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Noncompaction of the ventricular myocardium is a rare congenital cardiomyopathy caused by arrest of normal endomyocardial embryogenesis. Isolated right ventricular noncompaction (IRNC) is an even rarer form of this disease. We report herein on a 68 year-old male diagnosed with IRNC who presented with right-sided heart failure, without involvement of the left ventricle. Diagnosis was achieved with the aid of echocardiography and ventriculography. Medical treatment including prescription of diuretics, a calcium channel blocker, and digitalis, improved both the symptoms and right ventricular function. (Korean J Med 2015; 88:69-73)

Isolated Right Ventricular Noncompaction Accompanied by Right Ventricular Failure