Isolated Biventricular Noncompaction in an adult with severe pulmonary hypertension : an association reviewed.

Abstract

Biventricular noncompaction is a recently recognized rare form of cardiomyopathy. It is characterized by altered structure of myocardial wall as a result of intrauterine arrest of compaction of the myocardial fibers in absence of coexisting congenital lesion. Left ventricle is the most affected site for noncompaction, but right ventricular involvement has been reported in a few cases. Diagnosis is made with 2-dimensional echocardiography or cardiac magnetic resonance imaging. While major clinical manifestations are heart failure, arrhythmias and embolic events,pulmonary artery hypertension ( PAH)has not been well elaborated in the literature. We present a 13- year old boy who had Biventricular noncompaction complicated by severe pulmonary hypertension. Pulmonary hypertension may be a consequence of increased pulmonary venous pressures caused by systolic and diastolic left ventricular dysfunction secondary to noncompaction. This article reviews the literature particularly with reference to PAH in the context of this case.