Cardiovascular + Clinical Scenarios

8/11/2019 Cardiovascular + Clinical Scenarios

1/36

1

BLOOD VESSELSDr. Dexter MD FRC Path

Undercover Professor

DEPARTMENT OF PATHOLOGYSGUSOM GRENADA (W.I.)

Anatomy & Histology

Three layers ofarteries

Intima endothelial cells

Media smoothmuscle cells

Adventitia connectivetissue

3

ENDOTHELIAL CELL FUNCTIONS

Maintenance of Permeability Barrier Elaboration of Antithrombotic & Prothrombotic

Molecules Modulation of Blood Flow and Vascular

Reactivity Endothelin/NO

Regulation of Inflammation and Immunity IL-1, IL-6

Regulation of Cell Growth PDGF/TGF-

Oxidation of Low-Density Lipoprotein

Endothelial Dysfunction Endothelial stimulation rapid, re-

versible; independent of new proteinsynthesis.

Endothelial activation - alteration in

gene expression and protein synth

5

Characteristics of an activated endothelial cell

A normal arterial wall has anticoagulant properties and low leukocyte adhesivity

Complement products

Hypoxia

MHC moleculesViruses

Coagulation proteAdvanced glycosylationend products

Vasoactive mediaLipid products

Growth factorsHemodynamic forces

Cytokines / chemoBacterial productsAdhesion moleculCytokines

Induced genActivators

Endothelial activation


2/36

7

Vascular Smooth Muscle Cells

Functions

Vasoconstriction and dilation in

response to normal or pharmacological

stimuli

Elaboration of growth factors andcytokines

Migration to the intima and proliferation

Diseases of Blood Vessel

Two principal mechanisms

1. Narrowing or complete obstructiothe lumen either

progressively (atherosclerosis) or

suddenly (thrombosis)

2. Weakening of the wall of the vessleading to dilatation or rupture.

9

Other Categories

Hypertension

Inflammatory disorders -vasculitis

Congenital Malformations

Neoplasms

ARTERIOSCLEROSIS

ATHEROSCLEROSISLARGE BV

INTIMA

MEDIAL CALCIFICSCLEROSIS

MEDIA

ARTERIOLOSMALL

FULL TH

HYPER

HYALINE

11

Atherosclerosis

Derived from Greek word ATHEROS soft gruel or porridge like

Chronic inflammatory disorder of

intima of large arteries characterizedby formation of fibro fatty plaques

called atheroma.

EPIDEMIOLOGY

Five Leading Causes of Death for Males and Females U.S


3/36

13

Atherosclerosis Major RiskFactors

NONMODIFIABLE

Age

Gender

Geneticpredisposition

MODIFIABLE

Hyperlipidemia

Hypertension

Cigarettesmoking

Diabetes mellitus

Atherosclerosis Uncertain, Unquantifiable or Possibly L

Risk Factors

Hyperhomocystinemia

Lipoprotein (a)

Inhibitors of fibrinolysis

Low PA-1 Inhibitor

C-reactive protein

Lack of exercis

Type A perso Obesity Trans-unsat

intake

Postmenopaus

estrogen defic High carbohyd Chlamydia pne

15

NON-MODIFIABLE RISK FACTORS

AGE - begins in childhood and progresses withage

40 -60 yrs five fold increase in incidence of MI

SEX - Males > females Uncommon in premenopausal women

Postmenopausal Increased incidence

Favorable response to HRT

GENETICS

Familial clustering of other risk factors

Familial hypercholesterolemia

MODIFIABLE RISK FACTO

HYPERLIPIDEMIA

cholesterol most important

Major component associated with riskLDL bad cholesterol.

inverse relationship between HDL levelgood cholesterol.

17

Evidences linking cholesterol with

Atherosclerosis

Genetic defects in lipoprotein metabolism Familial hypercholesteremia defect in LDL

receptor hepatic uptake of LDL levelsof LDL

LDL because of abnormal apo E it fails tobind to LDL receptor

Genetic or acquired diseases like DM &

hypothyroidism premature & severe

A.S.

Evidences linking cholesterol w

Atherosclerosis

Atheromas contain cholesterol & cholestero

levels of cholesterol & LDL severity o

High cholesterol diets produce experimenta

levels of cholesterol by diet/drugs progof A.S.


4/36

19

RISK FACTORS

HYPERTENSION

More importance after age of 45

169/95- 5 fold risk of developing IHD ascompared to 140/90

Anti hypertensive therapy reduces

incidence of A.S. associated diseases

stroke, IHD

RISK FACTORS

CIGARETTE SMOKING

Smoking & A.S. Made For Each Other

1/> packs/day for several years death raincreases up to 200%

DIABETES MELLITUS

Cholesterol predisposition to AS

100x increased risk of AS induced gangrof the lower extremities.

risk of strokes and M.I. AS in young patientsSuspect DM

21

RISK FACTORSPLASMA HOMOCYSTEINE

Hyper-Homocystinuria -Patients haveremature vascularisease

Can be caused byow folate & vitaminB intake

RISK FACTORS

LIPOPROTEIN (a)

Altered form of LDL

Potentially atherogenic effects

Lipid accumulation

Endothelial cell modulation

Smooth muscle cell proliferation

Control of neo-vascularization

23

LESSER FACTORS

Stress

Type A personality

Obesity

MULTIPLE RISK FACTORS MAYHAVE A MULTIPLICATIVE & NOT

ADDITIVE EFFECT

LOWER THE BETTER

Blood sugar

Blood pressure

Body weight

LDL

Stress levels

No. of cigarettes


5/36

25

PATHOGENESIS

RESPONSE TO INJURY HYPOTHESIS

Chronic inflammatory response of thearterial wall initiated by some form of the

injury to the endothelium.

RESPONSE TO INJURY HYPOTHE

Role of endothelial cells endothelia

Endothelial activation

Increased permeability

Increased adhesion of leukocytes

Increased expression of adhesion molecules

Growth factors

Determinants of endothelial alterations

Hemodynamic factors increased frequenc

lesions at branch points, ostia of vessels

Hypercholesterolemia

27

RESPONSE TO INJURY HYPOTHE

Role of lipids

Cytotoxic to endothelium

Oxidized (modified) lipids have atherogproperties

readily ingested by macrophages foam ce

chemotactic for circulating monocytes

inhibits the motility of macrophages alreadylesion

stimulates release of growth factors and cy

29

RESPONSE TO INJURY HYPOTHESIS

Role of macrophages Cytokines like IL-1, TN(procoagulant

endothelium)F

Growth factors

Role of smooth muscle proliferation

Conversion of fatty streak into fibrofattyatheroma

Role of T-cells

Recruitment and activation of monocytes


6/36

31

Atherosclerotic plaque

Three principal components

Cells smooth muscle cells,

macrophages, and other leukocytes

Extracellular matrix including collelastic fibers, and proteoglycans

Intracellular and extracellular lipiddeposits

33

Complicated lesion ofatherosclerosis

Calcification may be patchy or massive.

