Biventricular myocardial noncompaction presenting with complete atrioventricular block.

Noncompaction of ventricular myocardium has been recognized as a distinct form of cardiomyopathy with its own clinical presentation and natural history. Concomitance of either valvular pathologies or complete atrioventricular block with noncompaction of ventricular myocardium has rarely been reported. Herein, we present a case with biventricular noncompaction with significant interventricular septum involvement presenting with complete atrioventricular block, who was formerly diagnosed to have mitral and aortic insufficiency.