1 Valvular Heart Disease Jay L. Rubenstone, D.O., F.A.C.C

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Valvular Heart Disease

Jay L. Rubenstone, D.O., F.A.C.C

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Normal StructureMitral Valve

Cross sectional Area 4-6cm² Anterior and Posterior Leaflets Chordae Tendineae Papillary Muscles

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Mitral StenosisEtiology & Pathology

Rheumatic Fever- 99% Other

– Congenital – Carcinoid– Lupus– Amyloid– Infective Endocarditis– Mucopolysaccharide Disease

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Stenotic Pathology

Etiology & Pathology Commissural 30% Cuspal 15% Chordal 10% Mixed Remaining

– Valve becomes funnel shaped or “fish mouthed”– Thickened immobile leaflets or chordal structures

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Stenotic Pathology

Debate: – Smoldering rheumatic process or – Constant blood flow trauma leading to valve fibrosis

and thickening

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Pathophysiology

Mild MS- orifice <2 cm² Critical MS- <1 cm²

– A-V pressure gradient >20mmHg– Increased LA Pressure– Increase Pulmonary Venous + Capillary Pressures– Increase Pulmonary Artery Systolic Pressure– Decrease RV Function (when PAS>30-60mmHg)

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Pathophysiology

Pulmonary HTN– Passive Backward Transmission Of Incr. LA

pressure Pulmonary Arteriolar Constriction Organic Obliterative Changes in Pulmonary Vascular Bed RV Failure

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History

Exertional Dyspnea Cough/Wheezing Orthopnea/PND/CHF Hemoptysis-Rupture of Pulmonary Vein-

Bronchial Vein Shunts

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History

Chest Pain-Increase RV Pressures or Unknown Etiology

Systemic Emboli (LA clots)– Increased LA size, Decreased C.O., Atrial Fib, IE– Significantly decreased w/anticoagulation

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Physical Exam

Auscultation O.S. Diastolic Rumble Assoc Murmur of MR Loud S1-thickened leaflets Increased P2-pulmonary hypertension

Decreased B/P if C.O. decreased Prominent a wave if sinus rhythm present

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Physical Exam

Mitral Facies-pink, purple facial patches due to decrease CO and systemic vasoconstriction

Hepatomegally Edema Ascites Hydrothorax With Right Heart Failure

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Diagnosis

ECG– Left Atrial Abnormality

P wave becomes bifid and greater than 0.12 sec in duration in V1 and Lead II

– RVH- right axis deviation– R wave > S wave in V1

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Diagnosis

Chest X-ray– Dilated LA, RA, RV– Elevated Left Main stem Bronchus– Interstitial Edema

Echo- Cornerstone of Diagnosis– Thickened Calcified Leaflets– Doming of Leaflets on Opening– Bernoulli equation

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Diagnosis

– Cardiac CatherizationGorlin Equation

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Natural History

Asymptomatic for 15-20yrs following Rheumatic Fever

Additional 5-10 yrs for progression from mild to severe stenosis

Stenosis progression approximately .09 cm²/yr

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Natural History

Presurgical Survival Rates– NYHA Class II 80%-10yrs– Class III 38%-10yrs, 62% 5yrs– Class IV 15%-5yrs

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Management-Medical

Endocarditis Prophylaxis Activity Limitation Diuretics- Decrease Na Intake Heart Rate Control for A-fib or Sinus Rhythm Anticoagulation

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Percutaneous Balloon Angioplasty

Moderate-Severe MS Mild MS- if Pulmonary Artery Pressures or

Wedge Pressure Elevate with Exercise

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Valve Replacement

Indications– Combined MS/MR– <1.5 cm²-NYHA III or IV– <1 cm²– Class II if Pulmonary Artery Pressure >70mmHg