Focal rupture or gross ulceration, or both,leading to

thrombus formation

cholesterol emboli or atheroemboli

Hemorrhage .

Superimposed thrombus

Aneurysmal dilatation atrophy of underlying

media

Sites of severeatherosclerosis in

order of frequency

35

Atherosclerosis-ClinicalManifestations

Coronary heart disease(CHD)

Acute myocardialinfarction (AMI)

Angina Stable orUnstable

Chronic ischemic heartdisease leading tocongestive heartfailure(CHF)

Sudden cardiac death

Abdominal aorticaneurysm (AAA)

Cerebral vasculardisease

Stroke

Transient ischemicattack (TIA)

Chronic ischemicencephalopathy

Peripheral vasculardisease

Claudication

Ischemic bowel disease(mesenteric occlusion)

GangrenePRECPHAS


7/36

37

Hypertension

Hypertension

Normal < 130 mm Hg systolic & < 85 mm diastolic)

Hypertension - Sustained increase in bloodpressure

Systolic > 140 mm Hg

Diastolic > 90 mm Hg

Mild + 20; Moderate +40; Severe +80 mm (systolic)

Malignant hypertension - > 210/120

39

Hypertension

25% of persons in general population are

hypertensive

Leading risk factor MI, DM, Stroke

Silent Killer painless complications

Complications bring to diagnosis but late

Chronic, end organ & vascular damage

Regulation of BP:

BP = Cardiac Output x Peripheral Resista

Endocrine Factors

Renin, Angiotensin, ANP, ADH, Aldosterone.

Neural Factors

Sympathetic & Parasympathetic

Blood Volume

Sodium, Mineralocorticoids, ANP

Cardiac Factors

Heart rate & Contractility.

41

Etiologic Classification:

Primary/Essential Hypertension (95%)Secondary Hypertension (5-10%)

Renal GN, RAS, Renin tumors

Endocrine Cushing, OCP, ThyrotoxicosisMyxdema, Pheochromocytoma, Acromegaly.

Vascular Coarctation of Aorta, PAN, Aorticinsufficiency.

Neurogenic Psychogenic, Intracranialpressure, polyneuritis etc.

Pathogenesis

Essential - multifactorial. Increased peripheral resistance (sympatheti stress, hormonal, neural.

Genetic, familial, life style.

Secondary - Known abnormal control. Increased blood volume - Sodium retention A

Aldosterone.

Increased sympathetic tone - Adrenal tumorsympathetic stimulation.

Increased vasoactive hormones - Cushing'sPheochromocytoma,


8/36

43

GENETIC FACTORSINCREASED

SYMPATHETICACTIVITY

INCREASEDCOP

INCREASED PERIPHERALRESISTANCE

HYPERTENSION

INCREASED RENALSODIUM RETENTION

INCREASEDBLOOD VOLUME

INCREASED RENINALDOSTERONE ACTIVITY

AUTOREGULATION ARTERIOLAR NARROWING

CESS DIETARY SODIUMRONIC RENAL DISEASE

CUSHING SYNDROMEHYPERALDOSTERONISM

PHEOCHROMOCYTOMA

Malignant Hypertension

Greater than 210/120 BP

May complicate any type of HTN.

Necrotizing arteriolitis.

Rapidly progressive end organ dama

Renal failure

Left ventricular failure ypertensive encephalopathy

45

Hypertension - complications: Large Blood Vessels

Atherosclerosis and its complications.

Small Blood Vessels

Arteriolosclerosis Hyaline & Hyperplastic

Heart

Left ventricular hypertrophy, Hypertensivecardiomyopathy IHD, MI.

Kidney

Nephrosclerosis Benign & Malignant.

Eyes:

Hypertensive retinopathy, hemor rhage

Brain: Haemorrhage, infarction (stroke),

Hyaline arteriosclerosis

Homogenous, pink, hyaline thickening of twalls of the arterioles with loss of underlyistructural details and with narrowing of thelumen.

Leakage of plasma components across vaendothelium and increasing extracellular mproduction by smooth muscle cells

Chronic hemodynamic stress in hypertensmetabolic stress in diabetes accentuates edothelial injury

47

Hyperplastic arteriosclerosis

Related to more acute or severeelevations of blood pressure

Onion-skin, concentric, laminatedthickening of the walls of arterioles withprogressive narrowing of the lumens.

Necrotizing Arteriolitis - deposits offibrinoid and acute necrosis of the vessel

wall.

VASCULITIS

Inflammation and necrosis of the bloovessels, including arteries, veins, andcapillaries.

can be classified based on

Type of vessel involved

Etiology


9/36

49

Type of vessel involvedEtiology

Direct infection- Bacterial, Rickettsial, Spirochetal, Fungal

Immunological

Immune complex mediated

(hepatitis B or C virus mediated)

SLE and rheumatoid arthritis

Drug induced

Antineutrophil cytoplasmic antibody (ANCA) mediated

Wegeners granulomatosis

Churg-Strauss syndrome

Direct antibody attack mediated

Goodpasture syndrome (anti-GBM antibodies)

Kawasaki disease (antiendothelial antibodies)

Cell mediated

Allograft organ rejection Unknown

Giant cell (temporal) arteritis

Takayasu arteritis

Polyarteritis nodosa

51

Anti- Neutrophil Cytoplasmic Antibodies

(ANCA)

Heterogeneous group of auto-antibodiesagainst enzymes mainly found within the

primary granules in neutrophils and inlysosomes of monocytes and in endothelialcells

P- ANCA: microscopic polyangiitis &

Churg-Strauss C-ANCA: Wegners granulomatosis

c-ANCAc-ANCA Proteinase-3

p-ANCA - Myeloperox

IMMUNOFLUORESCENT STAINING

53

Giant Cell (Temporal) Arteritis

Most common vasculitis

Affects mainly the arteries in the head

Temporal

Vertebral

Ophthalmic can lead to blindness

Pathogenesis unknown

T-cell mediated immunologic reactionagainst elastin?

