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Valvular Heart Disease Internal Medicine Board Review

Niels Engberding, MD, FACC, FESC

Assistant Professor of Medicine

Division of Cardiology

Emory University of School of Medicine

Case #1

• 78 y/o female is admitted to hospital with pulmonary edema

• She has had 3 months of progressive dyspnea

• On exam she has a late-peaking systolic murmur with a single heart sound

• An echocardiogram reveals a heavily calcified/stenotic aortic valve (area of 0.8 cm2)

Which of the following is appropriate? • 1) ACE inhibitor therapy for heart failure

• 2) Coronary angiography followed by aortic valve replacement

• 3) Exercise thallium to evaluate for ischemia

• 4) Balloon valvuloplasty

Major types of aortic valve stenosis

Baumgartner et al. JASE 2009; 22(1):1 Braunwald’s Heart Disease, 10th ed. 2015; Elsevier

Aortic Stenosis

• Congenital – Bicuspid Aortic Valve • Associated with dilation of the aorta

• Can present with aortic regurgitation

• Typically presents in 40’s-50’s

• Acquired • Rheumatic – usually with coexisting mitral disease

• “Senile” – age-related calcific degeneration

• Typically presents > 65 years

Senile Aortic Stenosis

• Most common cause of Aortic Valve replacement in the U.S.

• Incidence is 2% among people over the age of 65 – true AS not Aortic sclerosis.

• Pathophysiology may be related to atherosclerosis.

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Pathophysiology

Lilliy’s Pathophysiology of Heart Disease, 4th ed. 2007; Lippincott W &W

AS - History

• Asymptomatic – Small increased risk of sudden cardiac death

• Symptoms • Angina

• Syncope

• Heart Failure

Physical Examination

• Narrow Pulse Pressure

• Delayed Carotid Pulses

• Pulses (Parvus et Tardus)

• S4

• Late-peaking crescendo-decrescendo murmur

• Single second heart sound

Ross et al. Circulation 1968; 38:61

Outcomes of aortic stenosis

Management for Severe AS

• Asymptomatic • Consider exercise restriction

• Avoid vasodilators

• Caution with non-cardiac surgery

• Symptomatic - Surgery

Nishimura et al. JACC 2014; 63(22):2438

Main indications for aortic valve replacement

Severe AS

symptomatic asymptomatic

LVEF < 50%

Other cardiac surgery

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Signs of Severe AS

• EKG – LVH

• Echo • Calcified Leaflets with reduced valve opening

• LVH

• Mean gradient of greater than 40mmHg

• Calculated valve area of less than 1.0 cm2

• Cath • Confirm gradient

• Assess coronaries in patients at risk for CAD

Which of the following is appropriate? • 1) ACE inhibitor therapy for heart failure

• 2) Coronary angiography followed by aortic valve replacement

• 3) Exercise thallium to evaluate for ischemia

• 4) Balloon valvuloplasty

CASE #2

• A 42 y/o man with a history of a bicuspid aortic valve and a history of treated endocarditis.

• Echocardiography reveals severe aortic regurgitation

• He is asymptomatic.

Which of the following is not appropriate • 1) Nifedipine if left ventricular function is normal

• 2) Aortic valve replacement if the left ventricular end-diastolic diameter is >75 mm

• 3) All patients with severe chronic aortic regurgitation regardless of symptoms should undergo aortic valve replacement

Chronic Aortic Regurgitation - Etiology • Rheumatic

• Dilated Aortic Root

• Bicuspid Aortic Valve

• HTN

• Previous Endocarditis

Chronic Aortic Regurgitation - other etiologies

Ankylosing spondylitis

Reiter’s syndrome

Syphilis

Ehlers – Danlos

Osteogenesis imperfecta

Pseudoxanthoma elasticum

Marfan’s

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AR - Pathophysiology

Braunwald’s Heart Disease, 10th ed. 2015; Elsevier

AR – Hemodynamics

Braunwald’s Heart Disease, 10th ed. 2015; Elsevier

Normal Severe acute AR

Chronic compensated AR Chronic decompensated AR After AVR

Chronic AR – Physical Exam

• Widened Pulse Pressure

• Bounding pulses

• Austin-Flint murmur - a soft mid-diastolic murmur heard at the apex

• Displaced apical pulse

Timing of Surgery – the importance of symptoms

Braunwald’s Heart Disease, 10th ed. 2015; Elsevier from: Dujardin et al. Circulation 1999;99:1851

Chronic AR - Indications for surgery

Severe AR

Nishimura et al. JACC 2014; 63(22):2438

Chronic AR- Medical Therapy

• Asymptomatic patient – Vasodilator therapy.

• ACE-I or Nifedipine – Nifedipine is the only vasodilator proven in a clinical trial to slow progression of AR. A recent long-term trial did not show benefit in preventing surgery.

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Acute AR

• Usually a medical and surgical emergency

• Etiologies • Aortic dissection

• Endocarditis

• Trauma

Which of the following is not appropriate • 1) Nifedipine if left ventricular function is normal

• 2) Aortic valve replacement if the left ventricular end-diastolic diameter is >75 mm

• 3) All patients with severe chronic aortic regurgitation regardless of symptoms should undergo aortic valve replacement

CASE #3

• A 48 y/o recent immigrant from the Philippines presents with dyspnea and hemoptysis.

• No significant past medical history.

