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Key Features

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

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

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Clinical Findings

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  • 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 preservedsystolic function

  • Left ventricle is more severely affectedthan the right ventricle, butthe right ventricle is also affected in most cases resulting insigns and symptoms consistent with biventricular congestion

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Diagnosis

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  • Electrocardiography

    • Demonstrates marked right and left atrial enlargementwith normal ventricular voltages

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

  • Echocardiography

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

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

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Treatment

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  • Anticongestive therapy is used for symptomatic relief

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