Noncompaction of ventricular myocardium, complete atrioventricular block and minor congenital heart abnormalities

Abstract

Ventricular noncompaction is a rare cardiomyopathy characterized by numerous, prominent ventricular trabeculations and deep intertrabecular recesses and is caused by a disorder of endomyocardial morphogenesis. It can occur in patients with other congenital anomalies, such as various ventricular obstructive lesions, or in isolated form in the absence of associated anomalies. Patients with this unique disease may present with heart failure, systemic emboli, and ventricular arrhythmias. In this case report, we present an unusual coexistence of ventricular noncompaction and atrial septal aneurysm and mitral valve cleft in a young patient who presented with complete AV-block.