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Key Features

  • Rare; accounts for < 5% of all cases of cardiomyopathy

  • Cause is usually idiopathic but can be familial or secondary to an infiltrative process (eg, amyloidosis)

Clinical Findings

  • Exercise intolerance

  • Fatigue

  • Chest pain

  • Orthopnea

  • Prominent S4

  • Jugular venous distention

  • Signs of congestive heart failure as a consequence of diastolic dysfunction in the setting of preserved systolic function

  • Left ventricle is more severely affected than the right ventricle, but the right ventricle is also affected in most cases resulting in signs and symptoms consistent with biventricular congestion


  • Electrocardiography

    • Demonstrates marked right and left atrial enlargement with normal ventricular voltages

    • ST-T–wave abnormalities including a prolonged QTc interval may be present

  • Echocardiography

    • Presence of normal-sized ventricles with normal systolic function and massively dilated atria confirm diagnosis

  • Cardiac MRI is useful in ruling out pericardial abnormalities (restrictive or constrictive pericarditis) and infiltrative disorders


  • Anticongestive therapy is used for symptomatic relief

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