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Key Features

  • Underlying rheumatic heart disease in almost all patients (although history of rheumatic fever is often absent)

Clinical Findings

  • An opening snap following A2 due to stiff mitral valve

  • Interval between opening snap and aortic closure sound is long when the left atrial pressure is low but shortens as left atrial pressure rises and approaches the aortic diastolic pressure

  • Low-pitched rumble at apex with patient in left lateral position, increased by brief exercise

  • Moderate stenosis (valve area 1.5–1.0 cm2): exertional dyspnea and fatigue common, especially with tachycardia

  • Severe stenosis (valve area < 1.0 cm2): pulmonary congestion at rest, with dyspnea, fatigue, right-sided heart failure, orthopnea, and occasional hemoptysis

  • Sudden increase in heart rate may precipitate pulmonary edema

  • Paroxysmal or chronic atrial fibrillation develops in ~50–80%, may precipitate dyspnea or pulmonary edema

Diagnosis

  • ECG typically shows left atrial abnormality and, often, atrial fibrillation

  • Doppler echocardiography confirms diagnosis and quantifies severity by assigning 1–4 points to each of four observed parameters, with 1 being the least involvement and 4 the greatest

    • Mitral leaflet thickening

    • Mitral leaflet mobility

    • Submitral scarring

    • Commissural calcium

  • Cardiac catheterization to detect valve, coronary, or myocardial disease, usually done only after a decision to intervene has been made

Treatment

  • Control heart rate

  • Attempt conversion of atrial fibrillation

  • Once atrial fibrillation occurs, provide lifelong anticoagulation with warfarin, even if sinus rhythm is restored

  • Intervention to relieve stenosis indicated for symptoms (eg, pulmonary edema, decline in exercise capacity) or evidence of pulmonary hypertension

  • Percutaneous balloon valvuloplasty

    • Low mortality rate (< 0.5%)

    • Low morbidity rate (3–5%)

    • Can be repeated if the morphology of the valve remains suitable

    • Not effective when bioprosthetic valve stenosis occurs

  • Surgical valve replacement is done in combined stenosis and regurgitation or when the mitral valve is significantly distorted and calcified

  • Operative mortality rate is ~1–3%

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