Valvular Heart Disease Kenneth S Korr M D

Valvular Heart Disease Kenneth S. Korr M. D. Associate Professor of Medicine, Brown Medical School Director, Division of Cardiology The Miriam Hospital

Normal Valve Function n n Maintain forward flow and prevent reversal of flow. Valves open and close in response to pressure differences (gradients) between cardiac chambers.

Abnormal Valve Function n Valve Stenosis n n n Valve Regurgitation, Insufficiency, Incompetence n n n Obstruction to valve flow during that phase of the cardiac cycle when the valve is normally open. Hemodynamic hallmark -“pressure gradient” ~ flow// VA Inadequate valve closure--- back leakage A single valve can be both stenotic and regurgitant; but both lesions cannot be severe!! Combinations of valve lesions can coexist n n Single disease process Different disease processes One valve lesion may cause another Certain combinations are particularly burdensome (AS & MR)

Mitral Valve Competence: n Integrated function of several anatomic elements n n n Posterior LA wall Anterior & Posterior valve leaflets Chordae tendineae Papillary muscles Left ventricular wall where the papillary muscles attach

Mitral Valve Disease: Etiology v Mitral Stenosis v v v Rheumatic - 99. 9%!!! v Congenital Prosthetic valve stenosis Mitral Annular Calcification Left Atrial Myxoma Acute Mitral Regurgitation v v Infective endocarditis Ischemic Heart disease v v v Chordal rupture v Chest trauma Papillary ms dysfunction Inferior & posterior MI Mitral Valve prolapse Infective endocarditis Rheumatic Prosthetic Mitral annular calcification Cardiomyopathy Mitral valve prolapse v v Papillary ms rupture Chronic Mitral Regurgitation LV dilatation IHSS

Mitral Regurgitation. Pathophysiology n n n MR: Leakage of blood into LA during systole 10 Abnormality -Loss of forward SV into LA Compensatory Mechanisms n n Increase in SV (& EF) Forward SV + regurgitant volume LV (LA) dilatation Left Ventricular Volume Overload (LVVO)

Chronic Mitral Regurgitation LVVO n n n n LV dilatation Eccentric hypertrophy Increased LA pressure Pulmonary HTN Dyspnea Atrial arrhythmias Low output state

Pathophysiology –Acute vs Chronic Mitral Regurgitation n Acute MR n n n Chronic MR n n n Normal (noncompliant) LA Increase LA pressure large “V” waves Acute Pulmonary Edema Dilated, compliant LA LA pressure normal or slightly increased Fatigue, low output state Atrial arrhythmias- a. fib. Most patients fall between these two extremes!!

Mitral Regurgitation: Physical Findings n Auscultatory Findings n n n S 1 – soft or normal P 2 – increased Holosystolic blowing murmur @ apex n n n MVP – mid-systolic click IHSS – murmur increases with Valsalva Acute MR – descrescendo systolic murmur S 3 gallop & diastolic flow rumble Hyperdynamic Left Ventricle n n n Brisk carotid upstrokes Hyperdynamic LV apical impulse LA lift; RV tap

Mitral Stenosis -Pathophysiology n n Restriction of blood flow from LA LV during diastole. Normal MVA 4 -6 cm 2. n n MV Pressure gradient – MV grad ~ MV flow//MVA. n n Mild MS 2 -4 cm 2. Severe MS < 1. 0 cm 2. Flow = CO/DFP (diastolic filling period). As HR increases, diastole shortens disproportionately and MV gradient increases.

Relationship between MV gradient and Flow for different Valve Areas n n n Cross hatched area indicates range of normal resting flow. The vertical line represents the threshold for developing pulmonary edema. Pressure gradient increases as flow increases: n n n to a small degree with normal valve area(46 cm 2). to greater degrees with smaller valve areas. in severe stenosis, a significant gradient is present at rest.

