• M Cecilia Gonzalez Corcia, Juan Sieira, Gudrun Pappaert, Carlo de Asmundis, Gian Battista Chierchia, Mark La Meir, Andrea Sarkozy, and Pedro Brugada.
• Heart Rhythm Management, Universitair Ziekenhuis Brussel-Vrije Universiteit Brussel, Brussels, Belgium; Pediatric Cardiology Department, St. Luc Hospital, Brussels, Belgium. Electronic address: maria.c.gonzalez@uclouvain.be.
• J. Am. Coll. Cardiol. 2018 Jan 16; 71 (2): 148-157.

BackgroundYoung 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.ObjectivesThis study investigated clinical features, management, and long-term follow-up of young patients with Brugada syndrome and ICD.MethodsPatients diagnosed with Brugada syndrome, who underwent implantation of an ICD at an age of ≤20 years, were studied.ResultsThe 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.ConclusionsICD 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.Copyright © 2018 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.

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