Valvular Heart Disease: Recognition and Management in the Outpatient Setting

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1 Valvular Heart Disease: Recognition and Management in the Outpatient Setting Ian S. Harris UCSF Division of Cardiology Case 1: 80 year old man with a history of hypertension, complaining of exertional chest discomfort and dyspnea. The murmur can be described as: A. An ejection systolic murmur B. A holosystolic murmur C. A decrescendo early diastolic murmur D. A late diastolic rumble Case 1: 80 year old man with a history of hypertension, complaining of exertional chest discomfort and dyspnea. The murmur can be described as: A. An ejection systolic murmur s 4 B. A holosystolic murmur C. A decrescendo early diastolic murmur D. A late diastolic rumble 1

2 Case 2: 30 year old woman with no significant past medical history, in her second trimester of pregnancy. The most important characteristic of this murmur is: A. The fact that it is a grade 1-2 mid-systolic murmur B. The fact that S2 is normally split C. The fact that there are no associated abnormalities on exam D. All of the above Case 2: 30 year old woman with no significant past medical history, in her second trimester of pregnancy. The most important characteristic of this murmur is: A. The fact that it is a grade 1-2 mid-systolic murmur B. The fact that S2 is normally split C. The fact that there are no associated abnormalities on exam D. All of the above Case 3: 35 year old man with a lifelong history of murmur (no details available), asymptomatic, establishing primary care. The diagnosis is: A. Mitral valve prolapse B. Ventricular septal defect C. Bicuspid aortic valve D. It is impossible to tell without an echo 2

3 Case 3: 35 year old man with a lifelong history of murmur (no details available), asymptomatic, establishing primary care. The diagnosis is: A. Mitral valve prolapse B. Ventricular septal defect C. Bicuspid aortic valve D. It is impossible to tell without an echo Case 4: 52 year old woman with a history of hypertension, complaining of exertional dyspnea and orthopnea. Exam: Apical beat 2cm in diameter, located in the 5 th intercostal space, 2cm lateral to MCL. CVP 10 cm. The diagnosis is: A. Aortic stenosis B. Mitral stenosis C. Aortic regurgitation D. Mitral regurgitation Case 4: 52 year old woman with a history of hypertension, complaining of exertional dyspnea and orthopnea. Exam: Apical beat 2cm in diameter, located in the 5 th intercostal space, 2cm lateral to MCL. CVP 10 cm. The diagnosis is: s 3 A. Aortic stenosis B. Mitral stenosis C. Aortic regurgitation D. Mitral regurgitation 3

4 Case 4: 52 year old woman with a history of hypertension, complaining of exertional dyspnea and orthopnea. Exam: Apical beat 2cm in diameter, located in the 5 th intercostal space, 2cm lateral to MCL. CVP 10 cm. The patient needs an echo to: A. Determine the mechanism of mitral regurgitation B. To plan for potential surgery C. Both of the above D. None of the above-the patient does not need an echo Case 4: 52 year old woman with a history of hypertension, complaining of exertional dyspnea and orthopnea. Exam: Apical beat 2cm in diameter, located in the 5 th intercostal space, 2cm lateral to MCL. CVP 10 cm. The patient needs an echo to: A. Determine the mechanism of mitral regurgitation B. To plan for potential surgery C. Both of the above D. None of the above-the patient does not need an echo Suggested evaluation of a cardiac murmur You can hear a lot just by listening. -Yogi Berra Circulation 2006;114;e84-e231 4

5 58 yo woman with progressive dyspnea CC: Increasing exertional shortness of breath for several months. 2 pillow orthopnea, occasional palpitation. PMH: 2 uncomplicated pregnancies at age 18, 22 Medications: none PE: BP = 95/65, HR = 90 irreg irreg, afebrile CVP 10 cm H2O, carotids low volume without bruits Lungs: crackles 1/2 way up bilaterally Cor: sustained right parasternal impulse, irregularly irregular rhythm, variable and loud S1, Opening snap in early diastole, physiologically split S2 with accentuated P2, Grade 2/4 rumbling diastolic murmur, Grade 3/6 holosystolic murmur at R lower sternal border Chest Radiograph Straightening of the left heart border due to LA appendage Elevation of the left mainstem bronchus Retrocardiac density due to LA Enlarged RV Posterior displacement of LA No LV enlargement Echo: Mitral stenosis with a mean gradient of 12 mmhg, MVA = 0.8 cm 2 Moderate to severe TR PA systolic pressure estimated at 70 mmhg. Dilated RV, normal LV Severe biatrial enlargement 5

