• Yoav Michowitz, Anat Milman, Antoine Andorin, Georgia Sarquella-Brugada, M Cecilia Gonzalez Corcia, Jean-Baptiste Gourraud, Giulio Conte, Frederic Sacher, Jimmy J M Juang, Sung-Hwan Kim, Eran Leshem, Philippe Mabo, Pieter G Postema, Aviram Hochstadt, Yanushi D Wijeyeratne, Isabelle Denjoy, Carla Giustetto, Yuka Mizusawa, Zhengrong Huang, Camilla H Jespersen, Shingo Maeda, Yoshihide Takahashi, Tsukasa Kamakura, Takeshi Aiba, Elena Arbelo, Andrea Mazzanti, Giuseppe Allocca, Ramon Brugada, Ruben Casado-Arroyo, Jean Champagne, Silvia G Priori, Christian Veltmann, Pietro Delise, Domenico Corrado, Josep Brugada, Kengo F Kusano, Kenzo Hirao, Leonardo Calo, Masahiko Takagi, Jacob Tfelt-Hansen, Gan-Xin Yan, Fiorenzo Gaita, Antoine Leenhardt, Elijah R Behr, Wilde Arthur A M AAM European Reference Network for Rare and Low Prevalence Complex Diseases of the Heart; Heart Centre AMC, Department of Clinical and Experimental Cardi, Gi-Byoung Nam, Pedro Brugada, Vincent Probst, and Bernard Belhassen.
• Department of Cardiology, Tel Aviv Sourasky Medical Center, Tel Aviv, Israel; Sackler School of Medicine, Tel Aviv University, Tel Aviv, Israel.
• J. Am. Coll. Cardiol. 2019 Apr 16; 73 (14): 1756-1765.

BackgroundInformation on young patients with Brugada syndrome (BrS) and arrhythmic events (AEs) is limited.ObjectivesThe purpose of this study was to describe their characteristics and management as well as risk factors for AE recurrence.MethodsA total of 57 patients (age ≤20 years), all with BrS and AEs, were divided into pediatric (age ≤12 years; n = 26) and adolescents (age 13 to 20 years; n = 31).ResultsPatients' median age at time of first AE was 14 years, with a majority of males (74%), Caucasians (70%), and probands (79%) who presented as aborted cardiac arrest (84%). A significant proportion of patients (28%) exhibited fever-related AE. Family history of sudden cardiac death (SCD), prior syncope, spontaneous type 1 Brugada electrocardiogram (ECG), inducible ventricular fibrillation at electrophysiological study, and SCN5A mutations were present in 26%, 49%, 65%, 28%, and 58% of patients, respectively. The pediatric group differed from the adolescents, with a greater proportion of females, Caucasians, fever-related AEs, and spontaneous type-1 ECG. During follow-up, 68% of pediatric and 64% of adolescents had recurrent AE, with median time of 9.9 and 27.0 months, respectively. Approximately one-third of recurrent AEs occurred on quinidine therapy, and among the pediatric group, 60% of recurrent AEs were fever-related. Risk factors for recurrent AE included sinus node dysfunction, atrial arrhythmias, intraventricular conduction delay, or large S-wave on ECG lead I in the pediatric group and the presence of SCN5A mutation among adolescents.ConclusionsYoung BrS patients with AE represent a very arrhythmogenic group. Current management after first arrhythmia episode is associated with high recurrence rate. Alternative therapies, besides defibrillator implantation, should be considered.Copyright © 2019 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.

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