Clinical features

Age: 50 and above

Gender: M:F: 1-1

Symptoms, Vague: fever, weight lossfatigue, facial pain, headache

Ocular symptoms: diplopia, progress

hazy vision, loss of vision


10/36

55

Giant cell arteritis

Diagnosis: Temporal Artery Biopsy

2-3 cm segment

multiple sections

elastic trichrome stain

Treatment: Steroids, Analgesics

Prognosis: Good

Elevated ESR (sed-rate)

Association with polymyalgia rheumatica

Takayasu arteritis

Granulomatous vasculitis ofmedium and large arterieswith obliteration of lumen

Most commonly affects archof aorta with narrowing orvirtual obliteration of theorigins of great vesselsarising in the arch

Can also involve pulmonary,

coronary, and renal arteries. Etiopathogenesis

Unknown

57

Clinical features

Most common in females < 40 years

Ocular changes: visual disturbances, retinalhemorrhages, blindness

Progressive diminution of upper limb pulses withcoldness or numbness of fingers - PulselessDisease

Low BP in upper limb

Neurologic defects dizziness, focal weakness

or complete hemiparesis

Polyarteritis Nodosa (PAN

Systemic vasculitis

Transmural necrotizing inflammation

small or medium sized muscular arte

Typically involves renal and visceral

arteries and spares the pulmonarycirculation.

Arterioles, capillaries, and venules ar

affected

Associated with Hepatitis B antigen (

59

re

Remember

No glomerulonephritis

Pathguy..com

Disease of

oung adultsCourse may becute,bacute, or

ronic and isequentlymittent.0% of

atients haveBV antigen inerum

Clinical features Morphology

Gross - Distribution of lesions in descending ordfrequency Kidney, Heart, Liver, GIT

Lesions have a predilection for branching points abifurcations

Histologically Acute stage - Transmural inflammation, fibrinoid necros

inner half of the vessel wall.

Later stages - inflammation is replaced by fibrous thickeof the vessel wall. Risks of thrombus or infarction.

All stages of activity may coexist in different vor even within the same vessel characteristifeature


11/36

61

Complications - PAN

Weakening of arterial wall owing to

inflammatory process may causeaneurysmal dilatation or localized rupture.

Impairment of perfusion causingulcerations, infarcts, ischemic atrophy, or

hemorrhages in the areas supplied by

these vessels.

Kawasaki syndrome

Arteritis involving the large, medium,small arteries (often coronary arteries

Associated with mucocutaneous lymnode syndrome

Usually in young children and infantscases < 4 years)

Epidemic in Japan Leading cause of acquired heart dise

in children in US(can lead to MI)

63

Kawasaki disease

Pathogenesis Unknown

Autoantibodies against endothelium, smoothmuscle cells, leading to acute vasculitis

Clinical features Fever

Conjunctival and oral erythema

Edema of the hands and feet

Skin rash often with desquamation

Enlargement of cervical lymph nodes

Buerger disease(Thromboangitis obliterans

Characterized by segmental, thrombosing, and chronic inflammation of medium sized asmall arteries, characterized by microabces

Mainly the tibial and radial arteries and somsecondarily extending to veins and nerves oextremities(painful)

Predisposing factors

Cigarette smoking

Hypersensitivity reaction to tobacco direct end

injury Genetic influence

65

Wegener Granulomatosis Classic triad

1. Acute necrotizing granulomas of upper and lower respiratorytract

2. Focal necrotizing or granulomatous vasculitis affecting small tomedium sized vessels, most prominent in lungs, and upperairways but affecting other sites as well

3. Renal disease in the form of focal or necrotizing, oftencrescentic glomerulitis

Limited WG No renal involvement

Males > females

Average age of onset = 40 years

95% of the patients have c-ANCA

Clinical features

Persistent pneumonitis with bilateralnodular and cavitary infiltrates

Chronic sinusitis

Mucosal ulceration of the nasophary

Evidence of renal disease

Untreated patients die within 1 year.


12/36

67

Morphology

Gross ulcerativelesions of the nose,pharynx, palate

Lungs - dispersedfocal necrotizinggranulomascoalesce to

produce nodulesthat may undergocavitations.

Aneurysms

Localized abnormal dilatation of a blo

vessel caused by a congenital oracquired weakness in the media.

Aneurysms can be classified based o

Composition of wall

Gross morphology

Etiology

69

Composition of the aneurysmal sac

True

Complete but attenuated vessel wall. The bloodremains within the confines of the circulatorysystem.

Atherosclerotic, syphilitic, and congenitalvascular aneurysm

False (pseudoaneurysm)

is an extravascular hematoma that

communicates with the intravascular space.

71

Etiology

Atherosclerosis

Syphilis

Mycotic infective.

Vasculitis (PAN, Kawasakis disease)

Congenital defect in media Marfanssyndrome, Berry aneurysm

Iatrogenic Arteriovenous aneurysms for

chronic renal failure patients for dialysis

Atherosclerotic aneurysm

Most common site abdominal aorta

Abdominal aortic aneurysm - diamete

increased at least 50%.

Most frequent aneurysms, usually

developing after the age of 50 years.

6% after the age of 80 years


13/36

73

Clinical course Many aneurysms are asymptomatic.

Abdominal mass

Occlusion of a branch vessel- renal, mesentericvertebral vessels.

Embolism from atheroma or mural thrombus.

Impingement on an adjacent structure

compression of ureter or erosion of vertebrae.

Rupture with massive or fatal hemorrhage.

The risk of rupture - about 2% for a smallabdominal aortic aneurysm (50%.

75

Syphilitic aneurysms

Seen in the tertiary stage of the syphilis.

Due to obliterative endarteritis of the vasa

vasorum of the aorta - Destruction of the

tunica media

Leading to narrowing of the lumen of thesevessels which causes ischemic injury of

the aortic media followed by inflammationand scarring.

Clinical course

Encroachment on the mediastinal structures byenlarged heart (Cor bovinum)

Respiratory difficulties.

Difficulty in swallowing.

Persistent cough due to pressure on recurrent laryngea

Pain caused by erosion of the ribs or vertebrae.

Valvular incompetence (aortic regurgitation) leadleft ventricular hypertrophy, cardiac ischemia dueobstruction to coronary ostia.

Rupture of the aneurysm.

Most common cause of death - heart failure d

aortic regurgitation.

77

Aortic dissection (dissectinghematoma)

Entry of blood in between and along thelaminar planes of media and its extensionalong the length of the vessel.

Often ruptures causing massivehemorrhage

Not usually associated with marked

dilatation of aorta

Etiopathogenesis

Most commonly men 40-60 years of age, whom hypertension is almost always pres(>90% of cases of dissection).

Younger patients with abnormality of conntissue (Marfans syndrome).

As a complication of arterial cannulation (e.g. during diagnostic

catheterization or cardiopulmonary bypass)

During pregnancy - unknown reason. Can result from cystic medial necrosis (los

elastic tissue)


14/36

79

Morphology

Intimal tear - starting point

Occurs most commonly in the ascending aorta,1 or 2 cm above the aortic ring.

The dissection separates the inner two thirds ofthe aorta from the outer third.

The dissection can extend proximally toward theheart as well as distally along the aorta to

variable distances. Re-rupture into the lumen of the aorta - double-

barreled aorta.

Double-barreled aorta.

81

2 Types

Type A - the morecommon (and dangerous)proximal lesions,involving either theascending portion only orboth ascending anddescending aorta.

