, new dehiscence of prosthetic valve, or new or worsened valvular

regurgitation.

* Minor criteria:

1- Predisposition: heart disease, or IV drug use.

2- Fever: >38 c

3- Vascular phenomena:

4- Immunologic phenomena:

5- Microbiological evidence:

6- Echo findings:

II- Possible IE:

Findings consistent with IE that fall short of (definite) but not (rejected)

III- Rejected: Firm alternative diagnosis for manifestations of endocarditis or

Resolution of manifestations of endocarditis, antibiotic for 4 days or less

COMPLICATIONS:

The most important complications are:

5. Arterial emboli may cause hemiplegia, aphasia, infarction of the bowel, kidney, lung, or ischemia

or gangrene of arm or leg.

6. Destruction or perforation of cardiac valves. Large vegetations may interfere with prosthetic valve

function.

7. Congestive cardiac failure.

8. Uremia.

DIFFERENTIAL DIAGNOSIS:

The most important differential diagnosis is:

c. Rheumatic activity

d. Intercurrent infections and fevers.

3. Sometimes, it may be very difficult to differentiate infective endocarditis from rheumatic activity.

The presence of enlarged spleen, clubbing of the fingers, petechiae, embolic manifestations and

hematuria points towards infective endocarditis. On the other hand, the appearance of fleeting arthritis,

erythema marginatum, subcutaneous nodules and evidence of previous streptococcal throat infection

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favours the diagnosis of rheumatic activity. When in doubt, multiple blood cultures should be done and

the patient should be treated as infective endocarditis.

4. Infective endocarditis must be differentiated from other causes of fever in patients with previous

heart disease, e.g. specific infections as brucella, typhoid, tuberculosis etc., connective tissue diseases,

lymphomas, etc. These diseases are diagnosed by their specific signs and tests.

PROPHYLAXIS:

3. Amoxicillin (2 gm) prophylaxis should be given orally to all patients with rheumatic or

congenital heart disease, one hour before all procedures that may result in bacteremia. These include

dental extraction, tonsillectomy, urethral catheterization, prostatic massage, delivery, abortion, etc. In

cases of penicillin sensitivity clindamycin, erythromycin or vancomycin can be used.

4. In patients at very high risk of developing endocarditis (e.g. those with prosthetic valves or

history of previous endocarditis), more intensive prophylaxis is required. Ampicillin 2 gm IV or IM

should be given 30 minutes before the procedure together with gentamycin 1.5 mg/kg. This should be

repeated 6 hours later.

TREATMENT:

- Streptococcus viridans is usually penicillin sensitive and is eradicated by giving 3 million units of

penicillin l.V. every 4 hours (18 million per day) for 3-4 weeks together with gentamycin 1 mg/kg

every 8 hours I.M. for the first two weeks of treatment. For penicillin resistant streptococci and for

penicillin allergic patient vancomycin is given 15 mg/kg/12 hours + gentamycin.

4. Enterococcus is less sensitive and needs 20-40 million units of penicillin G I.V. plus gentamycin

3 mg/kg I.M. daily. Ampicillin in a dosage of 2 gm every 4 hours I.M. may be substituted for

penicillin. The effective dose should be maintained for at least 6-8 weeks.

5. Staphylococcus: most strains secrete penicillinase and these should be treated by penicillinase-

resistant penicillins (nafcillin or oxacillin) or cefazolin or vancomycin 15 mg/kg/12 hours for 6 weeks.

An aminoglycoside antibiotic and rifampicin 300 mg twice daily may be added.

6. Culture-negative endocarditis patients should be treated as enterococcal endocarditis.

Surgery will also be needed if:

a. Infection cannot be controlled.

b. An abscess forms around the valve ring.

c. Very big vegetations that may cause major emboli.

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d. Congestive heart failure develops due to destruction or perforation of a valve.

Duke Criteria for Infective Endocarditis

Major criteria :

Positive blood culture for Infective Endocarditis

Typical microorganism consistent with IE from 2 separate blood cultures, as noted below:

• viridans streptococci, Streptococcus bovis, or HACEK group, or

• community-acquired Staphylococcus aureus or enterococci, in the absence of a primary focus

or Microorganisms consistent with IE from persistently positive blood cultures defined as:

• 2 positive cultures of blood samples drawn >12 hours apart, or

• all of 3 or a majority of 4 separate cultures of blood (with first and last

sample drawn 1 hour apart)

Evidence of endocardial involvement Positive echocardiogram for IE defined as :

• oscillating intracardiac mass on valve or supporting structures, in the path of regurgitant jets, or on implanted material in the absence of an alternative anatomic explanation, or

• abscess, or

• new partial dehiscence of prosthetic valve or

New valvular regurgitation (worsening or changing of preexisting murmur not sufficient) Minor criteria :

Predisposition: predisposing heart condition or intravenous drug use

Fever: temperature > 38.0° C (100.4° F)

Vascular phenomena: major arterial emboli, septic pulmonary infarcts, mycotic aneurysm, intracranial hemorrhage, conjunctival hemorrhages, and Janeway lesions

Immunologic phenomena: glomerulonephritis, Osler's nodes, Roth spots, and rheumatoid factor

Microbiological evidence: positive blood culture but does not meet a major criterion as noted above¹ or serological evidence of active infection with organism consistent with IE

Echocardiographic findings: consistent with IE but do not meet a major criterion as noted above

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Samir Rafla: Principles of Cardiology pages 87-111

ISCHEMIC HEART DISEASE

Disease of the coronary arteries is due to atherosclerosis in the majority of cases. However, the

coronary arteries may be involved in other disorders as congenital abnormalities, coronary embolism,

dissection, polyarteritis and coronary spasm.

ATHEROSCLEROSIS

Atherosclerosis consists of deposition of fatty substance rich in cholesterol in the subintimal layer of

the arteries. Later, the intima overlaying the fatty deposition may fibrose resulting in fibrous cap. The

covering fibrous cap may disrupt and an intimal ulcer occurs. The lumen of the vessel becomes

progressively narrowed and finally complete obstruction occurs due to:

1. Progress of the narrowing.

2. Thrombosis over disrupted intimal plaque.

3. Hemorrhage in the subintimal region.

PATHOGENESIS OF ATHEROSCLEROSIS:

The response to injury hypothesis: It states that injurious agents e.g. hypertension and

hypercholesterolemia cause endothelial damage. The injured endothelium becomes more permeable

admitting cholesterol (specially the low density lipoprotein fraction) into the intima where it is oxidized

and becomes cytotoxic. Macrophages from the blood and smooth muscle cells from the intima migrate

into the lesion, proliferate, ingest the lipids, become foam cells and later rupture releasing their content

in the interstitium and the cycle is repeated.

Thus elements that play roles in atherosclerosis are: 1. Endothelium. 2. Monocytes/macrophages. 3.

Smooth muscle cells. 4. Platelets. 5. Blood lipids.

The mature plaque is composed of a lipid core that is covered by a fibrous cap. There are two types of

plaques:

a. Stable plaques which have a small lipid core and a thick fibrous cap. It is not liable to rupture and

thus unlikely to induce thrombus formation and acute occlusion of the artery.

Unstable or Vulnerable plaques have a large lipid core and thin fibrous cap. It may be invaded by

macrophages and inflammatory cells especially at its margins. These inflammatory cells secrete

enzymes that digest the fibrous cap. The plaques are liable to rupture at their margins exposing the

plaque core to the circulating blood. Platelets adhere to the exposed area and start a chain of platelet

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aggregation then coagulation cascade leading to an intravascular thrombus. This may reduce the vessel

lumen or occlude it partly or totally.

The diffusion of fats and cholesterol from the blood stream into the intima is accelerated by:

1. Increase in the concentration of cholesterol and other lipids. The normal total serum cholesterol is

150-190 mg%, but in persons with multiple other risk factors, diabetes or clinical coronary artery

disease, this level should not exceed 175 mg%, Low-density lipoprotein cholesterol (LDL) is the major

atherogenic lipid. LDL levels less than 130 mg% are considered desirable in otherwise normal persons.

However, in persons with ischemic heart disease, diabetes or two or more risk factors the level of LDL

should not exceed 100 mg%, reaching 70 mg% is now a desirable target.

High-density lipoprotein fraction of cholesterol (HDL) is, in contrast, protective as is assists in the

reverse transport of LDL from the cells back to the liver to be metabolized. The normal level of HDL in

serum should be at least 40 mg% in males and 50 mg% in females.

Triglycerides are less atherogenic but their level should not exceed 150 mg%.

2. Increase in the arterial blood pressure.

Risk factors leading to atherosclerosis

1. Abdominal obesity, 2. Hypercholesterolemia, 3. Hypertension, 4. Diabetes and 5. Cigarette

smoking are the most important risk factors leading to atherosclerosis. Additional risk factors are:

6. Family history of ischemic heart disease (genetics). 7. Insulin resistance syndrome (metabolic

syndrome).

8. Sedentary non-active life, 9. Mental and psychic stress. 10. Myxedema.

PREVENTION OF ATHEROSCLEROSIS:

Prevention of atherosclerosis is done by avoiding the above factors.

Life style modification: by

1) Diet: The diet should be low ‘in fully saturated fats, cholesterol”

2) Walking 20 min daily 5 days per week.

3) Hypocholesterolemic drugs: The most important are:

a. Statins i.e. HMG-CoA reductase inhibitors e.g. Simvastatin (Zocor, Atorvastatin (Lipitor),

Fluvastatin (Lescol) and Rosuvastatin (Crestor). These are very effective in reducing cholesterol

synthesis only. Side effects include rhabdomyolysis (i.e. skeletal muscle cell necrosis) and impaired

liver function.

b. Fibrate group of drugs e.g. gemfibrozil (Lopid), fenofibrate (Lipanthyl).

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c. Bile acid sequestrants e.g. Colestyramine (Questran) binds bile acids in the gut and prevents their

reabsorption.

d. Other drugs include fish oils, omega 3 fatty acids and ezetimibe (Ezetrol) (which inhibits

cholesterol absorption in the gut). Combinations of ezetimibe and statins are used in severe cases and

are very effective.

4) Treatment of underlying disease causing the increase in blood lipids, e.g. diabetes, myxedema,

nephrosis, etc.

5) Treatment of hypertension.

PRESENTATIONS OF ISCHEMIC HEART DISEASE

STABLE ANGINA PECTORIS

Definition: Angina pectoris is a discomfort in the chest and adjacent areas due to transiently

inadequate blood supply to the heart. It is precipitated by effort and relieved by rest.

ETIOLOGY:

Myocardial ischemia occurs when there is:

A. Decrease in coronary blood flow due to:

1. Coronary atherosclerosis is the commonest cause.

2. Coronary artery spasm (Prinzmetal or variant angina).

3. Low cardiac output.

4. Severe lowering of the diastolic blood pressure, e.g. in aortic regurgitation.

B. Excessive increase in cardiac work due to:

1. Severe hypertension.

2. Aortic stenosis.

3. Rapid tachycardia (tachycardia also shortens diastolic period during which the coronary arteries

fill).

4. Hypertrophic myopathies.

C. Reduction of oxygen carrying capacity of blood due to:

1. Severe anemia.

CLINICAL FEATURES:

The most characteristic feature is ischemic cardiac pain that is brought about by exertion and is relieved

by rest. The pain fibers travel with the sympathetic nerves to the upper 4-5 thoracic spinal segments.

That is why the pain is referred to the peripheral dermatomes supplied by these spinal segments.

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Character of cardiac pain: The pain is dull aching squeezing, compressing or burning of variable

intensity. Instead of pain, angina may be perceived as compression, burning or even dyspnea on effort

(angina equivalents).

Site of pain: Cardiac pain is mostly substernal. It usually radiates to the left arm, sometimes it spreads

to the root of neck, both shoulders and arms, back, epigastrium or the jaw. The pain may be felt only in

the left arm or jaw.

Relief: The pain forces the patient to stop and disappears after 1-3 minutes rest. Sometimes it lasts

longer but not more than 15 minutes. It is also always relieved by sublingual nitroglycerine.

Associated manifestations: The pain may be associated with sweating, tachycardia, anxiety and rise in

blood pressure.

Some patients may have silent ischemia i.e. episodes of ischemia manifested by E.C.G. changes but

without chest pain. This is particularly common in diabetics due to autonomic neuropathy. Silent

ischemia is detected by the ECG during stress testing or during a 24 hour ambulatory E.C.G recording

(Holter monitor).

Precipitating factors include:

1. Effort is the most common precipitating factor, e.g. climbing stairs, running, walking, carrying

heavy weight, etc. the pain occurs during effort.

2. Emotions.

3. Heavy meals.

4. Exposure to cold.

5. Sexual intercourse.

6. In severe cases pain may be precipitated by lying down i.e. angina of decubitus.

Physical examination is usually free in between the attacks. During the attack a fourth heart sound may

appear and a pansystolic murmur may be heard because ischemia of a papillary muscle may lead to

ischemic mitral regurgitation.

DIAGNOSIS AND INVESTIGATIONS:

1. History.

2. Exercise electrocardiography.

3. Radionuclide Perfusion Scintigraphy (Thallium study).

4. Multislice CT.

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5. Coronary arteriography. This procedure is essential if the diagnosis is in doubt. It is also needed to

help determine whether medical treatment, balloon angioplasty and stenting or surgery is most

appropriate.

Indications of coronary angio: in high risk patients such as:

a. Patients not responding to medical treatment.

b. Patients who develop chest pain or ECG changes on slight effort (highly positive exercise ECG).

c. Patients in whom the anginal pain is associated with dyspnea or hypotension.

d. Patients who develop angina after an attack of myocardial infarction (post-Infarction angina).

e. Those who develop the disease at young age e.g. below 40 years.

f. Any patient over 40 years of age undergoing open-heart surgery for other purposes e.g. valve

replacement.

g. Patients resuscitated from sudden cardiac arrest.

DIFFERENTIAL DIAGNOSIS:

The following conditions cause pain that may simulate angina:

1. Anxiety and neurosis are perhaps the commonest and the most difficult differential diagnosis.

2. Esophageal spasm, reflux esophagitis and hiatus hernia.

Here pain is related to meals but has no constant relationship to exertion.

3. Musculo-skeletal lesions, e.g. cervical or thoracic spondylosis, cervical rib, myositis,

costochondritis (Tietz’e syndrome).