Mitral Stenosis-Pathophysiology n n MV gradient Incr LA pr Pulmonary HTN n n n RV Pressure Overload n n n Passive Reactive- 2 nd stenosis RVH RV failure Tricuspid regurgitation Systemic Congestion Paradoxes of MS n n n Disease of Pulm Arts & RV LV unaffected (protected) As RV fails, pulmonary symptoms diminish

Mitral Stenosis- Clinical Symptoms n n n Symptoms related to severity of MVA reduction. Symptoms unrelated to severity of MSn Atrial fibrillation n Systemic thromboembolism Symptoms due to Pulmonary HTN and RV failuren Fatigue, low output state n Peripheral edema and hepato-splenomegaly n Hoarseness –recurrent laryngeal nerve palsy

Mitral Stenosis: Physical Findings n Auscultatory findings n n S 1 – variable intensity; increased early, progressively decreases OS –opening snap, variable intensity A 2 -OS interval – varies inversely with severity of MS; shortens as MVA diminishes Low-pitched diastolic rumble @ apex n n n Duration of murmur correlates with severity of MS Pre-systolic accentuation Increased P 2 Body habitus – thin, asthenic, female Low BP LA lift & RV tap

Mitral Valve Disease – Echo findings n Mitral Stenosis n n n Thickened, deformed MV leaflets 2 D MVA Doppler Gradient Associated LAE, RVH, PHTN, TR, MR, LV function Mitral Regurgitation n Determine etiology – leaflets, chordae, MVP, MI Doppler severity of MR jet LV function

Mitral Valve Disease : Treatment n Mitral Stenosis n Medical Rx for Class I & II n n HR control – Dig & BB Anticoagulation n Chronic Mitral Regurgitation n Afib, >40 yrs, LAE, MR, prior embolic event Surgical Rx -Class III &IV Balloon Mitral Valvuloplasty n Commissural fusion n pliable, noncalcified leaflets n No MR of LA thrombus Mitral Valve Surgery n Open commissurotomy n MV replacement n Medical Rx for mild to mod MR with vasodilators, diuretics, anticoagulation Surgical Rx –ideally before LV systolic function declines. n n n MV replacement MV ring & CABG MR repair – associated with improved long-term LV funvtion n MVP, ruptured chords, infective endocadritis, pap ms rupture.

Balloon Mitral Commissurotomy

Aortic Valve Disease: Etiology n n Aortic Stenosis n n Degenerative calcific (senile) Congenital – Uni or bicuspid Rheumatic Prosthetic n Chronic Aortic Insufficiency Aortic leaflet disease n n n Aortic root disease n n n Acute Aortic Insufficiency n n Infective endocarditis Acute Aortic Dissection n n Marfan’s Syndrome Chest trauma Infective endocarditis Rheumatic Bicuspid Aortic valve Prolapse & congenital VSD Prosthetic n n Aortic aneurysm/dissection Marfan’s syndrome Connective tissue disorders Syphilis HTN Annulo-aortic ectasia

Aortic Stenosis - Pathophysiology n Normal AVA 2. 53. 0 cm 2 n n n Severe AS <1. 0 cm 2 Critical AS <0. 7 cm 2; <0. 5 cm 2/m 2 Hemodynamic Hallmark n n Systolic pressure gradient AV grad ~ AV flow//AVA n AV flow = CO/SEP (systolic ejection period)

Relationship between AV gradient and Flow for different Aortic valve areas. n n Like Mitral Stenosis – as flow increases so does the gradient. Unlike Mitral Stenosis – n Resting flows are higher n n smaller AV area shorter SEP Larger gradients Significant (>50 mm. Hg) gradient can be present at rest in asymptomatic individuals.

Pathophysiology of Aortic Stenosis. LVPO n n Chronic LV Pressure Overload Concentric LVH “Stiff” noncompliant LV n n n Well tolerated for decades n n Increased LVEDP Increased LV mass Increased MVO 2 LV fails CHF Atrial fibrillation n Poorly tolerated n n n Loss of atrial “kick” Rapid HR Acute pulmonary edema and hypotension.