Type B distal lesionsnot involving theascending part andusually beginning distal tosubclavian artery

Type A Type B

Clinical features

Sudden onset of excruciating (tearing

pain, usually beginning in the anteriochest, radiating to the back, and mov

downwards as the dissection progres

Loss of one or more arterial pulses is

common

83

Complications

Most common cause of death - rupture of thedissection into any of the three major bodycavities (i.e., pericardial. Pleural, and peritoneal)

Retrograde dissection into the aortic root leadingto disruption of aortic valvular apparatus.

Compression of spinal arteries - Transversemyelitis.

Cardiac tamponade, aortic insufficiency, andmyocardial infarction can also occur.

Mycotic (infectious) aneurys

result from the weakening of the vesswall by a microbial infection.

Common sites of involvement include

aorta, cerebral vessels, and mesenterenal, and splenic arteries.


15/36


16/36

91

Blood supply

Functionally

- Right & left coronary arteries are end arteries

- Numerous interconnections collateral

circulation contains little blood in normal hearts

- Collaterals open up when one artery is severely

narrowed

Most blood flow to the myocardium is duringdiastole

Heart failure(congestive heart failure)

Is the inability of the heart, working a

normal or elevated filling pressure, to

pump enough blood to meet the meta

demands of the body.

93

Etiology Systolic dysfunction inability to contract properly

MI

Hypertension

Volume overload valvular regurgitation

Cardiomyopathy

Diastolic dysfunction - Inability of the heart chamberto relax/expand and fill sufficiently during diastole

Massive ventricular hypertrophy

Amyloidosis

Constrictive pericarditis

CHF can also be categorize

Left-sided failure left ventricle is the failin

chamber

Systemic hypertension

Mitral or aortic valve disease

Ischemic heart disease

Cardiomyopathy

Right-sided failure

Left heart failure

Intrinsic disease of lung parenchyma/vasculat

COPD, Pulmonary hypertension

95

Compensatory Mechanisms

- Adrenergic stimulation by endogenouscatecholamines

- Myocardial hypertrophy

- Concentric hypertrophy - hypertension

- Eccentric hypertrophy valvular regurgitation

- Myocardial dilation - Frank Starlings law increased force of contraction with dilation

Compensated heart failure dilatedventricle is able to maintain cardiac oto meet the needs of the body

Decompensated heart failure failingmyocardium is no longer able to prop

sufficient blood to meet the needs of body, even at rest.


17/36

97

HEARTFAILURE

DECREASEDDECREASEDDECREASEDDECREASED

CARDIAC OUTPUTCARDIAC OUTPUTCARDIAC OUTPUTCARDIAC OUTPUTRENALRENALRENALRENAL

OPERFUSIONOPERFUSIONOPERFUSIONOPERFUSION

SODIUM &SODIUM &SODIUM &SODIUM &

ER RETENTIONER RETENTIONER RETENTIONER RETENTION

RENINRENINRENINRENIN----ANGIOTENSIN SYSTEMANGIOTENSIN SYSTEMANGIOTENSIN SYSTEMANGIOTENSIN SYSTEM

EDEMA

PASSIVE CONGESTIONPASSIVE CONGESTIONPASSIVE CONGESTIONPASSIVE CONGESTION

OF PULMONARY CIRCULATIONOF PULMONARY CIRCULATIONOF PULMONARY CIRCULATIONOF PULMONARY CIRCULATION

PULMONARY ARTERIALPULMONARY ARTERIALPULMONARY ARTERIALPULMONARY ARTERIAL

HYPERTENSIONHYPERTENSIONHYPERTENSIONHYPERTENSION

RIGHT HEART FAILURE

SEVERE &SEVERE &SEVERE &SEVERE &SUSTAINEDSUSTAINEDSUSTAINEDSUSTAINED

SYSTEMIC VENOUSCONGESTION

PULMONARY EDEMAPULMONARY EDEMAPULMONARY EDEMAPULMONARY EDEMA

FORWARD FAILUREFORWARD FAILUREFORWARD FAILUREFORWARD FAILURE BACKWARD FAILUREBACKWARD FAILUREBACKWARD FAILUREBACKWARD FAILURE

Pathological consequences

of congestive heart failure

99

Clinical features

Left ventricular failure

Dyspnea

Orthopnea

Paroxysmal nocturnal dyspnea

Right ventricular failure

Systemic venous congestion

Distended neck veins

Enlarged tender liver

Soft tissue edema

Types of heart disease

Five categories of disease account

nearly all cardiac mortality

1. Ischemic heart disease (IHD)

2. Hypertensive heart disease (systemicpulmonary)

3. Valvular heart disease

4. Non-ischemic (primary) myocardial dis

5. Congenital heart disease

101

ISCHEMIC HEART DISEASE (IHD)

Definition

Generic term for imbalance

between myocardial need for and

supply of oxygenated blood

Epidemiology

Leading cause of death for both menwomen in the US and other industria

nations

Each year nearly half a million Ameridie of IHD.

May affect any age, but most commo

older individuals

Males > females until the ninth decad


18/36

103

IHD - syndromes

1. Various forms of angina pectoris (chest pain)

2. Acute myocardial infarction (MI)

3. Sudden cardiac death

4. Chronic ischemic heart disease with congestiveheart failure

Acute coronary syndromes

MI Unstable angina


ETIOLOGY

Atherosclerosis 90% of cases

Other causes of ischemia

Anemia - hypoxemia

Lowered systemic blood pressure - Sho

Increased cardiac demand hypertrophexercise

Vasculitis (Kawasaki, PAN)

Aortic dissection

105

ETIOPATHOGENESIS

Complex dynamic interaction between

the following factors

1. Fixed coronary obstruction

2. Acute plaque changes

3. Coronary intraluminal thrombosis

4. vasoconstriction

Role of Acute Plaque Chan

Disruption of plaque an inability of

plaque to with stand imposed mechastresses

Most often the initiating event

Changes in plaque morphology inclu

Hemorrhage

Rupture or fissuring

Erosion or ulceration

107

Vulnerable or Unstable Plaque

Characterized by

Moderately stenoticplaque (50-75%)

Thinner fibrous cap

A core rich in lipid,macrophages and T-cells

less evidence ofsmooth muscleproliferation

Markedly eccentric(not uniform aroundthe vesselcircumference)

METALLOPROTEINASES

T-cells activate

Role of Coronary Artery Thromb

Plaque rupture, erosion or ulceration exof thrombogenic lipid and subendothelialcollagen

Platelet aggregation, thrombin generation thrombus formation.

