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KEY FEATURES

ESSENTIALS OF DIAGNOSIS

  • Symptoms and signs of left or right heart failure

  • Normal left and right ventricular size and systolic function with dilated atria

  • Abnormalities of diastolic ventricular function suggestive of reduced ventricular compliance

  • Increased ventricular filling pressure (left > right) and reduced cardiac output

GENERAL CONSIDERATIONS

  • Rare disease of the myocardium accounting for approximately 5% of all cases of primary cardiomyopathies

  • Three major causes include cardiac amyloidosis, cardiac sarcoidosis, and cardiac hemochromatosis. Other causes include idiopathic, endomyocardial fibrosis, eosinophilic endomyocarditis, metastatic malignancy, carcinoid heart disease, mediastinal radiation, and following heart transplantation

  • Disease course varies depending on the pathology

  • Important to distinguish from constrictive pericarditis, which may have similar clinical and hemodynamic findings

  • Pathophysiology involves abnormal myocardial stiffness leading to a precipitous rise in ventricular diastolic filling pressures with a reduction in left ventricular filling volume and cardiac output

CLINICAL PRESENTATION

SYMPTOMS AND SIGNS

  • Gradually progressive dyspnea and exercise intolerance

  • Fatigue and weakness

  • Chest pain

  • Palpitations

  • Dizziness and syncope

  • Increasing abdominal girth (ascites)

  • One-third of patients may present with thromboembolic events

PHYSICAL EXAM FINDINGS

  • Reduced pulse pressure

  • Tachycardia

  • Increased jugular venous pressure with rapid X and Y descents

  • Kussmaul sign in the jugular venous pulse (increase with inspiration)

  • Loud S3

  • Murmurs of mitral and tricuspid regurgitation may be present

  • Evidence of abdominal ascites

  • Liver may be palpable and pulsatile

  • Peripheral edema

  • Decreased breath sounds due to pleural effusions

  • Pulmonary rales may be heard

DIFFERENTIAL DIAGNOSIS

  • Dilated cardiomyopathy with restrictive physiology

  • Hypertrophic cardiomyopathy

  • Constrictive pericarditis

DIAGNOSTIC EVALUATION

LABORATORY TESTS

  • Complete blood count with peripheral smear: to rule out eosinophilia

  • Serum iron concentrations, percent saturation of total iron binding capacity, and serum ferritin levels to exclude hemochromatosis

  • Serum protein electrophoresis and serum and urine immunofixation to exclude light-chain deposition disease

ELECTROCARDIOGRAPHY

  • Sinus rhythm, sinus tachycardia, or atrial arrhythmias may be present

  • Varying degrees of atrioventricular block may be present

  • Low QRS voltage

  • Nonspecific ST-segment and T-wave abnormalities

IMAGING STUDIES

  • Chest x-ray:

    • – Increased cardiothoracic ratio due to dilated atria with normal ventricular size

    • – Increased pulmonary vasculature and interstitial edema with Kerley B lines

    • – Pleural effusions may be present

  • Two-dimensional echocardiography findings:

    • – Normal systolic right and left ventricular function with normal or increased wall thickness, with normal or reduced ventricular cavity size

    • – Markedly dilated left and right atria

    • – A ventricular mural thrombus may be present

    • – Color Doppler evidence of mitral, tricuspid, or pulmonic valve regurgitation may be present

    • – Mitral inflow, pulmonary venous, and tissue Doppler evidence of restrictive diastolic filling pattern

    • – Non-Doppler speckle-tracking strain imaging to detect early cardiac involvement

DIAGNOSTIC PROCEDURES
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