Biventricular noncompaction presented with symptomatic complete heart block - Report of a case and review of literature

Ventricular noncompaction has been recognized as a distinct form of rare cardiomyopathy characterized by numerous, prominent ventricular trabeculations and deep intertrabecular recesses and is caused by a disorder of endomyocardial morphogenesis. Concomitance of either valvular pathologies or complete atrioventricular block with biventricular noncompaction has rarely been reported. Herein, we present a case of 67 years old male presented with syncopal attack and congestive heart failure due to biventricular noncompaction with significant left ventricular dysfunction associated with complete atrioventricular block. He was formerly diagnosed as dilated cardiomyopathy for last 2 years. Review of literatures of all reported cases has been discussed.