If the vessel is completely occluded MI

Incomplete obstruction Unstable angina or arrhythmias sudden card

death

Embolization to distal branches - micro infarct


19/36

109

Role of vasoconstriction

Increases mechanical forces that can contrib-ute to plaque rupture

Stimulated by

Locally released platelet contents Thromboxane A2

Impaired secretion of EDRF Nitric oxide (NO)relative to contracting factors (Endothelin)

Increased adrenergic activity

Smoking

Four syndromes

Angina pectoris (AP)

Myocardial infarction (MI)

Chronic ischemic heart disease

Sudden cardiac death (SCD)

Consequences

111

ANGINA PECTORIS

Intermittent chest pain or discomdue to transient, reversible myocdial ischemia, not quite infarction

No elevation in cardiac enzymes

Three types Typical (stable) angina pectoris

Prinzmetal (variant) angina

Unstable angina pectoris

113

Responds to vasodilatorsResponds to vasodilatorsieved by rest, vasodilators

Harbinger of subsequent MI pre-infarction angina

n - crushing or squeezingbsternal, may radiate down

left arm

Induced by disruption of

plaque with superimposed

thrombosis and possibly

vasospasm

Cause and mechanism not

clear

May be due to coronary artery

spasm

e to critical stenosis -

uction of coronary

fusion due to fixed

nosis

Associated with ST segment

elevation, indicative of

transmural ischemia

sociated with increased

mand -physical activity,

otional excitement

Pain that occurs with

progressively increasing

frequency and is precipitated

with progressively less effort,often occurs at rest, and

tends to be of more prolonged

duration.

Occurs at rest, awakens the

patient from sleep

st common form

UNSTABLE (CRESCENDO)PRINZMETAL VARIANT

ANGINA

(~50-75% occlusion)ANGINA

ABLE (TYPICAL) ANGINA

(~75% occlusion) MYOCARDIAL INFARCTIO(HEART ATTACK)

Indicates the development of an area ofmyocardial necrosis caused by local ische

About 1.5 million individuals in the US sufacute MI annually, and approximately oneof them die.

At least half of them die before they reachhospital.

Decreasing incidence since the early 1970


20/36

115

Age Occurs at any age

Incidence increases with increasingage

Gender Males 4 5 : 1 ages 45 54

Males 2 : 1 up to age 80

No difference > age 80

Epidemiology MAJOR CONTRIBUTING FACTO

Hypercholesterolemia

Smoking

Hypertension

Diabetes mellitus

CRP: Good marker for risk of MI

117

Pathogenesis

Coronary artery occlusion 90% of cases Disruption of plaque 2/3rd are < 50% stenotic & 85%

have < 70% stenosis.

Remaining 10% of cases Vasospasm isolated, intense and relatively

prolonged with or without coronary atherosclerosis

Emboli from the left sided mural thrombus

Unexplained ? Disease of small intramural coronary vessels

? Hematological abnormalities like hemoglobinopathies

Myocardial response

Coronary arteryobstruction loss ofcritical blood supply tothe myocardium

Induces profoundfunctional,biochemical, andmorphologicalconsequences > 1 hrMicrovascular

injury

20-40 Irreversible cellinjury

ATP reduced

- To 50% of normal

- To 10% of normal

< 2 miLoss ofcontractility

SeconOnset of ATPdepletion

TimeFeature

119

CCCOOORRROOONNNAAARRRYYY CCCIIIRRRCCCUUULLLAAATTTIIIOOONNN AAANNNDDD TTTHHHEEE LLLOOOCCCAAATTTIIIOOONNN OOOFFF IIINNNFFFSSS AAARRRCCCTTT

Progression ofmyocardial

necrosis after coronaryartery occlusion

Begins insubendocardial region


21/36

121

Morphology

The essential sequence of events in MI is

Coagulation necrosis followed by

Inflammation, resorption of necroticmyocardium followed by

Formation of granulation tissue and finally

Organization of granulation tissue to form a

collagen rich scar tissue

collagen cFirm, grey; scarring complete2-8 weeks

Active granulatiRed-gray depressed border10-14 days

Early coagulatioDark Mottling (Occasional)4-12 hr

LM FEATUGROSS FEATURESTIME

Dense collagenScarring complete2 months +

Well developed

Extensive macr

Maximum softening

(no myocytes/no collagen)

7-10 days

Macrophages a

Breakdown of m

Central softening (Yellow/tan)

Hyperemic border

3-7 days

Heavy neutrophMottling (Yellow/tan center)1-3 days

Early neutrophi

Coagulation ne

Contraction ban

Dark Mottling12-24 hr

Wavy fibersNo change1/2-4 hr

No changeNo change0-30 min

123

Contractile dysfunction cardiogenic shockArrhythmiasPapillary muscle dysfunctionExternal rupture of the infarct(3-7 days) Ventricular free wall Ventricular septum

Mural thrombiVentricular aneurysms(after fibrosis, 2+ months)

Pericarditis Acute fibrinous or fibro hemorrhagic

Autoimmune Dresslers syndrome

COMPLICATIONS Clinical features

Severe, crushing, substernal chest pwhich may radiate to neck, jaw, epigashoulder, or left arm

Levines sign (clenched fist over ches

Pain lasts several hours to days and significantly relieved by vasodilators

Diaphoresis, dyspnea, rapid weake p

Silent MI

elderly patients underlying Diabetes mellitus/ Hypertens

125

Clinical course

25% sudden death (SCD) vast majority of

deaths occurring before hospitalizationPatients reaching hospital alive

10 20% uncomplicated course

80 90% complicated but not necessarilyfatal course

Complicated course Cardiac arrhythmias 75 95%

LVF with pulmonary edema 60%

Cardiogenic shock 10%

Rupture of myocardium 4 8 %

Diagnosis of MI

Myocardial infarction is diagnosed wblood levels of sensitive and specificbiomarkers, such as cardiac troponin

the MB fraction of creatine kinase (CMB), are increased in the clinical sett

acute ischemia

JACC, vol 36, No. 3, 200


22/36

127

Electrocardiographic abnormalities

segmentevation

wave

ersion ofwave

Cardiac Injury Markers

Creatine Kinase isoenzymes (CK-M

Troponins (I and T)

LD (lactate dehydrogenase, LDH)

Myoglobin

129

Troponin I and TTroponins are most sensitive and specific

Regulate calcium-mediated muscle contraction

Normally not detectable in circulation

Troponin I (TnI) commonly measured clinically

Rise in 2-4 hours

Peak at 48 hours

Persists for 7-10 days

Creatine Kinase

CK is a dimer composed of M and B

subunits: MM, MB, BB

CK-MB: most specific for heart, trace

amount in skeletal muscle

Rises within 2-4 hours of MI

Peaks at 24 hours

Disappears by 72 hours

131

Cardiac Injury Markers

Early MI, TnI and CK-MB with similar sensitivity

Troponin is most specific for cardiac injury

Troponin STAYS elevated long after CK is gone

Both elevate sooner in reperfused patients

2 days of unchanged TnI and CK-MB r/o AMI

Intervention

Primary prevention

Thrombolysis

Angioplasty

Coronary bypass graft (CABG)