4. Cholecystitis.

5. Pleurisy and Pneumonia.

6. Pericarditis.

PROGNOSIS: The prognosis is variable depending on the status of left ventricular function, the

number of the vessels involved as seen in coronary arteriography and on the presence of associated

disease such as hypertension, heart failure, diabetes, myocardial infarction, arrhythmias, renal failure,

etc

TREATMENT:

A. During the Attack:

The most important drug is nitrates. They are used either as sublingual tablets e.g. isosorbide dinitrate

(Isodril or Dinitra 5 mg) or nitroglycerine (Angised 0.3 mg), or as oral spray. They are coronary

vasodilators but more importantly, they cause generalized decrease in arteriolar and venous tone. This

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reduces both the blood pressure and the venous return and thus lowers the oxygen demands of the

heart.

B. In Between Attack:

The long-term management of angina consists of the following lines.

1. Improvement of Coronary Blood Supply: This is done by:

a. Nitrates:

b. Calcium channel blocker: improve angina by two mechanisms.

i. They decrease or abolish spasm of coronary arteries.

ii. They decrease myocardial contractility and thus decrease the myocardial oxygen demand. Either

nifedipine or verapamil (Isoptin) or diltiazem can be used.

c. Revascularization: This can be achieved either by surgery or by percutaneous trans-catheter

angioplasty (PCI = Percutaneous Coronary Intervention). An operation has been devised to bypass the

atherosclerotic obstruction in the coronary artery by a bypass graft (CABG) made of the saphenous

vein, radial or internal mammary arteries. Dilatation of localized lesions can also be accomplished by a

balloon catheter i.e. coronary angioplasty with deployment of a stent to keep the dilated segment

widely open. Revascularization is indicated in all cases of severe angina not responding fully to

medical treatment and in all cases of post infarction angina.

Surgical bypass grafting should be considered in:

i. Patients with stenosis of the left main coronary artery exceeding 50% with or without symptoms.

ii. Patients with significant obstruction (over 70%) in the three major coronary arteries especially in

diabetics and those with LV dysfunction.

On the other hand, percutaneous coronary intervention (PCI) with stenting should be considered in

patients with localised lesions in one or two coronary arteries.

PCI is successful in over 90% of cases. However, restenosis at the site of dilatation occurs within 6

months in about 15 – 20 %. New stents that elute a drug (the metal is covered with a drug) that

decrease restenosis rate to 5- 10 % have been introduced (drug eluting Cypher and Taxus stents).

Lesions that were previously considered unsuitable for PCI can often now be stented. These include:

- Long coronary lesions (> 20 mm).

- Total coronary occlusion.

- Bifurcation lesions.

- Vein graft stenoses.

- Lesions of the left main stem.

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2. Reduction of Myocardial Oxygen Needs:

- Treatment of secondary factors that increase oxygen needs of the heart, e.g. hypertension, heart

failure, tachycardias, thyrotoxicosis, etc.

- Reduction of weight in obese patients.

- Exercise training. This is called rehabilitation. Exercise may also help develop collaterals.

- Avoidance of extreme emotions and treatment of anxiety by tranquilizers.

- Beta-adrenergic blockers: They cause a decrease in myocardial oxygen consumption by the following

mechanisms: Decrease the heart rate. Decrease the myocardial contractility. Decrease the blood

pressure.

In patients with bronchospasm, diabetes, or peripheral vascular disease a cardioselective beta-blocker

or beta and alpha blocker is preferred.

- Stopping smoking. Nicotine predisposes to angina by: - Increases catecholamines. - Increases serum

lipids. - Increases the heart rate and may precipitate arrhythmias. - Carbon monoxide in the smoke

binds some hemoglobin as carboxyhemoglobin.

- Avoiding situations leading to angina, e.g. walking after meals or in cold weather.

3. Prevention of Further Progression of Atherosclerosis. This is best done by avoiding factors

predisposing to it i.e. hypertension, obesity, cigarette smoking and physical inactivity and by treating

dyslipidemias (Statins for life).

4. Antiplatelet drugs e.g. aspirin 75-150 mg daily is used to prevent platelet aggregation and

subsequent thrombosis in the narrowed vessel.

SPASMODIC ANGINA: Angina can develop due to spasm of the coronary arteries. Spasm may occur

in normal arteries but more commonly it occurs on top of coronary atherosclerosis. As the spasm is

spontaneous, pain occurs in attacks not related to any precipitating cause and is relieved spontaneously.

It occurs more commonly in the early morning after awakening the patient from sleep. It may be

associated with arrhythmias. The attacks are associated with ST rise in the ECG instead of ST

depression that occurs in classic angina. This is Variant or Prinzmetal angina.

Attacks of coronary spasm respond to nitrates. However the best long-term prophylactic treatment is

calcium channel blockers (amlodipine, diltiazem or verapamil).

Myocardial ischemia and angina may occur also in patients with normal coronary arteries and without

evidence of spasm. This condition probably results of disease of the microcirculation. It has been

termed micro-vascular angina or syndrome X.

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Unstable Angina and Non-S-T Elevation Myocardial Infarction (NSTEMI)

This term unstable angina is given to any of the following:

a. When the severity, duration, or frequency of anginal attacks increase

b. Moderate to severe angina of recent (< 1 month) onset.

c. When angina appears on rest, or lying down.

d. Post-infarction angina.

The ECG may be normal or may show S-T segment depression or T wave inversion beside any

previous findings.

Unstable angina and NSTEMI may start de nova or may develop on top of chronic stable angina.

They develop when a break occurs in the fibrous cap covering an atherosclerotic plaque (plaque

disruption). This leads to exposure of the lipids and other contents of the interior of the plaque to

bloodstream. Platelet aggregation occurs on the raw surface and leads to the development of a non-

occlusive thrombus that markedly diminishes (but does not totally obstructs) the lumen of the artery.

Vasoactive substances released from the damaged endothelium and aggregated platelets leads to local

coronary spasm. The condition may progress to complete occlusion and infarction or may heal.

Treatment of unstable angina and NSTEMI:

a. Hospitalization and bed rest.

b. Anticoagulation by IV heparin or preferably by subcutaneous low molecular weight heparins

(LMWH) e.g. enoxaparin (Clexan) I mg/kg every 12 hours. Nitroglycerine is given initially by IV drip

and later by sublingual or oral route as long as anginal pain exists.

c. Beta-blockers should be routinely added.

d. Aspirin 150 mg should be immediately chewed in order to inhibit platelet aggregation then orally

daily afterwards.

e. Clopidogrel (Plavix, 75 mg) is a potent antiplatelet drug that should be added to aspirin. 4 tablets

(300 mg) should be given initially followed by I tablet daily.

f. When facilities exist, all cases of UA or NSTEMI who are at high risk should have coronary

angiography done and, if the anatomy is suitable, should have coronary angioplasty (PCI) with

stenting.

High risk is considered present if:

i. Pain persists over 20 minutes. iv. Elevated troponin level.

ii. Hemodynamic instability occurs. v. Age over 70.

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iii. Progressive ECG changes. vi. Presence of diabetes.

vii. Previous MI or PCI. viii Presence of renal insufficiency.

MYOCARDIAL INFARCTION

INCIDENCE: Myocardial infarction is the commonest cause of death in adults in the world today. It is

much more common in males between 40 and 70 years and is uncommon in females before

menopause.

ETIOLOGY: The vast majority of cases are due to coronary atherosclerosis. The predisposing factors

for infarction and atherosclerosis are the same:

d. Hypertension.

e. Hypercholesterolemia.

f. Positive family history

g. Smoking

h. Diabetes

i. Lack of physical activity and excessive mental strain

j. Obesity.

PATHOLOGY: The basic mechanism underlying MI is disruption of an atherosclerotic plaque. The

contents of the plaque are exposed to blood stream and platelets adhere to the ulcerated lesion. Further

platelet aggregation follow and then a thrombus forms that totally occludes the artery.

SITE:

Infarction mostly involves the left ventricle and the ventricular septum. Right ventricular infarction is

uncommon and always occurs as an extension of infarction involving the inferior surface of the left

ventricle because the blood supply of both areas is usually derived from the same artery (the right

coronary artery).

The location and extent of infarction depend on the anatomic distribution of the vessel involved and the

adequacy of collateral circulation. Thrombosis occurs most commonly in the anterior descending

branch of the left coronary artery resulting in infarction of the anterior wall of the left ventricle and the

septum (anteroseptal infarction). Occlusion of the circumflex artery produces anterolateral infarction.

Obstruction of the right coronary artery leads to infarction of the postero-inferior part of the left

ventricle and may involve the right ventricle.

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Fig: Anteroseptal MI. (A) One hour of onset of symptoms. (B) 12 h later.

Infarction may involve the whole thickness of the myocardium i.e. transmural; or only the

subendocardial region - nontransmural infarction).

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Fig: Inferior myocardial infarction. (A) 1 h after the onset of symptoms. (B) Right sided chest leads

recorded simultaneously. ST elevation is apparent in leads V3R to V7R, indicating right ventricular

infarction. (C) 12 h later. There are now deep Q waves in leads II, III and aVF.

EFFECTS:

- Part of the myocardium becomes necrotic.

- If the area involved is very big, a very large part of the left ventricle will not share in the

contraction resulting in a sudden severe drop in the cardiac output. Cardiogenic shock occurs.

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- Sudden death (sudden cardiac arrest within one hour of the symptoms) occurs in > 20 % of

myocardial infarctions.

- If the area involved is big left ventricular failure occurs and may lead to acute pulmonary edema.

- The ischemic area around the infraction is always electrically unstable and is the site of genesis of

many arrhythmias.

- Ischemia or infraction may involve a papillary muscle. The mitral valve becomes unsupported and

ischemic mitral regurgitation results.

- The infracted area may rupture. If rupture occurs in the left ventricular free wall, hemopericardium

and death occur. If the ventricular septum ruptures an acquired ventricular septal defect results.

- When the visceral pericardium overlying the infarction is also involved it becomes roughened and

pericarditis results.

- When the endocardium is involved a mural thrombus may form over it in the left ventricle. Parts

of the thrombus may detach later as systemic emboli.

a. When the infarction heals it is replaced by fibrous tissue which may yield and expand gradually

under the high intraventricular pressure resulting in a myocardial aneurysm. It leads to LV failure and

cavity thrombus.

CLINICAL PICTURE:

Symptoms:

1) Cardiac pain: sudden onset of severe pain. It is of aching, burning or constricting character

unrelated to exercise. The pain is maximal behind the sternum but may radiate to all the central part of

the chest, neck, jaws, epigastrium, both shoulders, specially the left, and the left arm. The pain is

identical with that of angina in character, site and radiation but much more severe and prolonged and is

not relieved by rest or by sublingual nitroglycerine. The pain may be preceded by recent onset of

angina or sudden increase in its severity (crescendo angina).

2) Accompanying the pain there is usually profuse sweating, sometimes nausea and vomiting or

syncope.

Signs:

d. Many patients have a sympathetic response leading to vasoconstriction, hypertension and

tachycardia. Sometimes, especially in inferior infarction, there may be strong vagal reflexes with sinus

bradycardia and hypotension.

e. Cardiac examination is commonly normal but the fourth heart sound is usually present. The

appearance of gallop rhythm, pericardial rub or systolic murmur indicates complications.

Other manifestations: a. Fever. b. Leukocytosis. c. High erythrocyte sedimentation rate.

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Atypical Presentations:

1. Painless myocardial infarction occurs in the following situations:

2. Pain may be atypical in character site or radiation.

3. Presentation may be dominated by one of the complications, e.g. cases may present with shock,

pulmonary edema, arrhythmias, etc.

INVESTIGATIONS:

I. Cardiac Enzymes: The most commonly used enzyme marker is creatinine kinase (CK) and its MB

fraction (CK-MB). It starts to rise in the serum 4-6 hours after the onset of infarction, reaches peak

value in 12 hours then starts to decline over 48-72 hours. CK-MB is cardio-specific while the total CK

is not.

Troponin (T or I), however, is the standard diagnostic enzyme test world-wide because it also has

value in risk stratification. Its level correlates with the extent of myocardial damage and indicate the

degree of risk. It also rises in 4-6 hours peaks in 12 hours but disappears in 14 days, so that it is also of

value in diagnosis of late cases.

2. Electrocardiography:

The final proof of myocardial infarction is by the electrocardiogram. The classic evolution of changes

is peaked (hyperacute) T waves, to ST segment elevation, to appearance of Q waves, to T wave

inversion and return to ST segment to base line. This may occur over few hours to several days.

However, Q waves may not appear in 30% of acute infarctions i.e. non-transmural or non-Q wave

infarction. In these cases the infarction is subendocardial or consists of localized areas of necrosis

within ischemic but viable myocardium.

3. Echocardiography:

Echo will show hypokinesia or akinesia of the ischemic or necrotic ventricular wall. The overall left

ventricular function can be estimated by the ejection fraction and it has a very important role in

evaluating the immediate risk and the long-term prognosis of the patient. Two dimensional and

Doppler echo are also very valuable in diagnosing complications such as ischemic mitral regurgitation,

ventricular septal defect, myocardial aneurysm, pericardial effusion, etc.

4. Other laboratory findings include leukocytosis and high ESR.

COMPLICATIONS:

1) Sudden Death: Occurs in 30% of acute MI in first month.

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2) Arrhythmias: these are very common in the first few days after the infarction. They are the most

common cause of death in the early stages. Nearly any arrhythmia can occur but the most common are:

a. Ventricular extrasystoles occur in over 90% of cases.

b. Ventricular tachycardia which causes marked circulatory deterioration.

c. Ventricular fibrillation (VF) is fatal if untreated within minutes. Primary VF occurs very early and is

caused by the onset of ischemia. Secondary VF occurs late and is usually secondary to heart failure,

shock etc and indicate poor prognosis.

d. Atrial arrhythmias (e.g. extrasystoles, tachycardia, and fibrillation) are common when there is heart

failure.

3) Left Ventricular Failure: results from extensive infarction and may present with dyspnea and

orthopnea or, in severe cases, with acute pulmonary edema. On examination sinus tachycardia, a third

heart sound or gallop rhythm and bilateral basal crepitations will be found.

Right Ventricular Infarction is uncommon but may lead to right ventricular failure presenting with low

cardiac output and high venous pressure.

4)Cardiogenic Shock: results from very extensive infarction. The cardiac output is very low. The

patient is pale, cold, sweating and mentally dull with hypotension, peripheral cyanosis, oliguria or even

anuria. When shock is severe the prognosis is very bad: mortality is over 80%. Shock may co-exist

with left ventricular failure.

5) Conduction Defects: heart block may occur in the course of inferior infarction. This is because the

right coronary artery usually supplies both the AV node and the inferior surface of the heart. In these

cases the block is due to vagal reflexes or to transient ischemia of the A-V node and is usually transient

and may respond to atropine. If the bradycardia causes hemodynamic deterioration a temporary

external pacemaker should be instituted to drive the heart at rate of 60/minute via a transvenous

electrode catheter.

Bundle branch block may occur. When this is associated with prolonged P-R interval it means that

complete heart block may develop.

