Abstract

A 16-year-old patient with auto-immune polyendocrinopathy, candidiasis and ectodermal dystrophy presented with signs of leftand right-sided heart failure. Echocardiography showed restrictive ventricular physiology with enlarged atria, suggesting restrictive cardiomyopathy. Computed tomography, however, showed extensive pericardial calcification surrounding the ventricles with relative atrial sparing (Fig. 1 and Video 1). The calcified pericardium was successfully removed surgically.

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