23/36

133

Chronic ischemic heart disease Insidious onset of congestive heart failure

in patients who have past episodes of MIor anginal attacks

Cardiac decompensation owing toexhaustion of the compensatoryhypertrophy of non- infarcted viablemyocardium or severe coronary

obstructive disease leading to diffusemyocardial dysfunction Dilation of all 4 heart chambers (may

be difficult to distinguish from dilatedcardiomyopathy

Sudden Cardiac Death

Unexpected death from cardiac causearly (usually within 1 hour) after orwithout the onset of symptoms

Causes IHD most common cause

Mitral valve prolapse

Aortic valve stenosis Dilated or hypertrophic cardiomyopathy

Myocarditis

135

Ultimate mechanism of SCD is a lethal

arrhythmia

Morphology

80-90% of cases critical stenosis of one ormore coronary arteries, high grade stenosisassociated with acute plaque disruption

10-20% - non atherosclerotic in origin

Hypertensive heart diseas

Presence of left ventricular hypertrop

an individual with a history of hyperteand in whom other causes of hypertr

have been excluded

137

Pathogenesis

Stimulus sustained pressure overload

Growth factorsLocal mechanical effects

Changes in genes controlling expression of actin, myosin

Hypertrophy

Morphology

Concentric hypertrophy of left ventricsymmetric, circumferential pattern

Long standing cases right ventricul

hypertrophy and dilation

Histologically enlarged myocytescontaining large, hyperchromatic,

rectangular box-car shaped nuclei


24/36

139

Clinical features

Early stages asymptomatic

Angina pectoris

Signs and symptoms of heart failure withprogression

Cerebrovascular accidents (stroke)


Pulmonary heart Disease(Cor Pulmonale)

Disease of right sided cardiac chamb

secondary to pulmonary parenchymapulmonary vascular diseases

Excluded from this definition are

Pulmonary hypertension due to left heafailure

Pulmonary hypertension due to congenheart disease

141

Acute cor pulmonale

Pulmonary embolism causing sudden

increase in burden on the right heart

Right ventricle is dilated but nohypertrophy

Possible cause - Saddle embolus

Valvular Heart Disease

Congenital

Acquired

Valvular involvement by disease causes

Stenosis failure of a valve to open completethereby impeding forward flow

Incompetence, regurgitation, or insufficiency of a valve to close completely, thereby allowinreversed flow

Abnormalities of flow often produce abnor

heart sounds known as murmurs

143ocarditis

Marfan syndromeated cardiomyopathy

Syphilitic aortitisective endocarditis

Degenerative aortic dilation

hypertension

eumatic heart disease

Rheumatic heart diseasexomatous degeneration (mitral

ve prolapse

AORTIC REGURGITATIONTRAL REGURGITATION

Calcification of congenitally deformed

valve

Senile calcific aortic stenosis

Rheumatic heart diseaseeumatic heart disease

AORTIC STENOSISTRAL STENOSIS

Aortic valve diseasetral valve diseaseRheumatic fever

Acute, immunologically mediated, multisysinflammatory disease that follows an episorheumatogenic group A streptococcalpharyngitis after an interval of few weeks.

Occurs in only about 3% of patients with gstreptococcal pharyngitis

Mainly a disease of third world countries acrowded, economically depressed areas owestern world


25/36

145

Pathogenesis

Exact pathogenesis is not known

Hypersensitivity reaction induced by group Astreptococci

Antibodies directed against the M proteins ofgroup A streptococci cross react with normalproteins present in heart, joints, and othertissues

Evidence in support of this hypothesis Streptococci are absent from the lesions

Symptoms do not develop until 2-3 weeks afterinfection

T-cells activated

by streptococcal

antigensB-cells produce anti-streptococcal

antibodies

Antibodies and T-cells cross rea

Cardiac sarcolemma and valvular gly

147

Morphology- Acute rheumatic fever

Acute rheumatic carditis inflammatory changesin all three layers of the heart Pancarditis

Myocarditis - Hallmark is the presence of Aschoffbodies within connective tissue of the heart.

Endocarditis edematous and thickened valveswith foci of fibrinoid necrosis

Pericarditis fibrinous pericarditis

Arthritis chronic inflammatory infiltrand edema in the joints and periartic

soft tissues.

arthritis is self limited and does not causchronic deformities

Erythema marginatum maculopapurash

Skin nodules contain focal lesions t

are essentially large Aschoff bodies

149

Chronic rheumatic heart disease

Chronic Mitral valvulitis Conspicuous irregular thickening and calcification of

the leaflets, often with fusion of the commissuresand the chordae tendineae

Chronic aortic valvulitis cusps are thickened, firm,and adherent to each other valve orifice is reduced torigid, triangular channel

Clinical features

Acute rheumatic fever Occurs anywhere from 10 days to 6 we

after an episode of group A streptococcpharyngitis

Peak incidence 5-15 years

Arthritis migratory, large joints

Carditis pericardial friction rub, weak sounds, congestive heart failure

Stenosis of mitral valve, most common characteristic complication


26/36

151

Clinical features

Chronic rheumatic carditis

Valvulitis - murmurs

Cardiac hypertrophy and dilation

Congestive heart failure

Arrhythmias

Infective endocarditis

Diagnosis Jones criteria

Major manifestations (ACCNE) Migratory polyarthritisof large joints Pancarditis Sydenham chorea involuntary, purposeless, rapid move

Subcutaneous nodules Erythemamarginatum of skin

Minor manifestations Fever, Arthralgia, and elevated

Presence of either of the 2 major manifestations major and 2 minor manifestations

+Evidence of preceding streptococcal infection in tof elevated serum ASO titers or positive streptocthroat culture

153

Calcific aortic stenosis

Narrowing of the aortic valve lumen as aresult of deposition of calcium in the cuspsand the valve ring.

It can occur in Elderly patients as a degenerative process.

90% of the patients are > 65 years of age

Congenital bicuspid aortic valve(40-65 years)

Aortic valve scarred as a result of rheumaticfever(5-15 years)

Mitral valve prolapse

One of the most common cardiac dis

occurring in 3-5% of the general adupopulation.

Most cases are discovered between ages of 20 and 40 years

More common in females (7x)

May arise as a complication of Marfa

syndrome

155

Morphology

Soft and enlarged mitral valve cusps ballooning of the valve leaflets into the left

atrium during systole

The chordae tendineae which are often

elongated and fragile, may rupture in

severe cases

The mitral annulus may be dilated

Clinical features

Most patients are asymptomatic Palpitations

Fatigue or atypical chest pain

Mid-systolic click (when valve prolap

Severe complications in about 3 % o Mitral regurgitation and congestive hea

failure


Ventricular arrhythmias


27/36

157


Infection of the cardiac valves or mural

surface of the endocardium resulting in theformation of adherent, bulky mass of

thrombotic debris and organisms termedvegetations

Classification

Clinical

Acute endocarditis destructive fulminant infrequently of a previously normal heart valve whighly virulent organism, that leads to death wdays to weeks of more than 50% of patients dantibiotics and surgery.