Aortic Stenosis: Natural History & Clinical Symptoms n n Asymptomatic for many years Symptoms develop when valve is critically narrowed and LV function deteriorates n n n Bicuspid AV 5 th - 6 th decade Senile AS 7 th-8 th decades Classic Symptom Triad n n Angina pectoris – 5 years CHF 1 -2 years Syncope 2 -3 years Sudden Death n Natural History Studiesn n n Pts grad 25 mm. Hg – 20% chance of intervention in 15 years Pts with asymptomatic severe AS require close f/u Gradient progression n n 6 -10 mm. Hg/yr Risk Factors n n n Age > 70 CAD, hyperlipidemia Chronic renal failure

Aortic Stenosis: Physical Findings Severity of AS Mild Moderate Severe Carotid pulse normal Slow rising Parvus et Tardus LV apical impulse normal heaving Heaving & sustained Auscultation S 4 gallop - +/- ++ Systolic ejection Click + +/- - SEM, peaking Early systole mid-to-late systole S 2 Normal or single Single or paradoxical normal

Aortic Insufficiency. Pathophysiology n n 10 abnormality – LVVO Severity of LVVO n n Size of regurgitant orifice Diastolic pressure gradient between Ao & LV HR or duration of diastole Compensatory Mechanisms n n n LV dilatation & eccentric LVH Increased LV diastolic compliance Peripheral vasodilation

LV Volume vs Pressure Overload Feature LVPO (AS) LVVO (MR, AI) LV Volume normal Dilated** Wall thickness Conc. LVH Normal to slightly increased LV compliance “stiff” noncompliant Increased compliance LV diastolic Pr increased Normal to slightly increased LV systolic Pr Increased** Normal to slightly increased LVEF normal increased

Acute vs Chronic AR Pathophysiology and Clinical Presentation n Acute Aortic Regurgitation n n Sudden Ao. V incompetence Noncompliant LV Acute Pulmonary Edema Emergency AVR Chronic Aortic Regurgitation n n Long asymptomatic phase Progressive LV dilatation DOE, orthopnea, PND Frequent PVC’s

Chronic Aortic Regurgitation: Physical Findings n n Widened Pulse Pressure > 70 mm. Hg (170/60) Low diastolic pressure <60 mm. Hg Hyperdynamic LV – n De. Musset’s signs n Corrigan’s pulse n Quincke’s pulsations, n Durozier’s murmur Auscultation: n Diminished A 2 n Descrescendo diastolic blowing murmur @ LSB n Austin-Flint murmur – diastolic flow rumble @ apex n n Due to interference with trans-mitral filling by impignement from aortic regurgitant jet. DDx - mitral stenosis(increases intensity with amyl nitrite)

Aortic Valve Disease: Diagnostic Testing n n Aortic Stenosis EKG- NSR, LVH with strain, LAE, LAD CXRay – frequently normal 2 D-ECHO n n n Aortic cusps –thickened, calcified, decreased mobility Assessment of LVH & LV systolic function Concomitant MR, AR Doppler assesment of Ao. V gradient Planimetry of AV area n n n Aortic regurgitaiton EKG- LVH without strain CXRayn n n Chronic AI – “cor bovinum” Acute AI – pulmonary edema with nl heart size 2 D ECHO n n Assess Ao valve and root Assess LV function/dilatation n n LVES dimension>55 mm Doppler severity of regurgitant jet

Relationship between AV gradient and Flow for different Aortic valve areas. n n Like Mitral Stenosis – as flow increases so does the gradient. Unlike Mitral Stenosis – n Resting flows are higher n n smaller AV area shorter SEP Larger gradients Significant (>50 mm. Hg) gradient can be present at rest in asymptomatic individuals.

Balloon Aortic Valvuloplasty n Indications for BAV in critical Aortic Stenosis n n n n Younger patients with congenital AS and predominant commissural fusion Bridge to eventual AVR Moderate to severe heart failure/cardiogenic shock Extremely high risk for AVR Urgent/emergent need for noncardiac surgery Patient with limited lifespan – cardiac or noncardiac Patient refuses surgery

Aortic Valve Surgery: Ross Procedure n n Autotransplant of pulmonic valve to the aortic position Reimplantation of the coronary arteries Homograft valve in the pulmonic position Indications n n n Younger patients No anticoagulation Requires similar sized aortic and pulmonic roots

Valvular Heart Disease The End