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

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

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

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

5. Mitral Valve Disease: Etiology  Mitral Stenosis  Rheumatic - 99.9%!!!  Congenital  Prosthetic valve stenosis  Mitral Annular Calcification  Left Atrial Myxoma  Acute Mitral Regurgitation  Infective endocarditis  Ischemic Heart disease  Papillary ms rupture  Mitral valve prolapse  Chordal rupture  Chest trauma  Chronic Mitral Regurgitation  Ischemic Heart disease  Papillary ms dysfunction  Inferior & posterior MI  Mitral Valve prolapse  Infective endocarditis  Rheumatic  Prosthetic  Mitral annular calcification  Cardiomyopathy  LV dilatation  IHSS

6. Mitral Regurgitation- Pathophysiology MR: Leakage of blood into LA during systole MR: Leakage of blood into LA during systole 1 0 Abnormality -Loss of forward SV into LA 1 0 Abnormality -Loss of forward SV into LA Compensatory Mechanisms Compensatory Mechanisms Increase in SV (& EF) Forward SV + regurgitant volume LV (LA) dilatation Left Ventricular Volume Overload (LVVO)

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

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

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

10. Mitral Stenosis -Pathophysiology Restriction of blood flow from LA  LV during diastole. Restriction of blood flow from LA  LV during diastole. Normal MVA 4-6cm 2. Normal MVA 4-6cm 2. Mild MS 2-4cm 2. Mild MS 2-4cm 2. Severe MS < 1.0cm 2. Severe MS < 1.0cm 2. MV Pressure gradient – MV Pressure gradient – MV grad ~ MV flow//MVA. MV grad ~ MV flow//MVA. Flow = CO/DFP (diastolic filling period). Flow = CO/DFP (diastolic filling period). As HR increases, diastole shortens disproportionately and MV gradient increases. As HR increases, diastole shortens disproportionately and MV gradient increases.

11. Relationship between MV gradient and Flow for different Valve Areas Cross hatched area indicates range of normal resting flow. Cross hatched area indicates range of normal resting flow. The vertical line represents the threshold for developing pulmonary edema. The vertical line represents the threshold for developing pulmonary edema. Pressure gradient increases as flow increases: Pressure gradient increases as flow increases: to a small degree with normal valve area(4- 6cm 2 ). to a small degree with normal valve area(4- 6cm 2 ). to greater degrees with smaller valve areas. to greater degrees with smaller valve areas. in severe stenosis, a significant gradient is present at rest. in severe stenosis, a significant gradient is present at rest.

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

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

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

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

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

17. Balloon Mitral Commissurotomy

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

19. Aortic Stenosis - Pathophysiology Normal AVA 2.5- 3.0cm 2 Normal AVA 2.5- 3.0cm 2 Severe AS <1.0cm 2 Severe AS <1.0cm 2 Critical AS <0.7cm 2 ; <0.5cm 2 /m 2 Critical AS <0.7cm 2 ; <0.5cm 2 /m 2 Hemodynamic Hallmark Hemodynamic Hallmark Systolic pressure gradient Systolic pressure gradient AV grad ~ AV flow//AVA AV grad ~ AV flow//AVA AV flow = CO/SEP (systolic ejection period) AV flow = CO/SEP (systolic ejection period) 50-100mmHg gradients are common in severe AS 50-100mmHg gradients are common in severe AS

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

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

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

23. Aortic Stenosis: Physical Findings Severity of AS MildModerateSevere Carotid pulse normal Slow rising Parvus et Tardus LV apical impulse normalheaving Heaving & sustained Auscultation S 4 gallop -+/-++ Systolic ejection Click ++/-- SEM, peaking Early systole midsystole mid-to-late systole S2S2S2S2normal Normal or single Single or paradoxical

24. Aortic Insufficiency- Pathophysiology 1 0 abnormality – LVVO 1 0 abnormality – LVVO Severity of LVVO Severity of LVVO Size of regurgitant orifice Size of regurgitant orifice Diastolic pressure gradient between Ao & LV Diastolic pressure gradient between Ao & LV HR or duration of diastole HR or duration of diastole Compensatory Mechanisms Compensatory Mechanisms LV dilatation & eccentric LVH LV dilatation & eccentric LVH Increased LV diastolic compliance Increased LV diastolic compliance Peripheral vasodilation Peripheral vasodilation

25. LV Volume vs Pressure Overload Feature LVPO (AS) LVVO (MR,AI) LV Volume normalDilated** 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 LVEFnormalincreased

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

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

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

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

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

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

32. Valvular Heart Disease The End