6) Mitral Regurgitation: is due to ischemia and fibrosis of a papillary muscle. Mild degrees of

regurgitation are common. Rupture of a papillary muscle leads to very severe mitral regurgitation and

intractable heart failure.

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7) Pericarditis: when the pericardium is involved a pericardial rub is heard 2 to 3 days after the

infarction and may lead to recurrence of pain. The pain of pericarditis must be differentiated ischemic

cardiac pain that may be caused by extension of the infarction. Pericardial pain is increased with

movements and inspiration. Hemorrhage in the pericardial sac (hemopericardium) may occur,

especially if anticoagulants are used in the presence of pericarditis.

8) Emboli: embolism following myocardial infarction has many sources:

a. When the endocardium is involved in the infarction, mural thrombi may form in the LV cavity and

may lead to embolism in brain, other areas.

b. In cases complicated by atrial fibrillation, thrombosis may form in the atria.

9) Myocardial Aneurysm: occurs after the healing stage and may lead to:

a. Heart failure.

b. Thrombosis and embolism.

c. Diffuse or double apical impulse.

10) Rupture of the heart: may occur leading to hemopericardium, cardiac tamponade and sudden

death. Rupture of the ventricular septum results in acquired ventricular septal defect. A pansystolic

murmur appears at the lower left sternal edge. It may be associated with left ventricular failure and low

cardiac output.

11) Ventricular Remodeling: The healed myocardial infarction zone consists mainly of fibrous tissue

that yields under the high intraventricular pressure resulting in infarct expansion. Other healthy areas of

the myocardium hypertrophy in order to compensate for the areas of lost function. The ventricular

cavity assumes a more globular form instead of the normal ovoid shape. These changes, taken together,

are called ventricular remodeling and result in long-term deterioration of cardiac function and may

ultimately end in left ventricular failure.

12) Depression, fear of death, and nervousness: Important complication to infarction.

PROGNOSIS:

If untreated, one fifth of cases of myocardial infarction die in the first few hours (sudden cardiac

arrest). Another fifth die from various complications during the first month after infarction (these

figures improved after advent of primary balloon angioplasty). Of those who recover 75% live 5 years

and 50% for 10 years. Some may show post infarction angina pectoris.

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DIAGNOSIS: Myocardial infarction is diagnosed when there are two of the following:

1- Typical pain.

2- Typical ECG changes.

3- Enzyme rise.

4- Hypokinesia-akinesia by echocardiography.

In typical cases the main diagnostic feature is ischemic cardiac pain associated with ST elevation of 1

mm or more in two contiguous ECG leads and elevated cardiac enzymes. However, the following

diseases may simulate the cardiac pain of myocardial infarction.

1. Pulmonary embolism: When a main pulmonary artery is occluded the pain may simulate that of

myocardial infarction. Dyspnea is the most common and outstanding symptom of pulmonary

embolism. Enzymes CK, (MB), troponin and SGOT are normal and LDH is high. Electrocardiographic

signs are different. If pulmonary infraction occurs the pain is pleuritic, increases with inspiration and

coughing and is associated with hemoptysis. D-Dimer enzyme rises in pulmonary infarction.

2. Acute pericarditis:

3. Dissecting aneurysm of the aorta:

4. Abdominal conditions: as acute indigestion, perforated peptic ulcer, biliary colic, acute

cholecystitis, acute pancreatitis, hiatus hernia, esophagitis, etc... may simulate infarction. In these cases

the pain is more epigastric and is accompanied by tenderness and rigidity over the affected viscous.

The electrocardiogram and cardiac enzymes are normal.

5. Spontaneous pneumothorax produces pain that is usually unilateral with severe dyspnea. The X-

ray is diagnostic and the ECG and the enzymes are normal.

6. Spinal and chest wall conditions like spondylitis, cervical disc, herpes zoster, pleurodynia etc.

7. Pneumonia and pleurisy.

Treatment of S-T elevation myocardial infarction (STEMI):

. Immediate treatment

. Aspirin, Clopidogrel

. Reperfusion therapy: - thrombolysis, - angioplasty PCI)

. Beta-blockers, Angiotensin converting enzyme inhibitors.

. Nitrates and other drugs.

All cases of chest pain must be managed as an emergency. If the ECG show, elevation of the S-T

segment of 1 mm or more in two contiguous leads, this must be considered a case of S-T elevation

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myocardial infarction (STEMI) and the patient should be rushed to hospital immediately. The primary

aim of treatment is:

a. Limit infarction size. b. Relieve symptoms. c. Prevent or treat complications.

1) Coronary Care Units: The most common complications and cause of death in acute myocardial

infarction are arrhythmias. In coronary care units, electric shock (i.e. defibrillation and cardioversion

for ventricular fibrillation and tachycardia) and pacemakers (for heart block and cardiac arrest) are

continuously available in addition to standard drugs. Resuscitation from cardiac arrest is most often

required at this time. The most urgent measure is the relief of pain by IV morphine. If morphine is not

available pethedine (50 mg) can be used.

2) Aspirin: A 300 mg tablet of aspirin (soluble or chewable) should be given as early as possible in

acute infarction in order to reduce platelet aggregation at the site of atherosclerotic plaque.. Clopidogrel

(Plavix) 4 (75 x 4 = 300 mg) tablets are given immediately also.

3) Reperfusion: By this is meant opening up of the occluded vessel so that blood can again perfuse

the myocardium. It is the most important therapeutic measure and must be done as soon as possible. It

is most useful when performed in the first 6 hours. Its value is doubted after 12 hours as by then

necrosis of myocardium has already happened. It can be achieved in two ways:

a. Thrombolytic or Fibrinolytic Agents: e.g. streptokinase, tissue plasminogen activators, and

tenectaplase (TNK), etc, can dissolve coronary thrombus if they are administered within less than 6 (12

to 24 ) hours of its formation. Reperfusion of the occluded artery is achieved in the majority of cases

and infarction is reduced or prevented. 1.5 million l.U of streptokinase is usually given over a period of

one hour by l.V. infusion. These drugs are indicated in every patient with infarction and with elevation

of the S-T segment in two contiguous ECG leads (STEMI). Hemorrhage is the most important

complication of fibrinolytic drugs. Contraindications to their use include bleeding diathesis,

uncontrolled hypertension, recent internal bleeding, recent stroke, pregnancy, recent trauma or surgery

and age above 80 years.

Class IIa 1. Reperfusion therapy is reasonable for patients with STEMI and symptom onset within

the prior 12 to 24 hours who have clinical and/or ECG evidence of ongoing ischemia.

Primary PCI is the preferred strategy in this population. (Level of Evidence: B)

b. Coronary angioplasty (Percutaneous Coronary Intervention PCI):

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Balloon dilatation of the site of obstruction in the coronary arteries is probably the best method and it

treats both the recent total occlusion and the underlying atherosclerotic plaque that led to it. If

successful the ischemic zone is reperfused with blood and the myocardium at risk is salvaged. It can be

used as the initial therapy (primary angioplasty) or when thrombolysis is contraindicated or has been

used but failed.

1. Class I: Primary PCI is the recommended method of reperfusion when it can be

performed in a timely fashion by experienced operators. (Level of Evidence: A)

4) Anticoagulant Drugs: Anticoagulation by unfractionated or low molecular weight heparin

definitely decreases the risk of thrombosis in the deep veins of the leg and subsequent pulmonary

embolism. They should be given during the acute phase when the patient is immobilized in bed for a

long period e.g. in cases associated with heart failure, shock, or in patients with previous venous

disease.

5) The use of beta blocking drugs is needed in patients with marked sympathetic overactivity

presenting with sinus tachycardia without evidence of heart failure. The bradycardia resulting from beta

blockade reduces the myocardial needs for oxygen and thus may help limit he infarction size.

6) ACE inhibitors: They have favorable impact on ventricular remodeling, improvements of

hemodynamics and reduction in congestive heart failure. They proved to reduce death from AMI.

7) Nitrates: They reduce ventricular filling pressure, wall tension and cardiac work with improvement

in coronary blood flow.

8) Physical Rest: Rest is essential until the infarct heals. Initially, it must be absolute except for the use

of a bed-side commode. Rest period depends on severity and complications. In uncomplicated cases 1

week bed rest (arm chair allowed) followed by a period of restricted indoor activity.

9) Mental Rest: All patients must be given a tranquilizer to relieve anxiety. If necessary a sedative or

hypnotic is given to help sleep.

10) Oxygen: Oxygen should be given specially if shock, left / ventricular failure or persistent chest

pain is present.

Diet: During the first few weeks diet must be: Low calorie, low salt, light, and easily digestible.

Smoking: Smoking must be completely forbidden during the acute phase and preferably for life

afterwards.

Treatment of complications:

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Heart failure is treated by diuretics, vasodilators and positive inotropic agents e.g. dobutamine.

Digoxin is not recommended in acute myocardial infarction except to control the ventricular response

in rapid atrial fibrillation.

Pulmonary edema by intravenous morphine, IV frusemide, positive inotropic drugs, oxygen,

aminophylline, and vasodilators.

Cardiogenic shock: The usual first aid measures are warmth, oxygen, relieve of pain, and raising the

foot of the bed. Other measures that are useful may include:

a. Drugs that increase myocardial contractility e.g. dopamine dobutamine. At low loses (2-4

mg/kg/mm) dopamine improves the renal blood flow. At intermediate doses (2.5-10 mg/kg/mm) it

stimulates the myocardial contractility. At higher doses it is a potent vasoconstrictor.

b. Fluids in cases with hypovolemia, due to sweating, vomiting and lack of fluid intake.

c. Vasoconstrictors as noradrenaline are used if above measures fail.

d, Coronary angioplasty is the best treatment of cardiogenic shock after MI.

f. When shock is due to the presence of severe ischemic mitral regurgitation or ventricular septal

defect, then emergency surgery must be done to correct the cause.

g. If the above measures fail the patient is stabilized by assisted circulation. A balloon tipped catheter is

introduced from the formal artery to the descending aorta and inflated mechanically during every

diastole. This is intra-aortic balloon counterpulsation. It partly relieves the heart and maintains blood

flow in diastole until the myocardium recovers.

Ventricular extrasystoles (if frequent) are best treated by lignocaine IV bolus or drip. Other oral drugs

such as amiodarone can be used for oral prophylaxis against recurrent arrhythmia.

Ventricular tachycardia is best treated by electric shock (cardioversion). If this is not available

intravenous lignocaine or amiodarone or procainamide can be used.

Ventricular fibrillation causes complete cessation of the circulation. Life is sustained by immediate

cardiac massage and artificial respiration. Defibrillation by electric shock causes the return of sinus

rhythm.

Ventricular aneurysm may result in refractory heart failure and recurrent emboli. It can be excised

surgically.

Heart block is treated by atropine and temporary external pacemaker. Sinus bradycardia should be

treated with atropine.

Long Term Management:

As in angina pectoris, the management includes:

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1. Increase in myocardial oxygen supply by coronary vasodilators and exercise, which promotes

formation of collaterals.

2. Reduction of myocardial oxygen needs by reduction of weight, sedation and beta-adrenergic

blockers. The long-term use of beta- blockers has definitely been shown to reduce the mortality and the

incidence of re-infarction.

3. Prevention of expansion of the area of infarction and progressive development of heart failure

(ventricular remodeling) by the use of angiotensin converting enzyme (ACE) inhibitors on long term

basis. They should be started from the first week after large infarction and continued indefinitely.

4. Prevention of further progression of atherosclerosis.

5. Long-term anticoagulants: The majority of cardiologists today tend not to use anticoagulants at all or

to discontinue them gradually as the patient returns to full activity.

6. If angina persists after the infarction (post-infarction angina) the patient must be subjected to

coronary arteriography to delineate the extent of coronary arterial lesions and decide the need for

surgery or balloon-angioplasty.

7. Rehabilitation: Graduated activity with increasing exercise is now considered the major factor in

increasing physical fitness and allowing more work to be done with the least oxygen needs.

ANTICOAGULANTS

HEPARIN: Two forms of heparin exist: unfractionated heparin (UFH) and low molecular weight

heparin (LMWH). UFH is given by the IV route starting with a loading dose of 5000 to 10000 units mg

then continued either by IV infusion of 1000 U/hour or by intermittent injection of 5000 U/4 hours. It

combines in the body with antithrombin III and acts both on activated factor II (thrombin) factor X.

LMWH act primarily on factor X. They do not need laboratory monitoring. They are given

subcutaneously every 12 hours. Eg enoxaparin (Clexan).

ORAL ANTICOAGULANTS: These drugs act by inhibiting the synthesis of prothrombin in the

liver. They do not affect already circulating prothrombin. That is why their action is delayed for about

48 hours and persists 48 hours after their withdrawal until new prothrombin is formed.

GENERAL INDICATIONS OF ANTICOAGULANTS:

1. Pulmonary embolism. 2. Deep vein thrombosis.

3. Atrial fibrillation. 4. Arterial embolism and thrombosis.

5. Some cases of myocardial infarction e.g. those associated with ventricular aneurysm or left

ventricular cavitary thrombus.

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GENERAL CONTRA-INDICATIONS OF ANTICOAGULANTS:

1. All conditions predisposing to hemorrhage, e.g. recent surgery, peptic ulcer, open ulcers,

gastrointestinal bleeding, etc... 2. In pericarditis to avoid (hemopericardium). 3. In infective

endocarditis. 4. In severe hypertension.

ANTI PLATELET DRUGS

1. Aspirin 75-160 mg daily is the most effective and most widely used.

2. Clopidogrel (Plavix, Stroka, 75 mg). Inhibit ADP action on platelet receptors.

3. The IIb IIIa receptor blockers: are the most potent but can be given only by IV infusion and are

used in patients with acute coronary syndrome who will undergo coronary angioplasty. E.g. tirofiban

(Aggrastat).

4. Dipyridamole (Persantin, 75 mg t.i.d.) is rarely used now.

2013 ACCF/AHA Guideline for the Management of ST-Elevation Myocardial Infarction

Reperfusion therapy for patients with STEMI. The bold arrows and boxes are the

preferred strategies. Performance of PCI is dictated by an anatomically appropriate culprit

stenosis. *Patients with cardiogenic shock or severe heart failure initially seen at a non–PCI-capable

hospital should be transferred for cardiac catheterization and revascularization as soon as possible,

irrespective of time delay from MI onset (Class I, LOE: B). †Angiography and revascularization should

not be performed within the first 2 to 3 hours after administration of fibrinolytic therapy. CABG

indicates coronary artery bypass graft; DIDO, door-in–door-out; FMC, first medical contact; LOE,

Level of Evidence; MI, myocardial infarction; PCI, percutaneous coronary intervention; and STEMI,

ST-elevation myocardial infarction.