Sub acute endocarditis organisms of low vcause infection in a previously abnormal heart

particularly on deformed valves. Most patientsrecover with appropriate therapy.

Etiological Bacterial, Fungal, Rickettsial

159

Predisposing factorsPreexisting cardiac abnormality

Mitral valve prolapse most common factor

Chronic rheumatic valvulitis

Degenerative calcific aortic stenosis

Prosthetic heart valves 10-20% of cases

ntravenous drug abuse (Tricuspid valve!)

Transient bacteremia dental procedures,

urinary catheterization, endoscopy.

Pathogenesis

Hemodynamic factors Abnormal blood flow across a damaged

Endothelial injury

Focal deposition of platelets and fibrin

Adherence properties of microorgani Fibronectin

Adhesion factors - polysaccharides

161

Causative organisms Native valve

Most common (50-60%) Streptococcus viridans ; deformed

valves Staph. aureus (10-20%) previously healthy or deformed valves

HACEK GROUP Haemophilus, Actinobacillus,Cardiobacterium, Eikenella, Kingella

Prosthetic valve Staph. Epidermidis

Gram negative bacilli

fungi

Intravenous drug abuse Staph. Aureus most common

Streptococci

Gram negative bacilli

Fungi

Morphology

Mitral and aortic most commonly involved

Tricuspid valve I.V. drug abuse Acute bacterial endocarditis Gross

Bulky, friable vegetations that may obstruct thorifice

Rapid destruction of the valves rupture of thleaflets, chordae tendineae, papillary muscles

Ring abscess abscesses in perivalvularmyocardium

Microscopy Large number of organisms mixed with fibrin a

blood cells Minimal inflammatory response


28/36

163

Subacute endocarditis

Gross

Vegetations are less friable

Associated with less valve destruction

Ring abscesses uncommon

Microscopy

Presence of granulation tissue

Fibrosis, calcification

Chronic inflammatory infiltrate

Clinical features

Acute bacterial endocarditis

High grade fever with chills

Features of septicemia

Subacute bacterial endocarditis

Low grade fever; malaise

Weight loss

Changing cardiac murmurs

Splenomegaly

Clubbing of fingers

165

Glomerulonephritis

Immune complex formationase of bacterial

en

CNS emboli and stroke,myocardial infarction, splenic or

kidney infarcts, mycotic aneurysmSplinter hemorrhagesnailsJaneway lesions (palms/soles)Roth spotsretinal hemorrhageOsler nodessubQ nodules inpulp of digits

Peripheral emboli

mentation

Congestive heart failure

Heart blocks (1, 2or 3)

Valvular insufficiency

AV conductionabnormalities

ue destruction

Joint, bone, organ diseaseSeeding of distant sitesConstitutional symptomsCytokine release

stent bacteremia

Host ConsequenceComplicationonsequence ofVegetation Diagnosis

Repeated blood cultures for both aer

and anaerobic organisms

Treatment

Difficult infection to eradicate avasculnature of the valves

Antibacterial therapy

167

Nonbacterial thromboticEndocarditis

Characterized by the presence of sterilevegetations

Pathogenesis incompletely understood Endothelial abnormalities

Hypercoagulable states

Adenocarcinomas(pancreatic & other abdominal)

Usually asymptomatic

Embolization and infective endocarditis possible complications

Libman- Sacks endocardi

Characterized by presence of sterilevegetations on the cardiac valves inpatients of SLE

No special predilection for the lines oclosure (vegetations on both sides)

Vegetations comprised of fibrin & WB


29/36

169

Myocarditis

Generalized inflammation of the myocar-dium associated with necrosis anddegeneration of myocytes.

The inflammatory process plays a primary

role in the development of myocardial

injury.

Major causes of Myocardi

Transplant rejectionBacteria c. diphtheriae

Helminthes trichinosis

Protozoa chagasdisease, toxoplasmosis

Drugs sulfonamidesFungi aspergillus,candida

SLERickettsia typhus fever

Giant cell myoPost streptococcal rheumatic fever

Chlamydia- c. psittaci

SarcoidosisPost viralViral coxsackievirus,CMV, ECHO, HIV

UnknoImmune-mediated

Infections

171

Viral Myocarditis

Most common cause of myocarditis in US

Coxsackie A and B and other enter-

oviruses account for most cases

Pathogenesis

Direct viral cytotoxicity

Cell mediated immune reactions against

infected myocytes Diffuse lymphocyte infiltrate with patchynecrosisinfected myocyte

Clinical features

Most cases self limiting disease

Flu like symptoms viral myocarditis

Complications

Acute heart failure giant cell myocarditis

Chronic congestive heart failure viral myoca

Arrhythmias ventricular arrhythmias mostdangerous

173

Cardiomyopathy

Primary disease of the myocardium ex-cluding myocardial diseases caused byischemia, hypertension, valvular lesions,congenital anomalies, or inflammatorydisorders.

Three categories Dilated cardiomyopathy

Hypertrophic cardiomyopathy

Restrictive cardiomyopathy

Dilated Cardiomyopathy

Most common type of cardiomyopath(90%)

Characterized by

Progressive cardiac hypertrophy

Dilation(of all 4 chambers)

Contractile (systolic) dysfunction


30/36

175

EtiopathogenesisIdiopathic unknown, majority of cases

Genetic mutations 20-30% of cases Dystrophin gene on X- chromosome

Mitochondrial genes abnormal oxidative phosphorylation

Alcohol direct cytotoxicity

Viral myocarditis

Nutritional disturbances thiamine deficiency,chronic anemia,

Pregnancy associated - peripartum cardiomyopathy pregnancy associated hypertension (reversible)

Nutritional disturbances

Clinical features

Fundamental defect is ineffectivecontraction

Ejection fraction is < 25% (normal 5065%)

20-60 year most common

Progressive congestive heart failure

refractory to therapy Death usually occurs due to progress

cardiac failure or arrhythmias

177

Hypertrophic cardiomyopathy

Characterized by

Myocardial hypertrophy

Abnormal diastolic filling

Intermittent left ventricular outflow obstructionin one third of cases

Primarily a diastolic rather than systolicdysfunction

Pathogenesis

Familial autosomal dominant conditio

50% of cases.

