Defibrillator for primary prevention in congenital heart disease.

A 21-year-old patient with repaired double-outlet right ventricle and normal ventricular function underwent internal cardioverter defibrillator implantation for primary prevention of sudden death. First occurrence of spontaneous ventricular tachycardia resulted in hemodynamic collapse and syncope but the internal cardioverter defibrillator rescued the patient. ICD implantation for primary prevention may be an appropriate goal in adults with repaired congenital heart disease, even in the setting or normal ventricular function.