6 Mitral Stenosis Severity v ΔP = 4v 2 Mitral stenosis: Etiology: 75% Post-inflammatory (ie. rheumatic) Only 50-70% have clear h/o RF Other causes < 10%: Mitral annular calcification SLE or RA Carcinoid Methysergide ingestion Congenital Pathology - important for treatment Commissural fusion Thickening, fibrosis and calcification of the leaflets Thickening, fusion and foreshortening of the chordae tendinae Mitral Stenosis: Indications for treatment Symptoms of dyspnea, exercise intolerance Hemoptysis Pulmonary hypertension Evidence of right heart failure Atrial fibrillation ± embolism 6

7 Mitral Stenosis: Medical Treatment Diuretic + Na restriction Beta-blocker Warfarin if AF present or H/O embolus, Heparin should be considered acutely Secondary prevention for rheumatic fever Up to age 25 or 10 yrs after last episode Older patients with continued exposure to Strep A pharyngitis Pen VK 250 bid or Benzathine PCN G 1.2 m units IM monthly Endocarditis prophylaxis not indicated Summary: Mitral stenosis Medical management with beta-blockers and diuretics may be helpful in mildly symptomatic patient PBMV indicated for most symptomatic patients MV replacement may be required Older pt with extensively calcified valve Mixed MS/MR 47 yr old man referred after episode of VT H/O MVP diagnosed by click and murmur Sudden onset lightheadedness while traveling to NY on business In ER had episodes of sustained VT Echo showed MR, started on amiodarone and returned home Asyx PE: BP 120/60 No evidence of CHF Normal S1, S2, mid-systolic click and late systolic murmur 7

8 Echocardiogram The Roles of Echocardiography How severe is the MR? Multiple criteria apply. The most reliable indicator is ventricular dilation. What is the mechanism for MR? How well compensated is the LV? What is the best way to reduce the MR? Classification of Mitral regurgitation: Organic - Primary pathology of the leaflets Degenerative Rheumatic Endocarditis Congenital (eg. cleft) Functional - Malcoaptation 2 to myocardial process Ischemic Dilated cardiomyopathy Hypertrophic cardiomyopathy 8

9 Physiology of Primary Mitral Regurgitation" LA enlargement" Left ventricular volume overload" Eccentric hypertrophy" LVEF normal to hyperdynamic" Pulmonary hypertension" Acute vs. Chronic" Mitral regurgitation: Treatment Organic mitral valve disease - surgical repair or replacement Degenerative (mitral valve prolapse or myxomatous valve disease) Rheumatic Congenital Functional mitral regurgitation - medical therapy Ischemic Dilated cardiomyopathy MR: indications for surgery Severe MR with: Symptoms of heart failure (NYHA class II-IV) Ventricular dilatation (end-systolic LV dimension > 40 mm) Reduced ejection fraction by echocardiography (EF < 0.60) preserved EF and Afib or PHT preserved LVEF when repair likelihood > 90% Severe LV dysfxn with EF< 0.30, ESD > 55 with primary MR when repair likelihood is high 9

10 Management: ICD placed Surgery now? If not, how often should you echo patient? What medical therapy is appropriate? Frequency of serial echocardiograms is based on MR severity" Severity" Clinical follow-up" ACC/AHA guidelines for Echo" Mild" Yearly" Δ clinical status only" Moderate" Yearly" Yearly or Δ clinical status" Severe" 6-12 mo" 6-12 mo" Management: Followed with yearly echocardiograms Medical therapy Vasodilators only indicated if patient is hypertensive or in the presence of LV dysfunction (if not a surgical candidate) Endocarditis prophylaxis Not routinely indicated Still needed in patients with prior history of endocarditis 10

11 75 yr old woman referred for evaluation prior to colectomy Asymptomatic Walks briskly 4 times/week for 30 minutes PMH: HTN, systolic murmur Meds: HCTZ 25 mg, Benazepril 10 mg PE: BP 135/85, HR 68 reg Carotids: delayed carotid upstroke Lungs: clear S1 nl, S2 single, grade 3/6 mid-late peaking SEM radiating to carotids, S4 Echocardiogram AS: clinical examination Arterial pulse Reduced amplitude Delayed upstroke Shudder Apical impulse Sustained Palpable S4 Displaced late Palpable thrill at base Auscultation S1 normal S2 paradoxically split and finally single Crescendo-decrescendo murmur Typically radiates to both carotids Gallavardin - radiation to apex simulating MR 11

12 Aortic Stenosis: Etiology Congenital Unicuspid Bicuspid Rheumatic Degenerative calcific AS: Adaptive Mechanisms Asymptomatic" Symptoms" Cardinal symptoms Angina Present in 2/3 of adults pts with AS 50% of AS pts with angina have associated CAD In the absence of CAD, angina is associated with: Inadequate LVH and higher wall stress Reduced coronary flow reserve Syncope Inability to increase cardiac output in the face of peripheral vasodilation Decreased cerebral perfusion Dyspnea Julius BK et al. Circulation 1997;103:1522! 12

