Implantable Cardioverter-Defibrillators in Children and Adolescents With Brugada Syndrome

BACKGROUND Young patients presenting with symptomatic Brugada syndrome have very high risks for ventricular arrhythmias and should be carefully considered for implantable cardioverter-defibrillator (ICD) placement. However, this therapy is associated with high rates of inappropriate shocks and device-related complications. OBJECTIVES This study investigated clinical features, management, and long-term follow-up of young patients with Brugada syndrome and ICD. METHODS Patients diagnosed with Brugada syndrome, who underwent implantation of an ICD at an age of <= 20 years, were studied. RESULTS The study included 35 consecutive patients. The mean age at ICD placement was 13.9 +/- 6.2 years. Ninety-two percent were symptomatic; 29% presented with aborted sudden cardiac death and 63% with syncope. During a mean follow-up period of 88 months, sustained ventricular arrhythmias were treated by the ICD in 9 patients (26%), including shocks in 8 patients (23%) and antitachycardia pacing in 1 patient (3%). Three patients (9%) died in an electrical storm. Seven patients (20%) experienced inappropriate shocks, and 5 patients (14%) had device-related complications. Aborted sudden cardiac death and spontaneous type I electrocardiogram were identified as independent predictors of appropriate shock occurrence. CONCLUSIONS ICD therapy is an effective strategy in young patients with symptomatic Brugada syndrome, treating potentially lethal arrhythmias in >25% of patients during follow-up. Appropriate shocks were significantly associated with previously aborted sudden cardiac death and spontaneous type I electrocardiograms. However, ICDs are frequently associated with complications and inappropriate shocks, both of which remain high regardless of careful device implantation and programming. (c) 2018 by the American College of Cardiology Foundation.