Mortality– 3-8%

Valve Type-Prosthetic or Bioprosthetic

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Mitral Regurgitation

Etiology– Rheumatic Heart Disease– Infective Endocarditis– Collagen Vascular Disease– Cardiomyopathy– Ischemic Heart Disease– Mitral Valve Prolapse-most common cause for valve

surgery in US

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Pathophysiology

Decreased Impedance to Ventricular Emptying Determinants of Regurgitant Flow

– Instantaneous Size of MV Orifice– Dependent on Preload, After load, LV Contractility,

LV Size– LA-LV Pressure Gradient dependent on Systemic

Vascular Resistance, LV Pressure, & LV Size

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Pathophysiology

LV Compensation– Increased End Diastolic Volume– Increased Wall Tension– Increased Preload– Increased LV Emptying– Normal Ejection Fraction should be Super Normal

>65% to maintain forward cardiac output and B/P

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Pathophysiology

LV Decompensation– Increase End Systolic Volume– Increased End Diastolic Volume– Leads to Annulus Dilatation (MR begets MR)– Decreased Ejection Fraction and Stroke Volume

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Pathophysiology

Ejection Fraction in Mitral Regurgitation– >65% normal in compensated MR– 50-65% mild impairment– 40-50% moderate-severe impairment– <35% advanced impairment

As ejection fraction decreases operative risk increases.

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History

Shortness of Breath Exertional Dyspnea Congestive Heart Failure Right Heart Failure Significant symptoms in chronic MR usually do

not develop until LV decompensation occurs.

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History

Medical Treatment Survival– 80% 5yr– 60% 10yr– 30-45% 5yr if MR severe

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Diagnosis

Physical Exam– Holosystolic Murmur– Increase Carotid Impulse

ECG– LA abnormality– LVH– RVH

Chest X-ray– Increase LA, LV, RV, Interstitial Edema

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Diagnosis

Echo– Transesophageal superior to transthoracic– Evaluation of Chamber Sizes, Regurgitant Jet,

Leaflets

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Management of Acute MR

Medical– After load Reduction (Nitropresside & Intra aortic

balloon pump) Decrease impedance to LV ejection Decrease regurgitant volume into left atrium

– Inotropic Support (Dobutamine)-if LV function reduced

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Management of Acute MR

Surgical Intervention– Progressive LV Failure or Hemodynamic

Deterioration– CHF– Hypertension– Valve Disruption

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Management of Chronic MR

Medical– Digoxin– Diuretics*– After load Reduction– Anticoagulation in A-fib– Endocarditis Prophylaxis

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Management of Chronic MR

Surgical– Indications

Asymptomatic Class I– EF < 60% or LV Systolic Diameter >45mm

Severe MR Class II, III, or IV – generally considered for surgery unless EF <30%

– Valve Repair vs. Replacement

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Mitral Valve Prolapse

Systolic Click-Murmur Syndrome Barlow’s Syndrome Billowing Mitral Valve Syndrome Floppy Valve Syndrome Myxomatous Valve Syndrome Parachute Valve

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Mitral Valve Prolapse

Over diagnosed– 2.4% of population– Females>Males 2:1– Severe MR- Elderly Male>Young Female

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MVP Etiology

Primary Valvular most frequent Connective Tissue Diseases Hyperthyroidism Myotonic Dystrophy Periarteritis Nodosa Von Willebrands

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MVP Pathology

Myxomatous Proliferation and Degeneration of Valve Leaflets

Increased Quantity of Acid Mucopolysaccharide in Middle Layer of Valve Tissue

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MVP History

Most are asymptomatic throughout life Chest pain, fatigue, anxiety Orthostasis-questionable autonomic

dysfunction Arrhythmia-SVT, PACs, PVCs Symptoms of MR if present

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Physical Examination

Body type– Asthenic, low weight body habitus, straight back

syndrome

Auscultation– Systolic click- multiple, non-ejection (after carotid

upstroke) due to tensing of elongated chordae and prolapsing valve

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Physical Examination

Auscultation– Murmur- mid to late crescendo progressing to

holosystolic if MR becomes severe– Click and murmur move closer to S1 during strain

phase of valsalva, sudden standing, and Amyl Nitrate

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Diagnosis

ECG and Chest X-ray– Normally unremarkable

Echo– Billowing of one or both leaflets into the left atrium

during systole at least 2mm– Parasternal long axis view for diagnosis– Associated MR– Leaflet thickness

