Noncompaction of the ventricular myocardium: characterization and follow-up of an affected population

Noncompaction of the ventricular myocardium (NVM) is a rare congenital disease caused by an arrest in normal myocardial embryogenesis, leading to persistence of numerous prominent trabeculations which communicate with the left ventricle. It was first described as a congenital condition affecting children, but several cases have been reported of late presentation, and recent studies suggest it may be underdiagnosed. The main clinical manifestations are congestive heart failure, arrhythmias (supraventricular or ventricular) and systemic embolism. We describe a series of twenty patients, focusing on clinical history, echocardiography and follow-up.