II. Epidemiology

  1. Uncommon congenital Cardiomyopathy (<1% of population)

III. Pathophysiology

  1. Disordered in-utero development of mature heart muscle
    1. Increased trabeculation of Myocardium
    2. Development of increased intertrabecular recesses within the left ventricle
  2. Results in Left Ventricular Dysfunction
    1. Heart Failure
    2. Arrhythmia
    3. Thromboembolic disease
    4. Sudden Cardiac Death

IV. Findings

V. Diagnosis

  1. Echocardiogram
    1. Initial, first-line test
  2. Cardiac MRI
    1. Confirms diagnosis

VI. Management

  1. See Congestive Heart Failure
  2. Anticoagulation indications
    1. Atrial Fibrillation
    2. Decreased systolic function
    3. Systemic Thromboembolism or intracardiac thrombus

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