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Natural History

Progressive MR in 15% over 10-15 yrs Infective Endocarditis Cerebral Emboli-tearing of endothelial covering

of myxomatous valve with platelet activation Sudden Cardiac Death-V fib, increased Q-T

interval (not well established)

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MVP Management

Endocarditis prophylaxis if MR present Holter monitor-beta blocker for ectopy? Aspirin if focal neurological events present MR-treat like any other MR, valves usually

amenable to repair *MVP is usually a benign disease*

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Aortic ValveNormal Structure

Valve sits at the base of Aortic Root Three Leaflets (cusps)-non coronary, right

coronary, left coronary Cusps give rise to ostea of right coronary

artery and left main coronary artery Normal cross-sectional area 3-4cm²

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Aortic Stenosis Etiology and Pathology

Valvular Supravalvular Subvalvular Hyperthrophic Cardiomyopathy

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Congenital Aortic Stenosis

Unicuspid– Presents less than one year of age

Bicuspid– Adult Presentation– Chronic turbulent flow– Leads to fibrosis, rigidity, calcification

Tricuspid– Leaflets of unequal size

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Acquired Aortic Stenosis

Rheumatic– Rare– Usually mitral valve also involved

Degenerative or Senile– Most common cause of adult AS– Most common cause of valve replacement– Years of normal mechanical stress leads to calcium

deposits on leaflets– Inflammatory or Infectious component??– >age 65 2% frank AS, 30% Aortic Sclerosis

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Is this atherosclerotic disease?

Degenerative A.S. accelerated in diabetes and hyperlipidemia.

Associated with tobacco use and HTN. Potentially treated with HMGcoA agents?

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Hemodynamics

Critical (Surgical) AS– Peak systolic pressure gradient > 50mmHg in the

presence of normal cardiac output– Valve area <0.7-0.8cm²

Moderate AS– 1-1.5cm²

Mild AS– 1.5-2cm²

Aortic Sclerosis

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History

Long latent period of increasing obstruction Symptoms usually begin in 5th or 6th decade Angina in 2/3 of patients

– Hypertrophied myocardium– Increased ventricular systolic pressure– All of which increase myocardial oxygen

consumption– Oxygen supply-demand imbalance leads to

subendocardial ischemia

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History

Syncope– Reduced cerebral perfusion– Vasodilatation in the presence of fixed cardiac

output leads to hypotension– Baroreceptor-vasodepression due to high LV

systolic pressure Dyspnea (CHF)

– Particularly with exertion due to fixed cardiac output– Pulmonary Venous HTN can lead to CHF

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Diagnosis

Physical Examination – Systolic Murmur

Diamond-Shaped, harsh, left sternal boarder to right intercostal spaces, neck and apex

Late peak, obliteration of S2, consistent with bedside Dx of Critical AS

– Pulses Parvus Delayed and Prolonged Carotid Impulse

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Diagnosis

ECG– Classic LVH

Chest X-ray– Concentric LVH– Calcification of Aortic Valve

Echo– Bernoulli (continuity) equation-calculation of LV-

Aortic pressure gradient and valve area

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Diagnosis

Cardiac Catherization– Gorlin Equation

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Natural History

Asymptomatic latent period With moderate-severe AS valve area can

decrease on average 0.12cm² per year *Angina, syncope or CHF

– Average 1-3 year survival 50% – Sudden cardiac death rare

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Medical Management

Endocarditis Prophylaxis Limit Physical Activity Watch Beta Blockers and Diuretics *Treatment of Critical AS in viable candidates

is surgery

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Surgery (Valve Replacement)

