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.