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Heart Valve Disease for ABIM

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Heart Valve Disease for the American Board of Internal Medicine Exam
Types of Heart Valve Disease
Heart valve disease includes stenosis and regurgitation of the four heart valves: aortic, mitral, tricuspid, and pulmonic. The two most commonly affected valves are the aortic and mitral valves. Valve disease may result in heart failure, arrhythmias, thromboembolism, and other complications. Each valve condition has unique clinical, diagnostic, and management features.
Aortic Stenosis
aortic valve stenosis
  • Etiology:
    • Calcific Degeneration: Most common in older adults due to progressive calcification.
    • Bicuspid Aortic Valve: Congenital condition leading to premature calcification, often manifesting in younger patients.
    • Rheumatic Fever: Rare in developed countries, typically affects multiple valves.
  • Pathophysiology: Stenosis causes left ventricular (LV) outflow obstruction, leading to increased LV pressure and hypertrophy.
  • Clinical Presentation:
    • Dyspnea on exertion, angina, and syncope are classic symptoms.
    • Heart Sound: Systolic ejection murmur best heard at the right second intercostal space, radiating to the carotids; crescendo-decrescendo quality.
    • Pulsus Parvus et Tardus: Weak and delayed carotid pulse.
  • Diagnosis:
    • Echocardiogram: Confirms stenosis severity and LV hypertrophy.
    • ECG: May show LV hypertrophy.
    • Cardiac Catheterization: Used if noninvasive testing is inconclusive.
  • Management:
    • Symptomatic Patients: Aortic valve replacement (AVR) is recommended, either surgical or transcatheter (TAVR) in select patients.
    • Medical Therapy: Limited to symptom management; avoid vasodilators due to risk of hypotension.
Aortic Regurgitation
  • Etiology:
    • Valve Disease: Bicuspid aortic valve, infective endocarditis, or rheumatic disease.
    • Aortic Root Disease: Aortic dissection, Marfan syndrome, or other connective tissue disorders.
  • Pathophysiology: Regurgitation causes volume overload in the LV, leading to eccentric hypertrophy and progressive LV dilation.
  • Clinical Presentation:
    • Dyspnea and fatigue, especially on exertion.
    • Heart Sound: Diastolic decrescendo murmur best heard at the left sternal border.
    • Widened Pulse Pressure: Bounding pulses and findings such as Quincke’s pulse (nailbed capillary pulsation) and Corrigan’s pulse (bounding carotid pulse).
  • Diagnosis:
    • Echocardiogram: Evaluates severity of regurgitation and LV function.
    • Chest X-Ray: May show LV enlargement.
    • MRI/CT: Used to assess aortic root disease if needed.
  • Management:
    • Symptomatic Patients or those with LV dysfunction: Surgical aortic valve replacement.
    • Medical Management: Vasodilators (e.g., ACE inhibitors) may help manage symptoms if surgery is not immediate.
Mitral Stenosis
  • Etiology:
    • Rheumatic Heart Disease: Most common cause globally, leading to thickening and calcification of the mitral leaflets.
    • Rarely, congenital mitral stenosis.
  • Pathophysiology: Stenosis impedes blood flow from the left atrium to the LV, causing increased left atrial pressure, atrial dilation, and risk of atrial fibrillation (AF).
  • Clinical Presentation:
    • Dyspnea on exertion, orthopnea, paroxysmal nocturnal dyspnea (PND).
    • Heart Sound: Low-pitched, rumbling diastolic murmur best heard at the apex with the bell, often preceded by an opening snap.
    • Complications: Increased risk of AF, pulmonary hypertension, and thromboembolism.
  • Diagnosis:
    • Echocardiogram: Confirms stenosis severity and left atrial size.
    • ECG: May show left atrial enlargement or AF.
    • Chest X-Ray: Left atrial enlargement and pulmonary vascular congestion.
  • Management:
    • Percutaneous Mitral Balloon Valvotomy: Treatment of choice for severe, symptomatic stenosis.
    • Medical Therapy: Beta-blockers or calcium channel blockers to reduce heart rate, anticoagulation if AF is present.
Mitral Regurgitation
  • Etiology:
    • Primary: Mitral valve prolapse (MVP), infective endocarditis, or rheumatic disease.
    • Secondary: LV dilation from ischemic or non-ischemic cardiomyopathy.
  • Pathophysiology: Regurgitation causes volume overload, leading to left atrial dilation, LV enlargement, and eventual heart failure.
  • Clinical Presentation:
    • Dyspnea and fatigue with exertion.
    • Heart Sound: Holosystolic murmur best heard at the apex, radiating to the axilla.
  • Diagnosis:
    • Echocardiogram: Primary tool to evaluate regurgitation severity and LV function.
    • Chest X-Ray: May show left atrial and LV enlargement.
  • Management:
    • Surgical Mitral Valve Repair or Replacement: Indicated in symptomatic patients or those with LV dysfunction.
    • Medical Therapy: ACE inhibitors or beta-blockers for symptom relief in patients awaiting surgery.
Tricuspid and Pulmonic Valve Disease
  • Tricuspid Regurgitation:
    • Often secondary to right ventricular (RV) dilation from pulmonary hypertension, left-sided heart disease, or RV infarction.
    • Clinical findings include a holosystolic murmur at the left sternal border, which increases with inspiration (Carvallo’s sign).
    • Managed with diuretics for volume control; surgery is reserved for severe, symptomatic cases.
  • Pulmonic Stenosis:
    • Usually congenital and presents with a systolic murmur at the left upper sternal border.
    • Treated with balloon valvuloplasty if symptomatic or severe.
  • Pulmonic Regurgitation:
    • Often due to pulmonary hypertension.
    • Typically asymptomatic and managed by treating the underlying cause.
Key Points
  • Aortic Stenosis:
    • Presents with dyspnea, angina, and syncope; diagnosed via echocardiography.
    • Managed with surgical or transcatheter aortic valve replacement for symptomatic patients.
  • Aortic Regurgitation:
    • Symptoms include dyspnea and fatigue with findings of widened pulse pressure.
    • Surgical replacement indicated for symptomatic patients or those with LV dysfunction.
  • Mitral Stenosis:
    • Commonly caused by rheumatic heart disease; presents with dyspnea and low-pitched diastolic murmur.
    • Treated with percutaneous mitral balloon valvotomy or rate control and anticoagulation if AF is present.
  • Mitral Regurgitation:
    • Presents with exertional dyspnea and holosystolic murmur; managed with valve repair or replacement if symptomatic or if LV function is impaired.
  • Tricuspid and Pulmonic Valve Disease:
    • Tricuspid regurgitation commonly secondary to RV dilation; treated with diuretics and surgery in severe cases.
    • Pulmonic stenosis often congenital and treated with balloon valvuloplasty if symptomatic.

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