1.Valvular Heart Disease: An Update in Management Speaker: Prof.Malik Tariq Mahmood Case: An active 75 yo farmer comes to your office after experiencing a fainting spell while baling hay. The episode occurred without warning and he had no symptoms following the episode. However, on close questioning he admits to some breathlessness and vague chest heaviness with his usual heavy exertion over the past few months and a very unwelcome tendency to want to slow down which he reluctantly attributed to his age. He has been healthy all his life, doesnt smoke and has not seen a doctor in 30 years. He served in the army in 1942; no abnormalities were reported during his induction physical Physical Exam: Robust looking older man with a laceration on his forehead from falling on the handle of his pitchfork. BP 135/90 P 68 bpm, regular RR-12 T-98.6 F JVP 6 cm with normal a and v waves Carotids: Difficult to palpate, delayed upstroke Lungs: Clear Heart: Palpation: Palpable thrill over the mid LSB. PMI 5 ICS, 2 cm lateral to the MCL. Palpable presystolic impulse followed by a sustained ventricular lift. Auscultation: Loud S4. S1 is normal. A single S2 (P2) is heard at the upper left sternal border but no S2 is heard at the lower left sternal border. There is a 4/6 systolic ejection murmur (crescendo-decrescendo) heard best at the R 2nd interspace but radiating widely to the LSB, and to the neck. No diastolic murmurs. Abdomen and extremities are unremarkable. Aortic Stenosis: Aortic Stenosis: Etiology: Congenital bicuspid aortic valve Rheumatic aortic valve disease Calcific (senile) aortic stenosis Aortic Stenosis Physiologic Principles-Natural History

2. Normal aortic valve area is 3.0 - 4.0 cm2 Circulation affected when valve area is reduced by ~ 75% (i.e. 0.75 - 1.0 cm2) valve area (cm sq) mean gradient (mm Hg)* Mild > 1.5 < 25 Moderate 1.0 - 1.5 25 - 50 Severe < 0.75 > 50 Aortic Stenosis Physiologic Principles-Natural History: Primary adaptation is concentric hypertrophy Latent phase usually lasts decades Risk of sudden death is very low during this phase Rate of progression ranges from 0-0.3 cm2/yr. (average rate is 0.12 cm2/yr) 50% of patients with severe AS do not progress Cannot predict who will Key Physical Findings in Severe Aortic Stenosis: Carotid impulse: parvus et tardus JVP: Prominent a wave Heart: Systolic thrill Palpable presystolic impulse (S4) Sustained apical systolic impulse S4 Coarse late peaking systolic ejection murmur (may radiate to neck and/or LSB) Attenuated/absent aortic component of S2 Natural History of Aortic Stenosis: Long asymptomatic latent period Cardinal symptoms of severe aortic stenosis Dyspnea Angina Syncope Sudden death Left ventricular dilatation and contractile failure Endocarditis Arrhythmias Ventricular tachycardia Conduction system disease Atrial fibrillation Natural History of AS: 3. Aortic Stenosis Physiologic Principles-Natural History: Once symptoms develop, average survival is 2-3 yrs With LV systolic dysfunction, there may be increased risk of sudden death and permanent LV dysfunction Ross J Jr, Braunwald E: Aortic stenosis. Circulation 38[Suppl V]:61, 1968 Aortic Stenosis Management Guidelines: Initial Diagnostic Testing: Lipids, renal fxn, Ca, P---all patients CXR, ECG, Echocardiography---all patients Cardiac catheterization with angiography If clinical and echo data are discordant To assess coronary circulation prior to surgery Aortic Stenosis Management Guidelines: Initial Diagnostic Testing (cont.) Treadmill stress testing Dangerous in symptomatic pts Not useful for dx of CAD May be used to assess functional significance of severe AS in pts who deny symptoms (e.g. bp response) Aortic Stenosis Management Guidelines: Scheduled Follow-up: 4. office intervalecho interval Mild AS 12 mos 5 yrs Moderate AS 6 mos 2 yrs Severe AS 6 mos 1 yr Aortic Stenosis Management Guidelines: Low Gradient AS Special case Minimal valve mobility and low cardiac output Calculated valve area is small but pressure gradient is also small Functional vs. fixed AS? Consider dobutamine stress test (DSE) to clarify ACC Classification of Recommendations: Class I: Conditions for which there is evidence and/or general agreement that a given procedure or treatment is useful and effective. Class II: Conditions for which there is conflicting evidence and/or a divergence of opinionabout the usefulness/efficacy of a procedure or treatment. IIa. Weight of evidence/opinion is in favor of usefulness/efficacy