Biventricular noncompaction associated with left ventricular systolic and diastolic dysfunction and severe pulmonary hypertension in a young man.

Noncompaction of the ventricular myocardium is a recently recognized genetic cardiomyopathy. The left ventricle is the most affected site, but right ventricular involvement has been reported in some cases. Diagnosis is made with 2-dimensional echocardiography or cardiac magnetic resonance imaging. The major clinical manifestations are heart failure, arrhythmias and embolic events. A 20-year old man had left and right ventricular noncompaction complicated by severe pulmonary hypertension, which is one of the first cases of biventricular noncompaction associated with severe pulmonary hypertension. Pulmonary hypertension may be a consequence of increased pulmonary venous pressures caused by systolic and diastolic heart dysfunction secondary to noncompaction.