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Class I 2. Reperfusion therapy should be administered to all eligible patients with STEMI with

symptom onset within the prior 12 hours. (Level of Evidence: A)

3. Primary PCI is the recommended method of reperfusion when it can be performed in

a timely fashion by experienced operators. (Level of Evidence: A)

4. Emergency medical services transport directly to a PCI-capable hospital for primary

PCI is the recommended triage strategy for patients with STEMI, with an ideal FMC-

to-device time system goal of 90 minutes or less. (Level of Evidence: B)

5. In the absence of contraindications, fibrinolytic therapy should be administered to

patients with STEMI at non–PCI-capable hospitals when the anticipated FMC-to-

device time at a PCI-capable hospital exceeds 120 minutes because of unavoidable

delays. (Level of Evidence: B)

189

6. When fibrinolytic therapy is indicated or chosen as the primary reperfusion strategy,

it should be administered within 30 minutes of hospital arrival.* (Level of Evidence:

B)

Class IIa 2. Reperfusion therapy is reasonable for patients with STEMI and symptom onset within

the prior 12 to 24 hours who have clinical and/or ECG evidence of ongoing ischemia.

Primary PCI is the preferred strategy in this population. (Level of Evidence: B)

The guidelines are 60 pages, this is a short summary for 5th

year medical students.

The guidelines never mentioned if the patient have money to pay for all this.

Samir Rafla: Principles of Cardiology pages 112 to end

HYPERTENSION AND HEART DISEASE

Hypertension is a major risk factor for cardiovascular morbidity and mortality. It accelerates the

process of atherosclerosis in the coronary, cerebral and renal arteries, as well as increasing the

workload of the heart. As a result, the hypertensive patient is at risk of developing myocardial

infarction, stroke, renal failure and congestive cardiac failure. In total, hypertension is probably directly

or indirectly responsible for 10-20% of all deaths.

The normal blood pressure ranges from 90 – 139 mmHg systolic and 60 – 89 diastolic. The BP is

classified as follows

190

From ESC Guidelines_arterial_hypertension-2013

191

Stratification of total CV risk, from ESC Guidelines_arterial_hypertension-2013

Malignant hypertension is said to be present when there is papillodema and diastolic BP > 130.

Isolated systolic hypertension when diastolic BP < 90 and systolic BP 140-159 (borderline isolated

systolic hypertension; > 160 mmHg (isolated systolic hypertension).

Prevalence of hypertension: According to survey done by Prof. Mohsen Ibrahim, high BP was found

in 26% of Egyptian population above age 25 years (thus about 9 million Egyptians have hypertension).

Blood pressure is usually measured in the arm. The usual method involves a ‘cuff’ which is wrapped

around the upper arm. The cuff contains an inflatable rubber bladder. The cuff is then inflated with air

until the pressure from the cuff occludes the brachial artery. The cuff is then slowly deflated and the

pressure in the cuff is continuously measured using either a mercury manometer or an aneroid

manometer. At the same time as the operator regulates the deflation of the cuff, he or she listens with a

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stethoscope over the brachial artery immediately distal to the cuff. As the cuff is deflating, the pressure

at which regular sounds first appear over the brachial artery is taken as the systolic blood pressure. As

the pressure continues to fall, the sounds become muffled and then disappear. The pressure at which the

sounds disappear completely is the diastolic pressure.

AETIOLOGY OF HYPERTENSION

In 95% of patients with high blood pressure, no specific cause can be identified. This condition is

termed ‘essential’ or ‘primary’ hypertension. In approximately 5% of hypertensive patients, a specific

cause can be identified and the hypertension is termed ‘secondary’. Although secondary hypertension

accounts for a small minority of all hypertensive patients, it is important to identify this condition

because specific and potentially curative treatment may be available.

Essential hypertension: Causes (predisposing factors):

Genetic influences

Dietary influences

There is an undoubted relationship between weight and blood pressure. Weight loss in the obese

substantially lowers the blood pressure.

Excessive sodium chloride intake.

High intake of saturated fats.

High alcohol consumption.

Cigarette smoking.

Physical activity: Physical exercise can reduce blood pressure in hypertensive subjects. This suggests

that inactivity may play a role in the genesis of hypertension in some individuals.

Hormonal changes

The adrenergic and renin—angiotensin systems, has a role in the genesis of essential hypertension.

Haemodynamic changes

There is good evidence that baroreceptors are reset in hypertension.

Secondary hypertension

The most common causes of secondary hypertension are renal disease, adrenal disease, coarctation of

the aorta and drug-related hypertension.

Renal disease

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All forms of parenchymal renal disease can be associated with significant hypertension. These include

acute and chronic glomerulonephritis, chronic pyelonephritis and polycystic kidney disease. Control of

the blood pressure in all of these conditions is important and slows the progression of renal damage.

Renal artery stenosis as a cause of hypertension deserves special consideration. In this condition, the

stenosis may be unilateral or bilateral and may take the form of a fibromuscular narrowing in young

patients or atheromatous narrowing in older patients, who will often have evidence of atherosclerosis

elsewhere. Renal artery stenosis results in ischaemia of the kidney with high circulating levels of

angiotensin II. High levels of angiotensin II then lead to hypertension by two different mechanisms.

Hypertension in these patients is often relatively resistant to drug treatment. Angiotensin converting

enzyme (ACE) inhibitors, by preventing the release of angiotensin II, will lower the blood pressure

markedly but should be avoided in patients suspected of having renal artery stenosis because they

reduce renal perfusion and may result in renal infarction. If a patient has bilateral renal artery stenoses,

the introduction of an ACE inhibitor can precipitate acute renal failure.

Fig: The renin—angiotensin system.

Investigation of suspected ‘renal’ hypertension - Abdominal ultrasonography provides a simple non-

invasive means of assessing renal anatomy in patients with a suspected renal cause for hypertension. In

patients with chronic nephritis, kidney size is reduced. In patients with pyelonephritis, there is likely to

be dilatation of the calyceal system. In unilateral renal artery stenosis, kidney size is reduced on the

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side of the stenosis. If renal artery stenosis is suspected, then renal arteriography remains the

investigation of choice although the diagnosis can now often be made using either multislice CT or

magnetic resonance imaging (MRI). If the patient is shown to have either unilateral or bilateral renal

artery stenosis, then renal revascularization by renal artery angioplasty should be considered.

Endocrine disease

Cushing’s syndrome

This results from cortisol excess and may be due to hyperplasia of the adrenal cortex, adrenal tumours,

or to the excessive administration of glucocorticoids or adrenocorticotrophic hormone (ACTH).

Adrenal hyperplasia is often the result of increased ACTH production by a pituitary microadenoma.

Hypertension, which occurs in more than 50% of cases, may be severe and may proceed to the

malignant phase. Other features of the syndrome are muscle weakness, osteoporosis, purple cutaneous

striae, obesity of the trunk, a ‘buffalo’ hump, a ‘moon’ facies and diabetes mellitus. There may also be

hirsutism, amenorrhoea, a liability to spontaneous bruising, and dependent oedema.

Diagnosis: The diagnosis should be suggested by the combination of hypertension, diabetes and truncal

obesity. Investigations include:

• excessive 24-h urinary free cortisol excretion

• failure to suppress plasma cortisol levels following dexamethasone administration

• the ACTH levels are valuable in determining the cause, being high with pituitary tumours and low

if the adrenal is responsible

• CT or MRI imaging of the adrenal glands.

Management: Treatment depends upon the aetiology of the condition. Surgical removal of one or both

adrenal glands or of a pituitary tumour may be necessary

Primary aldosteronism

Aldosterone, which is secreted by the zona glomerulosa of the adrenal cortex, promotes sodium

reabsorption and potassium excretion in the distal tubules of the kidney. Normally, aldosterone

secretion is largely regulated by angiotensin, but in primary aldosteronism there is an overproduction of

aldosterone as a result of an adrenal cortical adenoma (Conn’s syndrome) or bilateral hyperplasia;

angiotensin and, therefore, plasma renin levels are abnormally low. The condition occurs most often in

young and middle-aged females. Because of the mode of action of aldosterone, the symptoms and signs

are related to sodium retention, hypokalaemia and hypertension. Frequently, the patient presents with

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mild to moderate hypertension, but the predominant complaints are those of muscle weakness,

headache, thirst and polyuria. The hypertension is seldom severe and malignant changes are rare. There

is usually hypokalaemia, with a serum potassium level of less than 3.0 mmol/L and a serum sodium

concentration that is normal or high. Characteristically, there is a metabolic alkalosis and a low serum

chloride level. The diagnosis should be suspected in patients with hypertension and hypokalaemia,

particularly if this is associated with hypernatraemia. However, hypokalaemia is not uncommon in

other hypertensive patients, particularly if they have been treated with diuretics. Furthermore, patients

with malignant hypertension develop ‘secondary aldosteronism’ with low serum potassium. These

patients usually do not have high serum sodium.

Diagnosis. The diagnosis is suggested by:

• hypokalaemia, persisting after stopping diuretic therapy

• excessive urinary potassium loss

• elevated plasma aldosterone levels

• suppressed renin levels which fail to rise on assumption of an upright posture

• CT or MRI imaging is now the investigation of choice in establishing the presence of an adenoma

and differentiating this from hyperplasia.

Management. Adenomas should be removed surgically. Patients with hyperplasia should be treated

medically with spironolactone or amiloride, which antagonize the actions of aldosterone.

Phaeochromocytoma

Phaeochromocytoma arises in chromaffin tissue, usually in the adrenal gland. It is sometimes described

as the ‘10% tumour’. This is because 10% are said to arise outside the adrenal gland, 10% are

malignant and 10% are bilateral. The tumours usually secrete noradrenaline (norepinephrine), but

adrenaline (epinephrine) may predominate.

Phaeochromocytomas may produce either paroxysmal or persistent hypertension. The paroxysms are

associated with the sudden onset of bilateral headache, and with perspiration, palpitations and pallor

(features often regarded as neurotic). The attacks usually last from a few minutes to an hour. If the

hypertension is persistent, the clinical picture is that of severe hypertension, often of the malignant

variety. Because of the hypermetabolic state induced by the phaeochromocytoma, the patients are

rarely obese.

Diagnosis: The diagnosis should be suspected in any severe case of hypertension, particularly if the

hypertension is paroxysmal. The diagnosis is confirmed by:

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• excessive excretion of the catecholamine metabolite vanilmandelic acid (VMA) in the urine is a

useful screening test

• urine and plasma catecholamine levels

• CT scanning to localize the tumour.

Management. Phaeochromocytomas should be removed surgically. This is a potentially hazardous

procedure and requires close control of the blood pressure and careful anaesthesia. Beta-adrenergic

blocking drugs should not be used alone because unopposed alpha-adrenergic activation may aggravate

hypertension and lead to serious complications such as stroke. This can be avoided by the initial use of

an alpha-adrenergic blocking drug. The non-competitive alpha-antagonist phenoxybenzamine is

frequently chosen. Once alpha-adrenergic blockade is fully established, beta-blockade can be added.

Coarctation of the aorta

This is a congenital condition associated with a narrowing of the lumen of the aorta just beyond the

origin of the left subclavian artery. See page 33.

PATHOPHYSIOLOGY OF HYPERTENSION

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The high blood pressure in essential hypertension is due to increased peripheral vascular resistance as a

result of widespread constriction of the arterioles and small arteries. The cardiac output and the

viscosity of the blood are normal (usually). In the earlier stages, the hypertension is largely due to

increased arteriolar muscle tone, but subsequently, structural alterations take place in the arterioles.

These changes may account for the fact that hypertension tends to beget (induce) further hypertension,

and the removal of the cause of hypertension does not necessarily lead to a fall in the blood pressure to

normal.

In the heart, there are two major consequences of sustained hypertension. The increased work of the

heart imposed by the higher resistance results in hypertrophy of the myocardial cells. As this process

progresses, the myocardial hypertrophy may exceed the coronary blood supply; this occurs particularly

in the subendocardial layers which are the most vulnerable to ischaemia; ultimately, leads to heart

failure. The second effect of hypertension is to accelerate the development of atherosclerosis. This

occurs not only in the coronary arteries but also in the cerebral arteries, particularly those of the basal

ganglia, and in the renal arteries. The mechanism of this action is long-standing mechanical stresses; in

experimental situations, hypertension, like cigarette smoking and hypercholesterolaemia, has been

shown to induce dysfunction of the endothelial layer of the coronary arteries which, in turn, is

thought to start the development of atherosclerosis.

Examination and Investigation of the Hypertensive Patient

Examination and investigation should be directed towards the detection of an underlying cause of

hypertension (see secondary hypertension) and the assessment of end-organ damage, which may

influence the decision to treat the patient. Blood pressure levels should be recorded after the patient has

been lying quietly for 5 mm.

Examination of the hypertensive patient

Clinical examination should take note of:

Signs suggestive of secondary hypertension

• Features of endocrine abnormalities, particularly Cushing’s syndrome

• Multiple neurofibromatoma — present in 5% of patients with phaeochromocytoma

• Inappropriate tachycardia, suggesting catecholamine excess

• Abdominal or loin bruits, suggesting renal artery stenosis

• Renal enlargement (suggestive of polycystic kidney disease)

• Radial-femoral delay, due to coarctation of the aorta.

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Signs suggestive of end-organ damage

• A forcible and displaced apex beat due to left ventricular hypertrophy

• Added heart sounds. A fourth sound may be audible, reflecting decreased ventricular compliance. As

failure develops a third sound may occur.

• Fundal examination to detect hypertensive retinopathy.

Fundoscopy: Grading:

• grade I - increased tortuosity of the retinal arteries with increased reflectiveness, termed silver wiring

• grade 2 - grade I with the addition of compression of the veins at arteriovenous crossings (AV nipping)

• grade 3 - grade 2 with the addition of flame-shaped haemorrhages and ‘cotton wool’ exudates

• grade 4- grade 3 with the addition of papilloedema — the optic disc is pink with blurred edges and the

optic cup is obliterated.

Investigation of the hypertensive patient

• ECG. This is usually normal in patients with mild hypertension but may show evidence of left

ventricular hypertrophy. This is characterized by tall R waves in the lateral chest leads and deep S

waves in the anteroseptal leads. LVH is said to be present if the sum of the S wave in VI and the R

wave in V5 or V6 exceeds 35 mm. In severe hypertrophy, or if there is accompanying ischaemic heart

disease, the T waves in the lateral chest leads become flattened and then inverted, and the ST segment

may show down-sloping depression in the same leads. This is the so-called ‘left ventricular

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hypertrophy and strain’ pattern (Fig. 14.4) which carries a high risk of major events including sudden

death (30—40% 5-year mortality rate).