IIb. Usefulness/efficacy is less well established by evidence/opinion. Class III: Conditions for which there is evidence and/or general agreement that the procedure/treatment is not useful/effective, and in some cases may be harmful. Aortic Stenosis Management Guidelines: Recommendations for AVR Class I Severe AS and symptoms Severe AS (with or without sxs) and need for CABG, other valve replacement or aortic surgery Class IIa Moderate AS and need for other cardiac surgery Asymptomatic severe AS and diminished LVEF or hypotensive response to exercise Aortic Stenosis Management Guidelines: Recommendations for AVR (cont.) Class IIb Asymptomatic AS and VT, severe LVH (>15mm) or valve area 60 mmHg Pathophysiology of Chronic Aortic Regurgitation: Slowly progressive diastolic volume overload Augmented stroke volume with rapid runoff Increased systolic pressure with low diastolic pressure: wide pulse pressure Progressive left ventricular dilation, some hypertrophy Increased diastolic compliance with maintenance of normal diastolic pressures initially Late systolic failure with reduced ejection fraction and CHF 7. Additional Testing: ECG: LVH with massive voltage in the lateral precordial leads (V4-V6) Chest X-Ray: Large heart, predominant left ventricular enlargement. No congestive heart failure. Echo: Marked left ventricular dilation, estimated EF 65%. The end diastolic dimension is 65 mm and the end diastolic dimension is 55 mm. Aortic valve: bicuspid and thickened. Doppler: Severe aortic regurgitation. The aorta is slightly enlarged (4.2 mm). Acute Aortic Regurgitation: Sudden diastolic volume overload without LV dilation: - Acute elevation in left ventricular diastolic pressure pulmonary edema - Acute LV systolic failure hypotension Provide inotropic support, vasodilator therapy if tolerated, urgent valve replacement Natural History of Chronic Aortic Regurgitation: Long asymptomatic phase; may be decades long. Left ventricular systolic dysfunction ( decline in EF) NOTE!! LV dysfunction may occur in the absence of symptoms Symptoms associated with LV dysfunction: - Exercise intolerance - Dyspnea on exertion Angina (rare) Sudden death (rare) Natural history of aortic regurgitation: 8. Aortic Regurgitation Physiologic Principles-Natural History: LV faces combined pressure and volume load Primary adaptation is dilatation (eccentric hypertrophy) Since this adaptation takes time, AR classified as acute or chronic Acute AR results in sudden increase in LVEDP >>> pulmonary edema and cardiogenic shock Aortic Regurgitation Physiologic Principles-Natural History: Latent phase of AR, like AS, may last decades Decompensation when LV systolic function begins to fail Progressive LV dilatation occurs Spherical geometry develops Initially this is reversible LV systolic function and ESD are the most important predictors of postop survival and LV function Aortic Regurgitation Physiologic Principles-Natural History In asymptomatic pts with severe AS and nl LV systolic function, progression is slow 4.3%/yr develop symptoms of LV systolic dysfunction 1.3%/yr progress to LV dysfunction without symptoms pooled data from 7 series. 490 pts with mean follow-up of 6.4 yrs Aortic Regurgitation Management Guidelines: Scheduled Follow-up (office and echo) Severe AR without symptoms q 4-12 month depending on pace of change and current LV ESD/EDD Moderate AR without symptoms 1st follow-up in 2-3 months to establish pace, then ~ q 12 months 9. Aortic Regurgitation Management Guidelines: Vasodilator Therapy Expected to afterload, stroke volume and regurgitant volume Hemodynamic benefit shown with hydralazine and nifedipine, less consistent results with ACEi Improvement in clinical outcomes in trial of LA nifedipine vs. digoxin (need for AVR in 143 pts followed for 6 yrs--- 15% vs 34%) Dose titrated to achieve in SBP, not normalization Aortic Regurgitation Management Guidelines: Vasodilator Therapy Indications Class I Severe AR with symptoms or severe LV dilatation but contraindications to surgery Severe AR without symptoms but LV dilatation and elevated SBP Any degree of AR with hypertension Persistent LV systolic dysfunction s/p AVR (ACEi) Short term therapy prior to AVR Aortic Regurgitation Management Guidelines: Vasodilator Therapy Indications Class III Mild to mod AR without sxs and nl LV function In lieu of AVR in pts without contraindications Aortic Regurgitation Management Guidelines: Recommendations for AVR (chronic severe AR) Class I NYHA functional class