Indications– Symptomatic Patients -valve area 0.7-0.8cm² or less– Asymptomatic Patients-progressive LV dysfunction

(EF <35%) or hypotensive response to mild exercise Delaying surgery in asymptomatic patients with good

exercise tolerance is controversial

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Surgery (Valve Replacement)

Results– Effective prosthetic valve area not normal– Surgery replaces Critical AS with Non-critical AS– Symptoms can persist if valve-patient mismatch

occurs– 10 year survival –85%

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Aortic RegurgitationEtiology and Pathology

Valvular– Rheumatic-Fibrotic Retraction of Leaflets

Ankylosing Spondylitis, Behcets, Psoriatic Arthritis, Giant Cell Arteritis

– Degenerative AS-75% w/AR– Infective Endocarditis-Leaflet Destruction– Trauma-ascending aortic tear– Bicuspid aortic valve-prolapse or incomplete closure– Myxomatous Degeneration-like MVP– Appetite suppressant drugs-serotonin related valve

deposits

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Etiology and Pathology

Aortic Root Disease-More common than primary valvular. Root Dilatation leads to non-coaptation of leaflets.

– Degenerative-Hypertensive Aortic Dilatation– Cystic Medial Necrosis-Classic Marfans Syndrome– Aortic Dissection– Syphilitic Aortitis– Rheumatic Disease-same as valvular

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History

Acute AR– LV cannot accommodate acute regurgitant volume – can lead to cardiovascular collapse

Chronic AR– Gradual LV enlargement-eccentric hypertrophy– Exertional dyspnea, orthopnea, PND, CHF– Presents 4th or 5th Decade

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Physical Examination

Diastolic Murmur– Left sternal boarder– Decrescendo, high pitched– Best heard Sitting Up, End Expiration– Longer murmur equals worse AR

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Physical Examination

de Mussett’s Sign (head bobbing) Corrigan’s Pulse “water hammer”

– Abrupt Distention with Quick Collapse

Bisferiens-pulse– 2 peaks

Traube’s Sign– Pistol shot sounds over femoral pulse

Duroziez’s Sign– Murmur over femoral pulse with compression

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Physical Examination

Quinckes Sign– Capillary pulsations

Muller’s Sign– Systolic pulsations of uvula

Hill’s Sign– Popliteal pulse exceed brachial pulse by >

60mmHg

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Physical Examination

Korotkoff Sounds– Can persist to 0mmHg– Wide Pulse pressure

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Diagnosis

ECG– LVH

Chest X-ray– Cardiomegaly predominantly inferior and leftward

Echo– Can aid in detecting etiology, quantifying degree of

regurgitation, and assessing LV size and function Cardiac Catheterization

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Natural History

Acute AR– Cardiovascular collapse– Inotrophic agents and vasodilators – Prompt surgical intervention

Chronic AR– 75% Five Year Survival– 50% Ten Year Survival– Progressive downhill course of CHF, Episodic

Pulmonary Edema, Sudden Cardiac Death

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Medical Treatment

Acute AR– As above

Chronic AR– Asymptomatic Mild-Moderate

Follow by Echo Yearly Endocarditis Prophylaxis for all AR May not require medical treatment

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Medical Treatment

Symptomatic Moderate-Severe AR– Limit exertional activity– Aggressively treat B/P– Diuretics– Salt Restriction– Digoxin– Vasodilators (Nifedipine?)

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Surgical Treatment

Indications– Defer surgery for chronic severe AR if good

exercise tolerance, EF greater than 50%, end systolic diameter < 50 mmHg, and end diastolic diameter < 70 mmHg

– Be aware that progressive decline in LV function or size increases surgical morbidity and mortality

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Surgical Treatment

Mortality– 3-8% perioperative– 5-10% late mortality with significant preop LV

dysfunction