• Urinalysis. Proteinuria, hyaline and granular casts may be found when there is renal disease or

malignant hypertension.

• Urea and electrolytes. A raised level of urea suggests renal impairment, which may be the cause or an

effect of hypertension. A low serum potassium concentration in the absence of diuretic therapy might

suggest Conn’s or Cushing’s syndrome.

• Lipids. An increased level of cholesterol is a risk factor for cardiovascular events, which may require

specific treatment and which should be monitored in all hypertensive patients.

Fig: ECG showing left ventricular hypertrophy and ‘strain’ in a patient with severe hypertension. This

pattern is characterized by large voltages in the chest leads and the presence of ST segment depression

and T wave inversion in leads V5 and V6.

The following additional investigations may also be helpful:

• 24-h ambulatory blood pressure monitoring.

• Echocardiography. Echocardiography is much more sensitive than the ECG for the detection of

left ventricular hypertrophy.

• Detailed investigation of suspected secondary hypertension. This may involve CT or MRI of the

adrenal glands, MRI renal angiography and 24-h urine collections for catecholamines.

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It is impractical to screen all hypertensive patients for secondary causes of hypertension. Selection of

patient groups for further investigation is arbitrary, but investigation is particularly appropriate in the

following groups:

• young patients under 40 years of age

• patients with malignant hypertension

• patients resistant to antihypertensive therapy

• patients with unusual symptoms (such as sweating attacks or weakness) which might suggest an

underlying cause

• patients with abnormal renal function, proteinuria or haematuria

• patients with hypokalaemia off diuretic therapy.

THE DECISION TO TREAT

Almost all patients with untreated malignant hypertension die within 1 year. Death is usually due to

uraemia but heart failure and cerebrovascular accidents are common. In these patients, and in those

with moderate to severe hypertension, there is clear evidence that treatment prolongs life.

Any person with mild persistent hypertension should be treated. Life style modification is adopted and

observing patient for 3 months, if BP does not normalize treatment to be started.

Other risk factors should also be taken into account when deciding whether to initiate therapy. Factors

such as age, sex, hypercholesterolaemia, cigarette smoking and diabetes are not simply additive but

multiplicative in terms of the risk to the individual. Patients with multiple risk factors, therefore, are

more likely to benefit from antihypertensive treatment than those with the same level of blood pressure

but no other risk factors.

In deciding when to initiate treatment, two patient groups require specific mention. These are the

elderly (patients over 70 years of age) and those with isolated systolic hypertension (systolic blood

pressure greater than 160 mmHg and diastolic blood pressure less than 95 mmHg). Recent studies have

demonstrated that both elderly patients and those with isolated systolic hypertension derive very

considerable benefit from treatment.

In patients with mild to moderate hypertension, treatment has effectively eliminated death from cardiac

failure and has reduced the incidence of fatal and non-fatal strokes by around 35—40%.

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TREATMENT OF HYPERTENSION

Malignant hypertension, demonstrating retinal haemorrhages and exudates, requires urgent

hospitalization and treatment. In all hypertensive patients, attention should be paid to non-

pharmacological interventions that will reduce blood pressure and obviate the need for drug therapy in

mild hypertensives, particularly if there is no evidence of end-organ damage. These include

• Weight reduction.

• Regular exercise.

• Stop smoking.

• Reduce cholesterol intake and salt intake.

• Management of stress.

Drugs used in the treatment of hypertension

Beta-adrenoceptor blocking drugs

Beta-blockers are effective antihypertensive drugs. They are more effective when combined with a

diuretic or other antihypertensive drugs but are often sufficient on their own and produce no marked

orthostatic effects. These drugs may exacerbate obstructive airway disease and intermittent

claudication, and should probably be avoided in patients with these conditions. Minor side-effects, such

as fatigue and cold extremities, are relatively common and disappear on stopping the drug.

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Thiazide diuretics

Initially, plasma volume and cardiac output may be reduced, but these values later normalize.

Undesirable metabolic effects of thiazides include hypokalaemia, hyperuricaemia,

hypercholesterolaemia and hyperglycaemia. With the currently used low doses of thiazides these

effects are small.

ACE inhibitors

This class includes captopril, enalapril, ramipril, lisinopril and perindopril. These drugs block the

enzyme that converts angiotensin I to angiotensin II. They cause a fall in blood pressure by reducing

systemic vascular resistance, without having any major effect on heart rate and cardiac output. The fall

in systemic vascular resistance is probably mainly due to a reduction in plasma angiotensin II levels,

but there is also a secondary fall in aldosterone concentration.

ACE inhibitors are effective alone in all grades and types of hypertension, but their action is

potentiated by diuretics. A small rise in plasma urea and creatinine values is normal with ACE

inhibitors but a marked increase may indicate unsuspected renal artery stenosis and is an indication for

stopping the drug and considering renal angiography by MSCT. Hyperkalaemia can occur because of

the antialdosterone effects; therefore, concomitant use of ACE inhibitors and potassium-sparing

diuretics is not recommended. Profound hypotension may occasionally be induced on first commencing

treatment but this is usually seen only in patients who are already hypovolaemic as a result of high-

dose diuretic therapy. This can be avoided by omitting diuretics on the day of starting the ACE

inhibitor and also by starting with a small dose.

Cough is a particularly troublesome side-effect, occurring in some 15 % of patients. Other side-effects

include taste disorders, nausea, diarrhoea, rashes, neutropenia and proteinuria. Acute angioneurotic

oedema is a rare but serious side-effect which occurs in 0.1—0.2% of patients and is more common in

black patients.

Summary: Angiotensin Converting enzyme inhibitors (A.C.E.)

Types:

a- Captopril.

b- Benzapril, Perindopril, Enalapril.

c- Lisinopril. Fosinopril.

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Mechanism of actions:

- Inhibition of circulating A.C.E.

- Inhibition of tissue A.C.E.

Advantage:

- No metabolic side effect.

- Improvement in glucose tolerance and insulin resistance.

- Reno-protective.

- Cardiac protection: prevent cardiac dilatation and reversal of L.V.H.

Special indications:

1- H.T with C.H.F.

2- H.T with L.V.H.

3- Following myocardial infarction.

4- H.T. with type II diabetes and diabetic nephropathy.

5- H.T. with peripheral vascular disease.

6- High renin-hypertension.

7- No C.N.S. side effects.

8- No coronary vasoconstriction.

9- Older and younger hypertensives

Cautions:

Renal insufficiency.

Renovascular disease.

Contraindications: * Pregnancy.

* Bilateral renal artery stenosis.

Side effects: Cough, taste disturbance, rash and leucopenia.

Calcium-blocking drugs

The dihydropyridine group, including nifedipine, nicardipine and amlodipine, all act predominantly by

relaxing vascular smooth muscle and hence lowering peripheral vascular resistance. Side-effects with

these agents include headache, flushing and ankle swelling.

The phenylalkylamine calcium channel blockers, such as verapamil and diltiazem, act more on the

myocardium and conducting tissue. These are free from the vasodilator side-effects of the

dihydropyridine class but do have negative inotropic effects and may potentiate heart failure.

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Alpha-blockers

This class includes prazosin, terazosin and doxazosin. These drugs have marked arteriolar and venous

vasodilating effects and the initial dose may produce profound postural hypotension. For this reason,

the first dose should be taken on retiring to bed and the dosage gradually increased over a period of

several weeks.

Angiotensin receptor antagonists

These include Losartan, Valsartan, Irbesartan, Telmisartan and Candesartan. These agents act by

blocking the angiotensin II receptor. They appear to be effective in lowering blood pressure and are

relatively free from side-effects. Unlike the ACE inhibitors, they do not cause cough.

Other vasodilator agents

Drugs such as hydralazine and minoxidil are not now used as first-line therapy but may still be useful

in combination with other agents when multiple drugs are required to control blood pressure. Diazoxide

and nitroprusside are very effective vasodilators but are generally used only in hypertensive

emergencies.

Choice of therapy for the individual patient

All drugs cause side-effects in some people. This is a particular problem in the treatment of

hypertension where patients are usually asymptomatic before the commencement of medication. The

unexpected development of side-effects will often cause the patient to stop taking the medication and it

is generally better to warn patients that side-effects may occur.

As a first choice, many clinicians would use either a diuretic, such as Indapamide (Natrilix sr) 1.5 mg

once daily, or a long-acting beta-blocker, such as atenolol 50—100 mg once daily or bisoprolol 5 mg

once daily. ACE inhibitors are more effective than other agents in producing regression of left

ventricular hypertrophy.

The choice of medication is also determined by coexisting disease and the side-effect profile of a given

agent. In patients with angina, for example, a beta-blocker would be a logical choice to treat both the

angina and hypertension, whereas diuretics and ACE inhibitors would be preferable in patients with

impaired left ventricular function. Beta-blockers should be avoided in patients with asthma or severe

heart failure, and diuretics should be avoided in those patients with gout. ACE inhibitors should be

used with caution in patients with impaired renal function.

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If the response to a single drug is inadequate, a second agent should be added. Particularly useful

combinations include:

• beta-blocker plus diuretic

• beta-blocker plus dihydropyridine calcium antagonist

• ACE inhibitor or angiotensin II antagonist plus diuretic

If a two-drug regimen does not give adequate blood pressure control, a third or fourth agent can be

added If the patient has not already been investigated for secondary hypertension, this should be

considered if the hypertension appears to be resistant to drug treatment It is also important to remember

that non-compliance is common in the treatment of hypertension, and this should be suspected if the

blood pressure fails to fall despite the use of multiple drugs

Drugs to be preferred in specific conditions:

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Hypertensive emergencies

Hypertensive emergencies: situations in which the complication occur over a short period of time

(hours to days) and need immediate reduction of BP generally by I.V. therapy (situations in which there

is an end-organ damage):

1- C.N.S. compromise as in hypertensive encephalopathy, subarachnoid hemorrhage, intracerebral

hemorrhage.

2- Severe H.T. with pulmonary edema (Acute LV failure).

3- Severe H.T associated with acute myocardial infarction or unstable angina.

4- Severe H.T associated with eclampsia.

5- Acute aortic dissection.

6- Pheochromocytoma crisis.

Hypertensive urgencies

Hypertensive urgencies in which the complications occur over a period of days to weeks and require

gradual reduction in BP and not associated with end organ damage:

1- Accelerated & malignant H.T.

2- Perioperative & postoperative H.T.

3- Pre-eclampsia.

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4- Hypertension with angina & unstable angina.

5- Severe H.T. in kidney transplanted patient.

References:

Mancia et al . 2013 ESH/ESC guidelines for the management of arterial hypertension: the Task Force

for the Management of Arterial Hypertension of the European Society of Hypertension (ESH) and of

the European Society of Cardiology (ESC). Eur Heart J. 2013 Jul;34(28):2159-219

AORTIC ANEURYSM AND AORTIC DISSECTION

Aortic aneurysm: An aortic aneurysm is defined as a pathologic dilatation to more than 1.5 times the

normal diameter of the aorta.

Abdominal aortic aneurysms (AAAs)

Are much more common than thoracic aortic aneurysms; up to 75% of aortic aneurysms involve the

abdominal aorta. Risk factors include hypertension, hyperlipidemia, tobacco abuse, diabetes mellitus,

genetics, and age. There is a male-to-female ratio of 9:1, and most cases (95%) involve the infrarenal

aorta.

1. Clinical presentation. The majority of AAAs are discovered incidentally on physical examination

or during radiologic or ultrasound evaluation of the abdomen.

2. Diagnostic testing: a. Abdominal ultrasound. b. Multi Slice CT. c. Aortography.

d. Magnetic resonance imaging.

3. Therapy

a. Medical therapy. - B-Blockers. - Risk factor modification with hypertension and

hypercholesterolemia control is important. - Cigarette smoking should be eliminated.

b. Percutaneous therapy: Percutaneous catheter repair.

c. Surgical therapy.

Thoracic aortic aneurysms (TAAs)

Are much less common than the abdominal variety.

. Diagnostic testing: as above

. Therapy: a. Medical therapy. b. Percutaneous therapy. c. Surgical therapy.

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Aortic dissection

Dissection of the aorta is defined as cleavage of the intima from the media and adventitia.

A. Epidemiology: The incidence of aortic dissection is thought to be 2.000 cases per year in the United

States. The male- to- female ratio is 2:1. The mortality for untreated acute aortic dissection is

approximately 1% per hour within the first 48 hours. Around 65% of dissections originate in the

ascending aorta (Just above the right or noncoronary sinus), 20% in the descending thoracic aorta, 10%

in the aortic arch, and the remainder in the abdominal aorta.

B. Classification schemes:

- Currently in use are three classification schemes based on anatomy. These are the DeBakey, Stanford,

and anatomic classification.

C- Clinical presentation

1. Signs and symptoms

- Severe chest and/ or back pain are the presenting symptoms in 74% to 90% of acute aortic

dissections. This pain is of sudden onset, at its maximal level, which contrasts with the pain of MI,

which is more gradual in onset. The pain is usually described as tearing, ripping, or stabbing. The

location of the most severe aspect can help localize the dissection. Anterior chest discomfort is often

associated with ascending aorta involvement. Intrascapular pain is often associated with DeBakey type

I or III dissections.

- Unequal radial pulse is a very important sign. Severe chest pain with normal ECG should alert the

physician to this possibility.

- Less common presentations include CHF (usually due to severe aortic incompetence in proximal

dissection), syncope (in 4% to 5 of cases, due to rupture into pericardial space with resultant

tamponade), CVA, paraplegia, or cardiac arrest.

2. Physical examination:

a. Cardiac:

1. Hypertension is often seen with aortic dissection frequently as the cause and occasionally as a

complication. In distal dissections, which involve the renal artery, the increase in blood pressure is a

response to renal ischemia.

2. Hypotension can be seen in proximal dissection with aortic root involvement, hemopericardium,

and tamponade.

3. Pseudo-hypotension occurs when the subclavian artery is involved with resultant compression of

the vessel.

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4. The diastolic murmur of AI (16% to 67%) often indicates root involvement, with disturbance of

normal aortic valve coaptation.

b. Neurologic: Findings of cerebrovascular accident occur in 3% to 6% of proximal dissections.

Rarely, a dissection of the descending aorta involves the primary vessel to the spinal cord with resultant

paraplegia.

3. Laboratory examination.

a. Chest radiograph findings are suggestive of dissection.

b. ECG. The most common finding is left ventricular hypertrophy.

D. Etiology and pathology

1. Medial degeneration, as in Marfan and Ehlers-Danlos syndromes.

a. Aging and uncontrolled hypertension.

b. Other associated findings include congenital bicuspid aortic valves.