Occurs due to genetic defect in any o

following genes

-myosin heavy chain most common

Cardiac troponin T

-tropomyosin

Myosin-binding protein C

179

Clinical features

Exertional dyspnea

Angina or MI

Sudden death due to arrhythmias(especially in atheletes)

Restrictive Cardiomyopath(Decreased compliance)

Least common type of cardiomyopat Etiology

Endomyocardial fibrosis most commocause

Lofflers syndrome

Radiation fibrosis

Amyloidosis

Hemochromatosis

Metastatic tumors Sarcoidosis


31/36

181

Congenital Heart Disease

Abnormalities of the heart or great vessels

that are present from birth

Most common form of heart disease

among children

1% of live births, higher in prematureinfants and stillborns

Incidence increased due to increased

diagnostic sensitivity

Etiology and pathogenes

Only 10% well-defined environmental or ginfluence

Trisomy 13,15,18,21, and Turner syndrom Trisomy 21 the most common known genetic c

Defect in sibling or parent the greatest risk

Environmental factors (congenital rubella)

183

Frequency of cardiacmalformations

1%Tricuspid atresia

1%Truncus Arteriosus

4%Transposition of the Great Vessels

5%Tetralogy of Fallot

4%Aortic stenosis

5%Coarctation of the Aorta

8%Pulmonary Stenosis

10%Atrial Septal Defect

7%Patent Ductus Arteriosus

42%Ventricular Septal Defect

% of Congenital

Heart Disease

Malformation

Malformation categories Based on flow of blood

Left-to-right shunt

Right-to-left shunt

Obstruction

Based on cyanosis

Cyanotic from birth

Cyanotic later in life

Not cyanotic

185

NormalFetal circulation


32/36

187

Right-to-left Shunt:Cyanotic heart defects

At birth: The Ts (Tetralogy of Fallot,

Truncus Arteriosus, Tricuspid Atresia,Total Anomalous Pulmonary Venous

Connection, Transposition of the GreatVessels)

After birth, when right-sided pressures

increase: Atrial Septal Defect, VentricularSeptal Defect, Patent Ductus Arteriosus

Tetralogy of Fallot

Most common form of cyanotic cong

heart disease

The four features of tetralogy are

Ventricular septal defect

Pulmonary stenosis

overriding aorta

right ventricular hypertrophy

189

Clinical features

Usually present by 6 months

Dyspnea on exertion, cyanosis

Polycythemia cerebral thrombosis


Pulmonary vasculature decreased

Associated with Downs Syndrome

191

Transposition of the Great Arteries

Aorta off RV andPulmonary artery offLV

AV connectionsnormal

Incompatible withpost natal life, unlessa shunt (VSD or PDA)is present

Truncus Arteriosus

Failure of partitioning of embryologictruncus into aorta and pulmonary arte

Single great artery gets blood from b

ventricles

Underlying VSD

Blood from both ventricles mixes

Cyanotic


33/36

193

Truncus Arteriosus Left-to right shunts: The D

Atrial Septal Defect

Ventricular Septal Defect

Patent Ductus Arteriosus

195

Remember

Ds are red (acyanotic, left-to-right shunt) and Ts are blue(cyanotic, right-to-left shunt).

Os

S

197

Ventricular Septal Defect

Most common congenital heart lesion Associated with Trisomy 21 (DS), 13, 18 Most commonly (90%) membranous Most VSDs close spontaneously in childhood

Clinical features: Pulmonary hypertension; CHF,pansystolic murmur,

Shunt reversal leads to cyanosis -EISENMENGER COMPLEX

Atrial Septal Defect

10% of CHD

Age at presentation variable, may be

asymptomatic into adult

Most common congenital cardiac

malformation diagnosed in adults

Pulmonary vascularity increased if sigleft-to-right shunt


34/36

199

e typese typese typese types

Ostium secundumOstium secundum Ostium secundumOstium secundum15% Ostium primum15% Ostium primum15% Ostium primum15% Ostium primum

10% Sinus venosus10% Sinus venosus10% Sinus venosus10% Sinus venosus


7% of cardiac malformations

90% isolated anomaly

Females more common than males

Continuous, machinery-like murmu

May need to keep open with prostag

E if associated with other malformatio Shunt may reverse leading to cyanos

201

PDA Pathology

Connect left pulmonary artery to aortic

arch

Closes with high oxygen tension

Higher incidence in maternal rubella

infection

Associated with polycythemia


203

Obstructive Congenital Anomalies

Coarctation of the Aorta

Pulmonary Stenosis and Atresia

Aortic Stenosis and Atresia (HypoplasticLeft Heart Syndrome)

Coarctation of the Aorta

Narrowed aortic lumen

Associated with Turners Syndrome

50% cases isolated cardiac anoma

Remaining cases associated with PVSD, ASD

Two types

Preductal (infantile)

Postductal (adult)


35/36

205

THOGENESIS

Clinical features

Preductal - Presents in infancy as Congestive heart failure Selective cyanosis of lower extremities Femoral pulses are weaker than those of the u

extremities

Postductal presents in older children and no selective cyanosis is seen Hypertension of the upper extremities

Blood pressure is low and pulses are weak in extremities Notching of ribs due to collaterals Intermittent claudication arterial insufficiency

207

TUMORS OF THE HEART

Primary tumors are rare

Most common are metastatic neoplasms

Clinical presentation

Sudden onset of severe, rapidly progressiveheart failure without apparent cause and/orarrhythmia

Silent till impair function

Cardiac Myxomas

Most common primary tumor

Female preponderance

Age 30 60 years

Often calcify and can, at times, bseen on X- ray

209

Morphology

Gross lobulated pedunculated mass

Most common location - Left atrium

Histologically multinucleated

stellate cells suspended in anedematous mucopolysaccharide rich

stroma

Clinical features

Most are asymptomatic

Some may fragment and embolize

Ball-valve obstruction of atrioventricuvalve syncopal episodes, sudden d

Associated with diastolic murmur


36/36

211

Secondary Tumors

Direct extension of lung cancer

Breast

Lymphoma

Malignant melanoma

Pericardial diseases

Effusions - Serous

Congestive heart failure Hypoalbuminemia

Serosanguineous Trauma malignancy

Chylous mediastinal lymphatic obstructi

Hemopericardium cardiac tamponade Ruptured aortic aneurysm or myocardial infarc Penetrating traumatic injury

213

Pericarditis

Primary uncommon; usually infectious in

origin; virus most commonly

Secondary acute MI, cardiac surgery,

radiation, rheumatic fever, SLE,

Dresslers Syndrome

Uremia most common systemic disorderassociated with pericarditis

Clinical features

Chest pain; worsens on reclining

High-pitched friction rub

Cardiac tamponade

Faint distant heart sounds

Distended neck veins

Declining cardiac output

Shock

Morphology

Acute pericarditis fibrinous pericarditis

Chronic pericarditis

Delicate adhesions to dense, fibrotic scarsthat obliterate the pericardial space

Extreme cases heart is completely encasedith d ti t d

Documents

Cardiovascular + Clinical Scenarios