Isolated noncompaction of right ventricle--a case report

Abstract

Isolated noncompaction of ventricular myocardium (INVM) is a genetic cardiomyopathy due to abnormal arrest in endomyocardial embryogenesis between fetal 5th and 8th week. Noncompaction of right ventricle alone is rare. Here we present one such case where a young man presented with progressive right heart failure and atrial fibrillation. Subsequent evaluation by echo and cardiac magnetic resonance imaging confirmed our diagnosis. The cardinal manifestations of INVM are heart failure, arrhythmia, and embolic events and our case presented with former two manifestations. Echocardiographic criteria for diagnosing INVM are discussed.