2. Pregnancy increases the risk of dissection.

3. Direct trauma is also associated with dissection.

E. Diagnostic testing

1- Evaluation: define the following points: ascending versus descending aortic involvement; site of the

intimal tear; presence or absence of AI; presence or absence of pericardial tamponade; and coronary

involvement.

2- Magnetic resonance imaging/magnetic resonance angiography.

3- Transesophageal echocardiography/transthoracic echocardiography.

4- Multi Slice CT. 5- Aortography.

F. Therapy. Death in aortic dissection results from progression of the dissection resulting in either

vascular compromise or rupture. Proximal (type A) aortic dissection is universally felt to mandate

immediate surgical treatment. This greatly reduces the risk of poor outcomes (acute AI, CHF,

tamponade, and neurologic sequelae) from progression of the dissection and halts the 1% per hour

mortality rate. Management of distal (type B) aortic dissections is controversial, but it is generally

believed that medical management is initially indicated. Surgical interventions are reserved for

complication or treatment failure.

Difficult situations:

a- Hypotension and shock: The most likely causes are aortic wall rupture or tamponade.

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b- Acute MI can be seen in association with a type A dissection. In this setting, thrombolysis is

absolutely contraindicated. Lack of flow in the proximal coronary artery or a flap obstructing the

coronary may be visible on TEE.

- Medical therapy: a. β-Blockers should be initiated immediately if aortic dissection is being

considered in the differential diagnosis.

b. Once the patients is adequately β-blocked, sodium nitroprusside can be initiated

- Surgical therapy

- Patients with proximal or type A dissection should be taken to the operating room upon diagnosis.

- Patients with type B dissection in whom pain and/or hypertension cannot be controlled medically, or

who have evidence of rupture or end-organ involvement, signifying progression of the dissection,

should receive surgical repair.

Table . Aortic dissection classification systems

Classification Pathologic description

STANFORD

Type A Any dissection involving the ascending aorta

Type B Any dissections not involving the ascending aorta

DEBAKEY

Type I Entry point in the ascending aorta, extends to the aortic

arch and often beyond

Type II Confined entirely to the ascending aorta

Type III Entry in the descending aorta (distal to left subclavian);

extends distally (usually) or proximally (rarely)

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Fig. Anatomic appearances of three different aortic dissection classifications.

- Percutaneous therapy. New techniques are emerging in the treatment aortic dissections. The newest

of these is the percutaneous intraluminal stent-graft.

- Long-term management:

- Chronic management of distal (type B) aortic dissections is an extension of the acute management.

Aggressive blood pressure control is obtained with oral agents such as atenolol, metoprolol, labetalol,

carvedilol or diltiazem.

- In the event of treatment failure, these patients should always be considered for surgical treatment.

Failure is defined as evidence of aortic leak, progression with visceral organ involvement, recurrent

pain, or AI.

DISEASES OF THE PERIPHERAL ARTERIES AND VEINS

Definition: Peripheral arterial diseases are abnormalities that impair the ability of the circulation to

deliver blood to lower extremities.

Etiology: Atherosclerosis is the most common cause of arterial disease of the lower extremities. Risk

factors for the development and progression of Atherosclerotic disease include smoking, lipid

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abnormalities, diabetes mellitus, hypertension, and homocysteinemia. The arteritides (i.e., giant-cell

arteritis, Takayasu’s disease, Buerger’s disease) can be contributory and due to an inflammatory and /

or autoimmune process. Other important etiologies of compromised blood flow to the lower extremities

are emboli, dissection, trauma, vasoconstrictive medications, and profound systemic shock.

Clinical manifestations

History

Intermittent claudication is the classic symptom of chronic lower extremity arterial insufficiency. This

usually presents as calf fatigue and cramping with exercise that is relieved promptly by rest.

Progression of disease with involvement of multiple levels and inadequate collateral circulation can

result in pain with minimal exertion or at rest. Rest pain presents as a severe burning discomfort in the

foot and may progress to actual tissue necrosis. Acute ischemia produces excruciating pain with

progressive sensory and motor deficits.

PHYSICAL EXAMINATION

The hallmark of peripheral occlusive disease is diminished or absent pulses

Tests performed in evaluation (leg raising tests).

DIAGNOSTIC EVALUATION

Doppler ultrasound can very accurately detect the presence of lower extremity arterial insufficiency.

Multi slice CT is diagnostic. Contrast arteriography remains the “gold standard” to define the presence

and severity of arterial disease and is reserved to define anatomy prior to operative intervention.

TREATMENT: MEDICAL

- Cessation of smoking.

- An exercise program that stresses daily activity.

- Lipid abnormalities should be aggressively treated.

- Diabetes requires both strict control of their blood glucose and preventive foot care.

Pharmacologic intervention with pentoxifylline may benefit some patients with claudication (dose: 400

mg PO tid), and Cilostazol 100 mg (Pletaal). A trial of vasodilators is indicated in patients with

vasospastic disorders.

SURGICAL/ INTERVENTIONAL

Angioplasty and stenting of the common iliac artery has the best durability.

Raynaud’s phenomenon: Pathology: Vasospasm of digital vessels precipitated by cold and relieved

by heat. Clinical features: Underlying causes: Arterial occlusive disease; connective tissue disease;

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neurologic diseases; ingestion of vaso-constricting drugs; nerve compression syndromes;

cryoglobulinemia or cold agglutinins; post-frostbite or trench foot; Raynaud’s disease (females more

than males).

Physical findings: White, cyanotic digit upon exposure to cold or emotional upset; hyperemic upon

resumption of circulation.

Treatment: Limitation of cold exposure. Stop smoking. Vasodilators. Regional sympathectomy.

LOWER EXTREMITY DEEP VEIN THROMBOSIS

DEFINITION: Deep venous thrombosis (DVT) is clotting that develops in the deep veins of the calf,

thigh, or pelvis.

ETIOLOGY: Virchow’s triad of stasis of blood, vessel wall injury, and increased coagulability

concisely describes the primary etiologic factors that precipitate venous thrombosis. Stasis of blood

results from reduced venous return, as occurs in prolonged bed rest, limb paralysis, surgical

procedures, and venous valvular insufficiency. Vessel wall injury may be due to surgical injury,

iatrogenic catheterization, or blunt/ penetrating trauma. Hypercoagulability may be a primary or

secondary state: its causes include protein deficiencies (protein C, protein S, antithrombin III),

malignancy, thrombocytosis, and systemic lupus erythematosus.

CLINICAL MANIFESTATIONS

HISTORY

A majority of cases of DVT are silent and require a high index of suspicion for early diagnosis. The

patient may have only vague, nonspecific complaints. Local symptoms may include pain, swelling, or

tenderness in the involved extremity. Chest pain and shortness of breath is an infrequent but not rare

presentation due to an acute pulmonary embolus (PE) secondary to the DVT.

PHYSICAL EXAMINATION

Localized limb swelling and tenderness can be present. Homans’sign (pain or resistance on passive

dorsiflexion of the ankle) may be present and raises clinical suspicion but, contrary to past dogma, is a

nonspecific finding. The venous occlusion may become severe and, in are cases, may result in a pale

leg with compromised arterial perfusion (phlegmasia alba dolens).

DIFFERENTIAL DIAGNOSIS: DVT must be accurately distinguished from other causes of a

painful, swollen lower extremity. The important disorders in this list are malignancy, musculoskeletal

injury, infections, lymphedema, congestive heart failure, and ruptured Baker’s cyst.

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DIAGNOSTIC EVALUATION: A bedside examination with a simple Doppler ultrasound device can be

highly accurate in the diagnosis of DVT.

TREATMENT: MEDICAL: Outpatient therapy with self – administered subcutaneous injections of

low-molecular-weight heparin has been shown to be safe and effective.

SURGICAL/ INTERVENTIONAL: The need for surgical therapy for DVT is rare. Inferior vena cava

filter insertion is indicated in recurrent or progressive thromboembolic disease despite therapeutic

anticoagulation.

DISORDERS OF THE LUNGS AND PULMONARY CIRCULATION

PULMONARY EMBOLISM

Pulmonary embolism is a major cause of morbidity and mortality. Diagnosis can be difficult and both

under-diagnosis and over-diagnosis are common.

Pathophysiology

Pulmonary embolism, pulmonary thrombosis and pulmonary infarction are related conditions:

Pulmonary embolism results from the obstruction of the pulmonary arterial vessels by thrombus or by

material, such as fat or air, originating in some other site.

• Pulmonary infarction is the necrosis of a wedge of lung tissue resulting from pulmonary arterial

occlusion.

Approximately 90% of pulmonary emboli originate in the leg veins. One or more of three mechanisms

may contribute to their formation:

• Venous stasis. Venous stasis may result from prolonged immobilization or incompetent venous

valves, possibly as a result of previous thromboembolism.

• Blood hypercoagulability. Blood hypercoagulability may be a result of drug therapy, including the

oral contraceptive pill, hormone replacement therapy and steroids. In addition to this, there are a

number of genetic abnormalities that predispose to thrombosis.

• Injury to the vessel wall. This may occur as a result of local injury or vascular endothelial

damage, particularly previous thrombophlebitis.

In practice, several mechanisms may coexist. For example, there is an increased incidence of venous

thromboembolism in pregnancy resulting both from venous stasis and a hypercoagulable state. The

need to screen patients for possible underlying systemic hypercoagulability depends on clinical

circumstances. Screening should be considered in young patients with no other identifiable

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predisposing factors, patients with a family history of thromboembolism and in patients with recurrent

thrombosis.

The substantial majority of pulmonary emboli originate in the veins from the lower extremity.

However, upper extremity venous thrombosis can also provide an embolic source and should be

considered, particularly in patients with central venous catheters, pacing wires or intravenous drug

abusers. In addition, the heart itself should be considered as a source of embolism in patients with atrial

fibrillation, particularly in the presence of right heart failure.

Clinical features

The nature of the clinical presentation with pulmonary embolism depends on the size of the embolus:

• A small embolus may present with non-specific features such as dyspnoea or tiredness.

• A medium-sized embolus may cause the occlusion of a segment of the pulmonary arterial tree,

causing pulmonary infarction. This may result in

• Pleutritic pain, haemoptysis, a low-grade pyrexia and dyspnoea.

• Massive pulmonary embolism results from the occlusion of two-thirds or more of the pulmonary

arterial bed. This causes sudden death, otherwise right-sided failure, a low cardiac output and a rise in

venous pressure.

The physical signs of pulmonary emboli vary with the size of the embolus. Small and even medium-

sized emboli may be devoid of any abnormal clinical signs. Following pulmonary infarction, signs of a

pleural effusion and pleural rub may be present.

Large emboli may cause:

• hypotension and shock

• tachycardia

• cyanosis

• elevation of the jugular venous pulse with a prominent V wave

• accentuation of the pulmonary component of the second heart sound due to pulmonary

hypertension

• right ventricular third and fourth heart sounds

Massive pulmonary embolism should be suspected in any patient who suddenly develops the features

of shock, syncope, acute dyspnoea or chest pain, particularly if the subject has evidence of a venous

thrombosis or has been confined to bed during the preceding days.

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Table: Symptoms and signs in 327 patients with pulmonary emboli

Symptoms Per Cent Signs Per Cent

Chest pain

Pleuritic

Nonpleuritic

88

74

14

Respirations above 16/min 92

Dyspnea 84 Rales (crepitations) 58

Apprehension 59 High pulm. S2 53

Cough 53 Pulse above 100/min 44

Hemoptysis 30 Temperature > 37.8 C 43

Sweats 27 Phlebitis 32

Syncope 13 Gallop (RV S3 or RA S4) 34

Diaphoresis 36

Edema (Pulmonary) 24

Murmur (Tricuspid) 23

Cyanosis (Central) 19

Investigations

• Chest radiography. This may show features of pulmonary atelectasis or pleural effusion which may

accompany pulmonary embolism.

• Electrocardiogram (ECG). ECG changes accompanying pulmonary embolism are unreliable for

diagnostic purposes. In cases of mild to moderate pulmonary embolism, the ECG is generally normal,

except for demonstrating sinus tachycardia. In more severe pulmonary embolism, a number of ECG

features may be observed.

– S1 Q3 T3 pattern. A narrow Q wave and inverted T wave in lead III, accompanied by an S wave in

lead I, all due to changes in the position of the heart caused by dilatation of the right ventricle and

atrium

– P pulmonale. right bundle branch block

– ‘right ventricular strain’ pattern with T inversion in the leads of V1 to V4

– atrial arrhythmias.

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Fig: ECG appearances in pulmonary embolism. Note tall, peaked P waves, partial right bundle branch

block (rSr in V1), S in lead I, Q and negative T in III, and inverted T waves in V1 to V3.

The differential diagnosis from acute myocardial infarction may be extremely difficult. The ECG is of

considerable value, but the patterns associated with massive pulmonary embolism are often

misinterpreted as being those of a combination of inferior and anteroseptal infarction. The appearance

of Q waves and negative T waves in lead III (but not in lead II) in association with inverted T waves

from V1 to V4 strongly suggests pulmonary embolism.

• Blood gases. Characteristically pulmonary embolism causes a reduced arterial pO2 due to

shunting of blood through underventilated parts of the lung. Simultaneously pCO2 is normal or reduced

due to hyperventilation.

However, the sensitivity and specificity of these findings is relatively low.

• D-dimer. Plasma D-dimer is a useful screening test. The test is sensitive but not specific.

However, a negative D-dimer virtually excludes pulmonary embolism and obviates the needs for

additional tests.

• Pulmonary scintigraphy. Using radioactive technetium, this is a sensitive technique for

detecting perfusion abnormalities.

• Multislice (CT) scanning. This involves the injection of contrast medium through a peripheral

vein and enables the right and left pulmonary arteries to be visualized down to their segmental

branches. The test is both sensitive and specific in diagnosing pulmonary emboli and is considered by

many as the investigation of choice in patients unsuitable for a ventilation—perfusion scan or in whom

a ventilation—perfusion scan gives equivocal results.

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• Pulmonary angiography. It is an invasive investigation which carries significant risk,

particularly in this patient population who may already be critically ill. The advent of MSCT has

largely superceded the need for pulmonary angiography.

• Venous ultrasonography.

• Echocardiography: Echocardiography is of value as a means of assessing pressure overload on

the right ventricle which accompanies massive pulmonary embolism.

Management

There are two major objectives in management:

• The prevention of further thromboembolism

• In severe cases, the detection and relief of right heart failure.

Anticoagulation

In the majority of patients the haemodynamic consequences of pulmonary emboli are not severe. The

primary objective of treatment is the prevention of further emboli. Patients are treated initially with

intravenous heparin, adjusted according to the patient’s activated partial thromboplastin time. Warfarin

therapy is commenced and heparin discontinued after 5—7 days. Heparin should be maintained for at

least 2 days after achieving a therapeutic international normalized ratio (INR).