13 AS: Natural Hx Aortic Stenosis: Echocardiography Confirm diagnosis LV mass and function Associated lesions Aortic dilatation (bicuspid aortic valve) Mitral valve disease (rheumatic patients) Wall motion abnormalities (coronary artery disease) Evaluate severity Peak and mean gradients Aortic valve area Peak gradient 60 mmhg AVA = 0.8 cm2 LVEF normal 13

14 Severity of Aortic Stenosis Mild" Moderate" Severe" Jet Velocity" (m/sec)" Mean Gradient" (mmhg)" < 3" 3-4" > 4" < 25" 25-40" > 40" Aortic Valve Area > 1.5 " " < 1.0 " (cm 2 ) " (critical < 0.75)" Aortic Valve Index (cm 2 /m 2 )" < 0.6" Aortic Stenosis: Indications for Valve Replacement Class" Bonow et al JACC 2006 Should she have AVR before surgery? AS (n=30)" Control (n=60)" HR" Hypotension" 9 (30%)" 10 (17%)" 2.5" Post-op MI" 1" 2" 0.63" Death" 0" 2" 0.95" CHF" 0" 0" 30 pts age >75 Pk AV gradient = 85 mmhg Mean AV gradient = 50 mmhg CAD in 37% Calleja et al AJC

15 Recommendation: Surgery in the pre-operative patient should only be performed if it otherwise would have been indicated. How often should she be seen? How often should echo be done? How rapidly does aortic stenosis progress? Average rate of progression AVA 0.1 cm 2 /yr Peak gradient 7 mmhg/yr Predictors of progression rate: Heavy calcification Renal failure Hypercholesterolemia 15

16 How often should asymptomatic AS pt be followed? Clinical Evaluation! Echo! Mild" q 1-2 yrs" q 3-5 yrs or clinical status" Moderate" q 1 yrs" q 1-2 yrs or clinical status" Severe" q 6-12 mo" q 1 yr or clinical status" Summary: Aortic stenosis No specific medical management Endocarditis prophylaxis no longer recommended Exercise restrictions: Mild - no restrictions Moderate - restrict to low to moderate intensity competitive sports Severe with or without symptoms: no participation in competitive sports Surgery indicated for symptoms Surgery may be considered in the asyx with high risk indicators although these are almost always associated with symptoms 48 year old man with recent episode of endocarditis Rx with antibiotics After 2 weeks of fevers and malaise, blood cultures positive for viridans strep Received 6 weeks of penicillin Denies symptoms except during extreme exertion such as chopping wood PE: BP 145/50, HR 60, afebrile Brisk and collapsing carotid pulses Sustained and displaced apical impulse Soft S1, diminished A2, slightly increased P2 2/6 early SEM, 2/4 holodiastolic murmur, 2/4 diastolic rumble 16

17 Aortic Regurgitation Etiology: Valvular: rheumatic, bicuspid, endocarditis Aortic root disease: Marfan, dissection, syphilis, ankylosing spondylitis, etc History: Acute (shock) or Chronic (dyspnea, CHF) PE: Chronic AR: Wide pulse pressure, soft S1, soft or absent A2, diastolic blowing murmur. Systolic ejection murmur is invariably present and may be loud (> 4/6). Acute AI: short/soft murmur, narrow pulse pressure, tachycardia Tx: Chronic: Valve replacement if syx or asyx with LV dilatation Acute: Surgery, IV vasodilators, No IABP Echocardiogram Like with mitral regurgitation, quantification of AR severity is complex, relying on several parameters. If the left ventricular size is normal, the AR is not severe. Indications for Vasodilator Therapy Patients who are not undergoing aortic valve replacement for severe symptomatic AR because of comorbid conditions (I) Short term therapy for patients with severe AR and severe LV dysfunction scheduled for surgery to improve hemodynamic profile (IIa) Chronic therapy for asymptomatic patients with severe AR with normal LV function (IIb) 17

18 Indications for AVR Symptomatic patient with moderately severe or severe AI (includes NYHA II) (I) Asymptomatic patient LVEF < 0.55 (I) LVEDd > 75 mm (IIa) LVESd > 55 mm (consider use of indexed ESV of 25 mm/m 2 ) (IIa) The 55 Rule Summary Stenotic valvular lesions tend to produce symptoms when they become hemodynamically significant. Compensatory responses tend to be reversible after surgery. Echo indices of severity are objective and straightforward. Timing of surgery hinges on identification of symptoms Regurgitant valvular lesions are usually tolerated well when they progress gradually Irreversible adaptations to hemodynamically significant lesions may occur in the absence of symptoms. Echo indices of severity tend to be subjective, multiple criteria apply. Timing of surgery hinges on identification of symptoms and of signs of maladaptive compensation 18

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