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Valvular Heart Disease

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

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Normal Structure Mitral Valve

 Cross sectional Area 4-6cm ²   Anterior and Posterior Leaflets Chordae Tendineae  Papillary Muscles

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Mitral Stenosis Etiology & Pathology

 Rheumatic Fever- 99%  Other – Congenital – Carcinoid – Lupus – Amyloid – – Infective Endocarditis Mucopolysaccharide Disease

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Stenotic Pathology

 Etiology & Pathology –  Commissural  Cuspal  Chordal 30% 15% 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 cm ² Critical MS- <1 cm ² – 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 S 1 -thickened leaflets  Increased P 2 -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 V 1 and Lead II RVH- right axis deviation – R wave > S wave in V 1

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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

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Cardiac Catherization

 Gorlin 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 cm ²/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 cm ²-NYHA III or IV <1 cm ² 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 S 1 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 Valve Normal 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 3-4cm ²

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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

Acquired Aortic Stenosis

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 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

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 Critical (Surgical) AS – – Peak systolic pressure gradient > 50mmHg in the presence of normal cardiac output Valve area <0.7-0.8cm

²  Moderate AS – 1-1.5cm

²  Mild AS – 1.5-2cm ²  Aortic Sclerosis

History

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  Long latent period of increasing obstruction Symptoms usually begin in 5 th or 6 th 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 S 2 , 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 0.12cm

² 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 0.7-0.8cm

² 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%

Aortic Regurgitation Etiology and Pathology

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 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 4 th or 5 th 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