Control of idiopathic ventricular fibrillation by implantable cardioverter-defibrillator in a child who survived sudden death

Idiopathic ventricular fibrillation (VF) is extremely rare in children who have not previously undergone cardiac surgery. Patients resuscitated from i diopathic VF remain at risk for recurrence. The use of an implantable cardi overter-defibrillator (ICD) effectively prevents such recurrences. We repor t the case of a 12-year-old girl who had a history of recurrent syncope and had survived an episode of VF. Serial studies after prolonged but successf ul resuscitation, including echocardiography, an electrocardiogram (ECG), a nd coronary angiography failed to reveal abnormal cardiac structures respon sible for VF. No abnormal conduction pathways or abnormal early or late aft erdepolarization were found on electrophysiologic study. The ST segments of the 12-lead ECG remained normal after procainamide challenge. The patient underwent ICD implantation 2 weeks after admission and syncope did not recu r during a follow-up of 14 months. This report emphasizes that idiopathic V F may be responsible for syncope in children. ICD therapy prevents the recu rrence of idiopathic VF and the associated risk of sudden death.