The recent introduction of low molecular weight heparins has substantially replaced the use of

conventional heparin. Low molecular weight heparins carry several advantages.

The target INR therapeutic range for the management of pulmonary embolism is generally 2.0—3.0.

The duration of anticoagulation varies with clinical circumstances. If a predisposing factor can be

identified and this factor no longer exists, treatment for 3 months may be adequate. If no predisposing

factor can be identified, a minimum of 6 months anticoagulation is generally recommended. In patients

with recurrent thromboembolism, lifelong anticoagulation is recommended. Similarly, in patients with

a defined hypercoagulable state, lifelong anticoagulation may be necessary

In exceptional patients who have recurrent pulmonary emboli while on warfarin, an inferior vena caval

filter device can be considered. This device, inserted percutaneously via a catheter, traps clots

preventing migration to the lungs.

Management of massive pulmonary embolism

In patients with massive pulmonary embolism, sufficient to cause severe haemodynamic compromise,

thrombolytic therapy should be considered. Unlike thrombolytic treatment of acute myocardial

infarction, there is no evidence-based consensus as to which patients should be treated. However, there

is general agreement that patients with systemic hypotension should receive thrombolytic therapy,

because the prognosis of this group if left untreated is so poor.

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General management and pain relief

Opiates such as diamorphine are appropriate, but care is needed in hypotensive patients. Hypoxaemia is

likely and high concentrations of oxygen (at least 40%) should be administered. If right-sided pressure

should fall, it may become necessary to give intravenous fluids to maintain cardiac output.

Anticoagulation

The patient should be heparinized to prevent further embolism. Therapy should be initiated with a

bolus of 5000—10 000 units, followed by a maintenance infusion of 1000 units/h, adjusted according

to the activated partial thromboplastin time (APTT). A low molecular weight heparin, such as

enoxaparin 1.5 mg/kg every 24 h given by subcutaneous injection can be used as an alternative.

Thrombolysis

In patients with severe haemodynamic compromise thrombolytic therapy should be given to dissolve

the embolus A loading dose of 250 000 units of streptokinase should be given over 30 mm, followed by

a maintenance infusion of 100 000 units hourly for 23 hours.

Embolectomy: Pulmonary embolectomy is rarely undertaken.

PULMONARY HYPERTENSION

The pulmonary circulation is a low pressure system (16/8 in men, 20/10 in women). Pulmonary

hypertension exists when the systolic pressure exceeds 30 mmHg or mean pressure > 25 mmHg. There

are two types of pulmonary hypertension:

• Pulmonary hypertension is most commonly secondary to other cardiac or lung disease - this is

termed secondary pulmonary hypertension.

• Less commonly, pulmonary hypertension occurs in isolation, unrelated to other heart and lung

problems — this is termed primary pulmonary hypertension.

Causes of secondary pulmonary hypertension

Pulmonary arterial hypertension (greater than 30/15 mmHg) may result from:

• an increase in pulmonary capillary pressure

• an increase in pulmonary blood flow

• an increase in pulmonary vascular resistance.

Elevated pulmonary capillary pressure

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Passive pulmonary hypertension due to a raised pulmonary capillary pressure occurs in all conditions in

which the left atrial pressure rises, such as mitral stenosis and left ventricular failure. The pulmonary

artery pressure rises in proportion to the pulmonary capillary pressure.

Increased pulmonary blood flow

Pulmonary hypertension due to increased flow develops in disorders in which there are left-to-right

shunts. These include:

• ventricular septal defects (VSD)

• persistent ductus arteriosus (PDA)

• atrial septal defect (ASD).

Causes of increased pulmonary vascular resistance

Cor pulmonale

Chronic thromboembolism

Eisenmenger’s syndrome

Collagen vascular diseases

Primary pulmonary hypertension

Mechanism of increased pulmonary vascular resistance:

• Pulmonary vasoconstriction.

Blockage of the pulmonary arteries or arterioles by thrombosis and embolism as in

thromboembolism and schistosomiasis.

• Arterial medial hypertrophy:

Combinations of the three mechanisms are common. In mitral stenosis for example the initial phase of

passive pulmonary hypertension is often complicated by vasoconstriction and by the obliterative

changes of pulmonary embolism. In many cases of ventricular septal defect both high blood flow and

pulmonary vascular disease contribute to pulmonary hypertension. In emphysema, obliteration of the

vascular bed and hypoxia are contributory factors.

Clinical features of pulmonary hypertension

Independent of causation certain clinical features are characteristic of severe pulmonary hypertension.

The symptoms include

• Dyspnoea. Fatigue. Syncope. Haemoptysis. chest pain. symptoms of right-sided cardiac failure.

Summary: Important causes of pulmonary hypertension

- Mitral stenosis

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- Recurrent pulmonary emboli

- Left ventricular failure

- Schistosomiasis

- Parenchymal lung disease

- Congenital heart disease, L to R shunt (Eisenmenger syndrome)

- Primary pulmonary hypertension

Abnormal features on clinical examination may include:

• elevation of the jugular venous pulse with a prominent ‘a’ wave

• features of tricuspid regurgitation

• a forceful right ventricular heave along the left sternal edge

• a right ventricular fourth-heart sound at the lower left sternal edge

• a loud pulmonary component to the second sound, which may be followed by an early diastolic

murmur of pulmonary regurgitation (Graham Steel murmur).

Investigation: The chest radiograph may show enlargement of the proximal pulmonary arteries, right

ventricle and right atrium. The peripheral lung fields appear oligaemic.

• The ECG demonstrates features of right ventricular hypertrophy:

— tall peaked P waves in lead II due to right atrial enlargement

— right axis deviation, a predominant R wave in lead VI

— inverted T waves in leads VI—V3

Management

Both management and prognosis of pulmonary arterial hypertension depend upon its aetiology and on

its severity Passive pulmonary hypertension responds well if the underlying disorder can be corrected

(e.g. mitral stenosis) Pulmonary hypertension due to high pulmonary arterial flow can usually be

reversed by the correction of the underlying congenital abnormality Increased pulmonary arterial

resistance due to vasoconstriction can often be diminished by relieving hypoxia or by the successful

treatment of mitral valve disease.

When pulmonary hypertension is due to severe pulmonary vascular disease as in the Eisenmenger

syndrome, the prognosis is poor and life is usually sustained for only a few years In these cases, cardiac

failure is progressive in spite of treatment and the only hope may lie in heart—lung transplantation

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Primary (‘unexplained’) pulmonary hypertension

Pulmonary hypertension is said to be primary when the aetiology cannot be determined. The pathology

is complex and poorly understood. The diagnosis is made by exclusion in patients found to have the

clinical features of pulmonary hypertension. Lung biopsy can aid diagnosis, but is potentially

hazardous.

The condition, which is rare, is most common in young women. The onset of the disease is insidious

The first symptoms are usually fatigue and exertional dyspnoea Chest pain syncope and right heart

failure may also occur By the time symptoms occur, the disease is generally advanced with severe

pulmonary hypertension Physical findings: A right ventricular lift and accentuation of the pulmonary

component of the second sound may be evident. As the disease progresses, many patients develop

tricuspid regurgitation and right heart failure.

Echocardiography is a particularly important diagnostic tool. The right atrium and right ventricle are

generally dilated and tricuspid regurgitation often present. The right ventricular to right atrial pressure

gradient can be calculated from the velocity of the regurgitant jet. Extensive additional investigations

are then likely to be required to exclude secondary causes of pulmonary hypertension.

Management

A number of therapies should be considered:

• Thrombosis may contribute to the progression of hypertension and anticoagulants are

recommended; oral contraceptives and pregnancy must be avoided.

• Vasodilator therapy may prove of value in some patients. Patients may be given a trial of an oral

calcium channel blocker. Amlodipine is commonly chosen, on account of its relative lack of negative

inotropic effects.

• Phosphodiesterase inhibitors: Sildenafil is a selective inhibitor of cGMP-specific phosphodiesterase

and hence potentiates vasodilatation. It is very valuable in primary pulmonary hypertension.

Pulmonary Heart Disease (Cor Pulmonale)

Definition: Cor pulmonale is right ventricular hypertrophy or dilatation secondary to pulmonary

hypertension resulting from diseases affecting lung vessels or lung parenchyma and not from left heart

lesion.

The prevalence of pulmonary heart disease varies greatly between one geographical area and another.

The commonest cause of cor pulmonale is chronic obstructive pulmonary disease (COPD) and this is

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reflected in its prevalence. There is abundant evidence that heavy cigarette smoking and air pollution

are major factors in the production of COPD.

Causes and types of Cor Pulmonale

I. Acute cor pulmonale:

A) Massive pulmonary embolism.

B) Extensive lung disease: Spontaneous pneumothorax. Massive atelectasis.

II. Subacute cor pulmonale

A) Recurrent showers of minute pulmonary embolization.

B) Lymphangitis carcinomatosis of lung.

C) Collagen vascular disorders (SLE, polyarteritis nodosa).

III. Chronic cor pulmonale

A) Vascular cor pulmonale: (non hypoxic)

Schistosomal cor pulmonale

Primary pulmonary hypertension

(B) Parenchymatous cor pulmonale (Hypoxic)

Obstructive airway disease

Extensive lung fibrosis, Bronchiectasis, Interstitial fibrosis of the lung

Defective movement of chest wall

Schistosomal cor pulmonale

Schistosomal cor pulmonale is a chronic vascular cor pulmonale due to widespread affection of the

small pulmonary arterioles leading to wide spread obliteration, with little or no involvement of lung

parenchyma.

Mechanism of pulmonary hypertension in schistosomal cor pulmonale:

- Obliterative: related to heavy embolization of schistosoma ova to the lungs.

- Vasospastic:

- Increased pulmonary blood flow due to various vascular shunts.

Clinical features:

- History of bilharziasis and signs of bilharzial affection of other organs (e.g.

hepatosplenomegaly).

- Signs of pulmonary hypertension and right ventricular hypertrophy.

- Often there is marked dilatation of the pulmonary artery causing pulmonary artery aneurysm

and even pulmonary regurgitation (Graham Steel murmur).

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Treatment: see pulmonary hypertension.

DISEASES OF THE PERICARDIUM

Pericardial disease, which may be acute or chronic, is usually associated either with a generalized

disorder or with pulmonary disease.

ACUTE PERICARDITIS

Causes of pericarditis

Infective: Viral - Coxsackie B, influenza, measles, mumps, chickenpox, human

immunodeficiency virus. Pyogenic. Fungal. Tuberculous.

Connective tissue disorder: Rheumatic fever. Rheumatoid arthritis. Systemic lupus erythematosus,

Polyarteritis. Scleroderma. Autoimmune

Sarcoid

Acute myocardial infarction

Post-myocardial infarction (Dressler) syndrome

Post-pericardiotomy syndrome

Neoplastic invasion

Metabolic and endocrine: Uraemia. Gout.

Trauma

Clinical features

Chest pain is the commonest symptom of acute pericarditis and is characterized as follows: • Its

distribution simulates that of acute myocardial infarction, being central and sometimes radiating to the

shoulder and upper arm. The pain may be most severe in the xiphisternal or epigastric regions.

• It is often sharp and severe, but may be aching or oppressive.

• Unlike ischaemic cardiac pain, pericardial pain is commonly accentuated by inspiration, by

movement and by lying flat.

The most definitive sign of pericarditis is a pericardial rub, although this is not always present. A to-

and-fro scratchy or grating noise may be heard in systole, mid-diastole and presystole, or in only one of

these phases.

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Investigations: Electrocardiogram (ECG): In the early stages, the ECG usually shows widespread ST

elevation with the ST segment concave upwards.

• Echocardiography is frequently of value in the detection of a pericardial effusion, but the absence of a

pericardial effusion does not exclude the diagnosis of acute pericarditis.

• Raised C-reactive protein (CRP). Autoimmune markers may be abnormal in cases of pericarditis

associated with connective tissue diseases.

Differential diagnosis: Acute pericarditis is most likely to be confused with:

• Acute myocardial infarction.

• Pneumothorax. • Pleurisy.

Etiological diagnosis

- Viral pericarditis should be suspected if there is a history of an upper respiratory infection and

fever preceding the chest pain.

- Tuberculous pericarditis may be difficult to diagnose, because there is often no evidence of

either pulmonary or miliary infection. Usually, however, there is a history of malaise and weight

loss for some weeks prior to the pericarditis. Tuberculosis is unlikely if tuberculin skin tests are

negative. If necessary the diagnosis may be confirmed by pericardial aspiration or biopsy.

- In pericarditis due to staphylococci, streptococci or pneumococci, there is usually infection in

the lungs or elsewhere in the body.

- In rheumatic fever, there is accompanying evidence of the rheumatic process as well as of

myocarditis and endocarditis. In pericarditis due to hypersensitivity or autoimmunity, there is no

preceding respiratory infection but there is often a history of similar episodes in the past.

Acute pericarditis may also occur in patients with acquired immune deficiency syndrome (AIDS).

Treatment

This consists of the symptomatic relief of pain with anti-inflammatory analgesics and the treatment of

the underlying cause when this is possible.

Bacterial pericarditis should be treated with the appropriate antibiotics; surgical removal of pericardial

pus may be necessary.

PERICARDIAL EFFUSION

Pericardial effusion may result from:

Transudation (in cardiac failure), exudation of serous fluid or pus (in pericarditis)

Blood (from trauma or malignant disease).

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It is also a feature of myxoedema. The hydropericardium of cardiac failure causes few, if any,

symptoms, although it may cause compression of the lungs and reduce the vital capacity. Pericardial

effusion due to other causes my produce pain and pericardial tamponade.

Clinical features

Heart sounds are generally soft on auscultation. Pericardial friction rubs are uncommon in chronic

effusions. If the effusion is constricting, there may be associated signs of cardiac tamponade (see

below).

Investigations

The chest radiograph is valuable in diagnosis. When there is a considerable effusion, the cardiac

silhouette is enlarged and rounded, with loss of the normal demarcation between the cardiac chambers.

Similar abnormalities may be seen in some cases of cardiac failure, but the presence of a very large

heart shadow in the absence of pulmonary vascular congestion makes the diagnosis of pericardial

effusion likely.

• Pericardial effusion produces low-voltage ECG complexes which may vary considerably in amplitude

from cycle to cycle (‘electrical alternans’), reflecting changes in the position of the heart within the

pericardial effusion.

• Echocardiography is the most useful diagnostic method.

Treatment: Paracentesis may occasionally be required for diagnostic purposes, e.g. to identify a

causative organism. No specific treatment is required for a pericardial effusion unless there is

tamponade. Otherwise treatment is of the underlying condition.

