Cardioverter defibrillator implantation in an adult with isolated noncompaction of the ventricular myocardium

Isolated noncompaction of the ventricular myocardium is an unclassified cardiomyopathy and is thought to be due to arrest of myocardial morphogenesis. Fatal ventricular arrhythmias may occur in approximately half of the patients and account for half of the death in this disorder. In this report, we describe a patient with isolated noncompaction of the ventricular myocardium in whom implantation of automatic cardioverter defibrillator was thought to be effective to prevent the risk of sudden cardiac death.