Adipositas Cordis: A Rare and Poorly Understood Cardiomyopathy

Adipositas cordis is a rare cardiomyopathy characterized by diffuse fatty infiltration of the ventricular myocardium or interventricular septum. This occurs without myocardial cell destruction, unlike arrhythmogenic right ventricular cardiomyopathy. A 40-year-old obese woman was found to have a II/VI systolic murmur that worsened with standing. A transthoracic echocardiogram showed interventricular septal hypertrophy with a preserved left ventricular ejection fraction. Cardiac magnetic resonance imaging revealed a fatty mass in the interventricular septum. An endomyocardial biopsy revealed structurally normal myocytes with diffuse adipose cell infiltration and no evidence of malignant cells. Left and right cardiac catheterizations and stress echocardiography showed no abnormalities. This case shows the importance of considering a broad differential when approaching rare diseases. It also demonstrates the utility of noninvasive imaging and its impact on clinical decision making.