PERICARDIAL TAMPONADE

Probably the commonest causes are neoplasm and idiopathic or viral pericarditis, but it may develop in

such conditions as uraemia, myocardial infarction, and after a traumatic cardiac catheterization,

perforation by a pacing wire, cardiac surgery and chest injury.

The inability of the ventricles to fill during diastole leads to raised diastolic pressures in right and left

ventricles, an increase in systemic and pulmonary venous pressures, and a fall in cardiac output.

Clinical features

Clinical features include: • Sinus tachycardia

• Elevation of jugular venous pulse. A further rise may occur during inspiration (Kussmaul’s sign).

• Hypotension and shock in severe cases.

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• Pulsus paradoxus — variation in systemic blood pressure in relation to the respiratory cycle. In pulsus

paradoxus, there is a respiratory variation in pulse amplitude, with a decrease on inspiration.

Investigations

Echocardiography is the most important investigation in pericardial tamponade. Echocardiographic

findings include:

• Right and left atrial diastolic collapse

• Right ventricular diastolic collapse

• Inspiratory increase in tricuspid flow

Management of cardiac tamponade: This is an emergency.

Pericardial aspiration - Samples of aspirate should be sent to microbiology, cytology.

PERICARDIAL CONSTRICTION (CONSTRICTIVE PERICARDITIS)

Constriction of the heart by a fibrosed or calcified pericardium is relatively uncommon. In most

patients, no identifiable cause can be found, although in some communities a tuberculous infection is

responsible for the majority of cases. Constriction can also be a late complication of other types of

infection, neoplastic invasion and intrapericardial haemorrhage, including previous cardiac surgery.

Adequate filling of the ventricles during diastole is prevented by thick, fibrous and, often, calcified

pericardium.

Clinical features and diagnosis

The inability of the ventricles to distend during diastole leads to an increase in diastolic pressure and to

a consequent rise in pressure in the left and right atria and in both pulmonary and systemic veins.

Symptoms resemble those of right- sided cardiac failure. The presenting complaint is often that of

abdominal swelling due to ascites, but dyspnoea and ankle swelling are also common. The clinical

features are as follows:

Sinus tachycardia is usually present, but atrial fibrillation develops in the advanced case.

The neck veins are grossly engorged and show two characteristic features; a rapid ‘y’ descent and

an increase in pressure on inspiration.

There is nearly always an early diastolic sound heard best at the lower end of the sternum.

The liver is enlarged and often tender. In contrast with the severity of the ascites, peripheral oedema

is comparatively slight.

Investigations: One of the most characteristic features of pericardial constriction is a shell- like rim of

calcified pericardium, which is particularly well seen in lateral radiographs of the heart. Computed

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tomography (CT) and magnetic resonance imaging (MRI) are of value, demonstrating thickening of the

pericardium in almost all cases.

The ECG is not diagnostic, but usually shows low-voltage QRS complexes associated with flattened or

slightly inverted T waves.

• Raised left ventricular diastolic, left atrial, pulmonary arterial, right ventricular diastolic and right

atrial pressures.

• The right ventricular pressure pulse shows an early diastolic dip followed by a plateau (Fig.).

Fig: Characteristic RV pressure pulse in pericardial

constriction. Note early diastolic dip, followed

by a plateau.

Differential diagnosis

A number of other disorders should be considered in the differential diagnosis:

• Restrictive cardiomyopathy. • Other causes of heart failure

• Pulmonary disease and cor pulmonale

• Tricuspid stenosis and regurgitation

• Superior vena cava obstruction. • Hepatic disease.

Features of constrictive pericarditis and restrictive cardiomyopathy

Constrictive pericarditis Restrictive cardiomyopathy

S 3 gallop Absent May be present

Palpable apical impulse Absent May be present

Pericardial calcification Frequently present May be present

CT / MRI findings Thickened pericardium Normal pericardium

RV and LV pressures Usually equal LV> RV

Rate of LV filling Rapid early diastolic filling Reduced early diastolic filling

CARDIOMYOPATHY AND MYOCARDITIS

The terms myocarditis and cardiomyopathy are reserved for those relatively uncommon types of

myocardial disease which cannot be attributed to coronary atherosclerosis, congenital or valvular heart

disease or hypertension.

MYOCARDITIS

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Causes of myocarditis

Infective: Viruses — Coxsackie B, cytomegalovirus, infectious mononucleosis, human

immunodeficiency virus

Mycoplasma. Bacteria, Spirochetes. Rickettsiae. Fungi, Parasites and protozoa

Radiation. Drugs — Sulphonamides, doxorubicin, lithium, emetine, cyclophosphamide. Heavy metals.

Hypersensitivity states. Insect stings

Clinical features

• Chest pain is common, but usually attributable to associated pericarditis.

• Heart rate: Tachycardia.

• Heart failure: The symptoms and signs of left and right cardiac failure may develop, with dyspnoea,

gallop rhythm, cardiac enlargement and murmurs due to dilatation of the ventricles.

Investigations: Echocardiography and radionuclide imaging. ECG. Myocardial biopsy.

Management: There is no specific treatment. Therapy is primarily supportive, treating the

complications of heart failure and arrhythmias if they occur. Bed rest is advisable, followed by a period

of restricted activity for approximately 6 months.

H IV: Clinically apparent cardiac involvement occurs in about 10% of patients with acquired immune

deficiency syndrome (AIDS).

CARDIOMYOPATHY

The term cardiomyopathy refers to a disease process involving heart muscle. Cardiomyopathies are

divided into primary and secondary:

• Primary cardiomyopathy. Disease confined to heart muscle and not arising from any other identifiable

disease processes.

• Secondary cardiomyopathy. Heart muscle diseases arising as part of a more generalized disorder,

which closely resemble the clinical characteristics of a primary cardiomyopathy.

The commonest cause of heart muscle disease is damage as a result of myocardial infarction. This is

referred to as ischaemic cardiomyopathy, but differs from the true cardiomyopathies in the focal nature

of the myocardial abnormality.

Functional categories

Three types of functional impairment are observed in patients with cardiomyopathy:

Dilated. The ventricles are dilated with impaired function.

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Hypertrophic. The left ventricle is inappropriately thickened, but contractile function is preserved.

Restrictive. Diastolic filling is impaired.

Systemic disorders causing secondary cardiomyopathy

Connective tissue disorders (systemic Lupus erythematosus, scleroderma and Polyarteritis).

Amyloidosis. Sarcoidosis

Neuromuscular diseases (Friedreich’s ataxia, progressive muscular dystrophy and myotonic

dystrophy). Haemochromatosis. Glycogen storage diseases

Dilated cardiomyopathy

Some causes of dilated cardiomyopathy

Infection: Viral myocarditis. Human immunodeficiency virus

Toxins and drugs: Ethanol

Nutritional and related deficiencies: Thiamine deficiency

Pregnancy

Clinical features: Dyspnea, tachycardia, signs of heart failure, mitral and tricuspid incompetence and

gallop.

Treatment: Management is the general management of heart failure.

HYPERTROPHIC CARDIOMYOPATHY

In this condition, there is massive hypertrophy of the ventricles. The hypertrophy arises in the

absence of any obvious cause that is in the absence of underlying aortic stenosis or hypertension. The

ventricular septum is often the site of the most conspicuous hypertrophy, which may obstruct the left

ventricular outflow tract (hypertrophic obstructive cardiomyopathy). An outflow gradient is present in

approximately one-quarter of patients with hypertrophic cardiomyopathy.

Pathogenesis: Disorganization of the muscle bundles (myofibrillar disarray).

Genetics: In about 50% of cases autosomal dominant inheritance is present.

Clinical features: Symptoms are often similar to those that occur in aortic stenosis, including

dyspnoea, angina and syncope. Arrhythmias are common and there is a high risk of sudden death.

Physical signs include:

In Patients with left ventricular outflow obstruction, evidence of an ejection systolic murmur, which

is best heard at the apex or the left sternal border. The murmur is characteristically labile and increases

with a Valsalva maneuver.

Third and fourth heart sounds which are common.

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Investigations

- ECG. The ECG is commonly abnormal. It may show ST/T wave abnormalities or criteria of left

ventricular hypertrophy.

- Echocardiographic features of hypertrophic cardiomyopathy

Non-concentric hypertrophy with asymmetrical hypertrophy of the septum (ASH); and the

obliteration of the ventricular cavity during systole

In cases with a left ventricular gradient, systolic anterior movement of the mitral valve (SAM) and

mid-systolic closure of the aortic valve may be evident.

Doppler echo can be used to measure any intraventricular pressure gradient.

Abnormal diastolic relaxation.

The presence of mitral regurgitation

Differential diagnosis

The murmur of obstructive cardiomyopathy has usually to be differentiated from other types of left

ventricular outflow obstruction, most particularly aortic stenosis:

Factors favoring hypertrophic cardiomyopathy over athlete’s heart include:

unusual and unequal distribution of hypertrophy (e.g. septal hypertrophy)

decreased left ventricular cavity size

left atrial enlargement

abnormal ECG; although ECG abnormalities can also be seen in trained athletes

family history of hypertrophic cardiomyopathy

the persistence of hypertrophy on detraining.

Management

- In patients with refractory symptoms, surgical resection of part of the interventricular septum is

occasionally indicated.

- An alternative approach is septal ablation. An injection of phenol is given directly into the septal

branch of the left anterior descending coronary artery. This results in ‘controlled infarction’ of the

septum thereby reducing outflow obstruction.

- Some patients appear to derive benefit from dual-chamber pacing.

- Patients thought to be at high risk of sudden cardiac death should be considered for an implantable

cardioverter defibrillator (ICD).

Restrictive and infiltrative cardiomyopathies

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Restrictive cardiomyopathy is the least common of the three major functional categories of

cardiomyopathy (dilated, hypertrophic and restrictive). In restrictive cardiomyopathy, the ventricles are

abnormally stiff and impede ventricular filling, with the result that there is abnormal diastolic function.

Systolic function, by contrast, may remain normal.

Cardiac Tumors

Cardiac tumors are rare. The commonest is the myxoma which occurs most frequently in the left

atrium. Because its position may vary with posture, transient or complete obstruction of the mitral

valve may result.

The hemodynamic effects of left atrial myxoma usually resemble those of mitral stenosis. The tumor

may also be responsible for embolic phenomena, and can produce constitutional effects such as fever,

weight loss, anemia, raised plasma viscosity and ESR and abnormal serum protein level.

The diagnosis is made by echocardiography and the treatment is by surgical excision.

Questions and Answers asked in the Exam of the Medical Rounds

- Manifestations of R. heart failure

Congested neck veins

Enlarged tender liver

Lower limb edema

R. ventricular gallop

- Manifestations of L. heart failure

Basal crepitations

L. ventricular gallop

Orthopnea

Paroxysmal nocturnal dyspnea

Neck Veins:

Systolic pulsations in tricuspid

incompetence and atrial fibrillation.

Giant prominent ‘a’ wave in tricuspid

stenosis and RV hypertrophy as in

pulmonary stenosis and pulmonary

hypertension. Canon ‘a’ wave in

complete heart block.

- Clubbing causes

Cardiac causes

Cyanotic heart diseases

Infective endocarditis

Respiratory

Supportive lung disease (bronchiectasis,

lung abscess)

Bronchogenic carcinoma

- Kussmaul sign in

1- constrictive pericarditis

2- restrictive cardiomyopathy

3- severe Rt. side heart failure

4- pericardial effusion with tamponade

- Causes of atrial fibrillation

Mitral valve disease

coronary heart disease

Hypertension

hyperthyroidism

Constrictive pericarditis

ASD

- Complications of atrial fibrillation

Thromboembolic (embolus)

Decreased cardiac performance

- Diagnosis of Infective endocarditis

Blood culture

vegetations by ECHO

- Aortic dissection

Severe lancinating chest pain

Unequal blood pressure in both arms

(due to closure of LT subclavian artery)

- Valvular heart disease

- Mitral stenosis:

Signs: - Snappy S1, mid diastolic

rumbling murmur, diastolic thrill, O.S.

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BUT (bronchial asthma, TB) never cause

clubbing

Blue clubbing: congenital heart disease

Pale clubbing: Infective endocarditis

- Myocardial infarction

Chest pain compressing

Severe radiation to the arm and back

Syncope, sweating

Auscultation S4 or nothing

- Congested non pulsating neck veins:

SVC obstruction

Causes : wide fixed splitting of S2: ASD

- Signs of DVT:

1- one calf larger than the other

2- tender calf muscles

3- Homan's sign: pain in dorsiflexion of

ankle

- Aortic regurgitation

Soft blowing early diastolic murmur

propagated to the apex

- Aortic stenosis

Harsh ejection systolic murmur

Systolic thrill

Absent A2

Sustained apex

Plateau pulse

- Tricuspid regurgitation Pansystolic murmur increases in

inspiration

Systolic pulse in the liver

Systolic neck veins

- Rheumatic fever

John’s criteria major and minor

A-Major criteria

- Myocarditis (L. heart failure- gallop-

arrhythmia)

- Endocarditis (mitral regurgitation –

aortic regurgitation)

- Pericarditis. (How to diagnose

pericarditis by ECHO: by presence of

pericardial effusion)

B-Minor criteria

- Clinical (fever, arthralgia, history of

previous tonsillitis 1-3 wks., presence of

Rh. valve lesion)

- Lab. (Increased CRP, ESR, prolonged

PR)

- 60 year male patient with hypertension

presented with BP 200/100 mmHg,

severe chest pain, unequal pulse:

Most probable diagnosis is dissecting

aortic aneurysm

- Pulsus alternans occurs in heart

failure.

- Paradoxical pulse occurs in pericardial

tamponade, constrictive pericarditis.

MCQs

- Accentuated first heart sound in all

except:

a- Short P-R interval

- Eisenmenger syndrome occurs in all

except:

a- Large VSD

b- Large ASD

c- PDA

d- Fallot`s tetralogy

- Epigastric pulsations in all except:

a- portal hypertension

b- aortic aneurysm

c- congestive heart failure

d- Rt ventricular enlargement

- In AF all is present except:

a. absent a wave

b. S4

c. S1 of variable intensity

d. Systolic neck venous pulsation

e. Irregular irregularity in the pulse

- In pulmonary hypertension all is present

except:

a. systolic thrill on the pulmonary area

b. S2 close (narrow) splitting

c. Diastolic shock

d. Ejection systolic murmur

- AF is caused by all except:

a. Ischemic heart disease

b. Thyrotoxicosis

c. Mitral valve disease

d. Cor pulmonale

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b- Increase cardiac output

c- Mitral stenosis

Myocarditis -d

- Edema in congestive heart failure occur

in all except:

a- Peri-orbital

b- Ankle

c- Sacrum

d- External Genitalia