• On examination, there is a loud S1 and a low pitched sound in diastole

Which is true about the opening snap of mitral stenosis 1) A short aortic closure sound (A2) to opening snap interval indicates severe stenosis

2) It cannot be appreciated if you own an iPhone

3) It is best appreciated in early systole

Physical Exam of MS

• History – Dyspnea, PND, Fatigue, Hemoptysis.

• Exam – Loud S1, Opening snap, Diastolic rumble, Loud P2.

• Severe MS – Short A2-opening snap interval.

Pathophysiology

Lilliy’s Pathophysiology of Heart Disease, 4th ed. 2007; Lippincott W &W

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Braunwald’s Heart Disease, 10th ed. 2015; Elsevier

Diastolic LA – LV pressure gradient Mitral Stenosis - EKG

• Atrial fibrillation is common

• Left atrial abnormality

• Rightward Axis/ RVH

Management of Mitral Stenosis

• Atrial fibrillation – anticoagulation, Rate Control with digoxin, B-blockers, diltiazem, verapamil

• Surgical intervention if symptoms with heavy exertion (NYHA Class II) and moderate to severe stenosis

Balloon valvuloplasty is alternative for pliable, noncalcified valve with minimal MR and no atrial clot

Which is true about the opening snap of mitral stenosis • 1) A short aortic closure sound (A2) to opening

snap interval indicates severe stenosis

• 2) It cannot be appreciated if you own an iPhone

• 3) It is best appreciated in early systole

In chronic mitral regurgitation which is not true? • 1) ACE inhibitor therapy can decrease the degree of

valve regurgitation acutely

• 2) Severe mitral regurgitation requires surgery if the LVESD is greater than 45mm

• 3) The operative approach for MR should always be valve replacement

• 4) Patients with mitral valve prolapse without regurgitation do not need SBE prophylaxis

Pathophysiology

Acute MR Chronic MR

Lilliy’s Pathophysiology of Heart Disease, 4th ed. 2007; Lippincott W &W

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Chronic MR - Etiologies

Lilliy’s Pathophysiology of Heart Disease, 4th ed. 2007; Lippincott W &W

Chronic MR

History – fatigue and dyspnea

Exam – hyperdynamic PMI, systolic murmur (late, holo, or early).

Diastolic rumble can be heard in severe MR.

EKG – left atrial enlargement, LVH

MITRAL REGURGITATION - THERAPY

• Severe • surgery prior to decline in LVEF • repair or replacement if

LVESD > 40 mm or LVEF < 60%

•Repair is preferred over MVR when possible

•Acute afterload reduction may decrease severity of MR

MITRAL VALVE PROLAPSE

Generally benign – women (20-50 y.o.) – mild regurgitation

Often progressive – men (40 – 70 y.o.) – myxomatous mitral disease, chordal rupture

Midsystolic click in MVP

Braunwald. Essential Atlas of Heart Diseases, 1997; Current Medicine

• Standing • Valsalva • Amyl nitrate

• Squatting • Handgrip

In chronic mitral regurgitation which is not true? • 1) ACE inhibitor therapy can decrease the degree of

valve regurgitation acutely

• 2) Severe mitral regurgitation requires surgery if the LVESD is greater than 45mm

• 3) The operative approach for MR should always be valve replacement

• 4) Patients with mitral valve prolapse without regurgitation do not need SBE prophylaxis

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New Guidelines for Endocarditis Prophylaxis

• Dental Procedures

• Procedures of respiratory tract that involve biopsy or incision of mucosa

• Not for GU or GI procedures unless active infection

• Prosthetic heart valves

• Prior endocarditis

• Heart Transplant patients

• Congenital Heart Disease

Procedures Patients

Hard Question #1

During a patient exam, you hear a mid-diastolic rumble at the apex and a diastolic decrescendo-blowing murmur at the LLSB. He has mild dyspnea on effort. The murmurs have been present for years.

Which of the following features on examination suggests the apical diastolic murmur is Austin Flint and not Mitral Stenosis?

Answers

• A) Increased S1

• B) Atrial Fibrillation

• C) Presystolic accentuation of rumble

• D) BP 160/50

• E) Opening Snap

Answers

• A) Increased S1

• B) Atrial Fibrillation

• C) Presystolic accentuation of rumble

• D) BP 160/50

• E) Opening Snap

Hard Question #2

• Which of the following is the most reliable finding on physical examination to indicate that a severe degree of Aortic Regurgitation is present?

• A) Wide arterial pulse pressure

• B) Lateral displacement of PMI

• C) Grade 2 mid-to-late diastolic apical murmur

• D) Absent A2

• E) Loud AR murmur

Hard Question #2

• Which of the following is the most reliable finding on physical examination to indicate that a severe degree of Aortic Regurgitation is present?

• A) Wide arterial pulse pressure

• B) Lateral displacement of PMI

• C) Grade 2 mid-to-late diastolic apical murmur

• D) Absent A2

• E) Loud AR murmur

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Hard Question #3

• A 52 y/o male comes to clinic with moderate dyspnea on exertion. On examination he has n S3 and II/VI systolic ejection murmur.

• An echo shows and LVEF of 15% and aortic stenosis. The mean aortic valve gradient is 10mmHg and the calculated aortic valve area is 0.7

Answers

• A) Refer for emergent aortic valve replacement

• B) Refer for coronary angiography and valvuloplasty

• C) Order a dobutamine stress echocardiogram

• D) Order a transesophageal echocardiogram to review

Answers

• A) Refer for emergent aortic valve replacement

• B) Refer for coronary angiography and valvuloplasty

• C) Order a dobutamine stress echocardiogram

• D) Order a transesophageal echocardiogram to review