III or IV sxs NYHA functional class II sxs and progressive LV dilatation or declining LVEF on serial studies CCS class II angina Mild or moderate reduction in EF (25-50%) Need for CABG or surgery on other valves Aortic Regurgitation Management Guidelines: Class IIa NYHA class II sxs with nl LVEF (>50%) with stable EF, LV size and exercise tolerance Asymptomatic pts with nl LVEF but severe LV dilatation (ESD > 55 mm or EDD > 75 mm) Class IIb LVEF < 25% 10. Asymptomatic pts with nl LVEF and progressive LV dilatation with ESD 50- 55 Valvular Heart Disease Mitral Stenosis A 75 year old woman with loud first heart sound and mid-diastolic murmur: Chronic dyspnea Class 2/4 Fatigue Recent orthopnea/pnd Nocturnal palpitation Pedal edema Mitral Stenosis: Etiology Symptoms Physical Exam Severity Natural history Timing of Surgery Mitral Stenosis: Etiology: Primarily a result of rheumatic fever (~ 99% of MVs @ surgery show rheumatic damage ) Scarring & fusion of valve apparatus Rarely congenital Pure or predominant MS occurs in approximately 40% of all patients with rheumatic heart disease Two-thirds of all patients with MS are female. Mitral Stenosis: Pathophysiology Normal valve area: 4-6 cm2 Mild mitral stenosis: MVA 1.5-2.5 cm2 Minimal symptoms Mod mitral stenosis MVA 1.0-1.5 cm2 usually does not produce symptoms at rest Severe mitral stenosis MVA < 1.0 cm2 Mitral Stenosis: Symptoms: Fatigue Palpitations Cough SOB Left sided failure Orthopnea PND 11. Palpitation Afib Systemic embolism Pulmonary infection Hemoptysis Right sided failure Hepatic Congestion Edema Worsened by conditions that cardiac output. Exertion,fever, anemia, tachycardia, Afib, intercourse, pregnancy, thyrotoxicosis Recognizing Mitral Stenosis Palpation: Small volume pulse Tapping apex-palpable S1 +/- palpable opening snap (OS) RV lift Palpable S2 ECG: LAE, AFIB, RVH, RAD Auscultation: Loud S1- as loud as S2 in aortic area A2 to OS interval inversely proportional to severity Diastolic rumble: length proportional to severity In severe MS with low flow- S1, OS & rumble may be inaudible Mitral Stenosis: Physical Exam First heart sound (S1) is accentuated and snapping Opening snap (OS) after aortic valve closure Low pitch diastolic rumble at the apex Pre-systolic accentuation (esp. if in sinus rhythm) Common Murmurs and Timing (click on murmur to play) Systolic Murmurs Aortic stenosis Mitral insufficiency Mitral valve prolapse Tricuspid insufficiency Diastolic Murmurs Aortic insufficiency Mitral stenosis Auscultation- Timing of A2 to OS Interval: Width of A2-OS inversely correlates with severity 12. The more severe the MS the higher the LAP the earlirthe LV pressure falls below LAP and the MV opens Mitral Stenosis: Natural History: Progressive, lifelong disease, Usually slow & stable in the early years. Progressive acceleration in the later years 20-40 year latency from rheumatic fever to symptom onset. Additional 10 years before disabling symptoms Mitral Stenosis: Complications: Atrial dysrrhythmias Systemic embolization (10-25%) Risk of embolization is related to, age, presence of atrial fibrillation, previous embolic events Congestive heart failure Pulmonary infarcts (result of severe CHF) Hemoptysis Massive: 20 to ruptured bronchial veins (pulm HTN) Streaking/pink froth: pulmonary edema, or infection Endocarditis Pulmonary infections Mitral Stenosis: EKG: LAE RVH Premature contractions Atrial flutter and/or fibrillation freq. in pts with mod-severe MS for several years A fib develops in 30% to 40% of pts w/symptoms A 75 year old woman with loud first heart sound and mid-diastolic murmer: 13. Mitral Stenosis: Role of Echocardiography: Diagnosis of Mitral Stenosis Assessment of hemodynamic severity mean gradient, mitral valve area, pulmonary artery pressure Assessment of right ventricular size and function. Assessment of valve morphology to determine suitability for percutaneous mitral balloon valvuloplasty Diagnosis and assessment of concomitant valvular lesions Reevaluation of patients with known MS with changing symptoms or signs. F/U of asymptomatic patients with mod-severe MS Mitral Stenosis:Therapy: 14. Medical Diuretics for LHF/RHF Digitalis/Beta blockers/CCB: Rate control in A Fib Anticoagulation: In A Fib Endocarditis prophylaxis Balloon valvuloplasty Effective long term improvement Recommendations for Mitral Valve Repair for Mitral Stenosis: ACC/AHA Class I Patients with NYHA functional Class III-IV symptoms, moderate or severe MS (mitral valve area