Characterization and Management of Arrhythmic Events in Young Patients With Brugada Syndrome

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 JespersenShingo 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, Arthur A M Wilde, Gi-Byoung Nam, Pedro Brugada, Vincent Probst, Bernard Belhassen

Research output: Contribution to journalArticlepeer-review

44 Citations (Scopus)

Abstract

BACKGROUND: Information on young patients with Brugada syndrome (BrS) and arrhythmic events (AEs) is limited.

OBJECTIVES: The purpose of this study was to describe their characteristics and management as well as risk factors for AE recurrence.

METHODS: A 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).

RESULTS: Patients' 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.

CONCLUSIONS: Young 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.

Original languageEnglish
Pages (from-to)1756-1765
Number of pages10
JournalJournal of the American College of Cardiology
Volume73
Issue number14
DOIs
Publication statusPublished - 16 Apr 2019

Bibliographical note

Copyright © 2019 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.

Keywords

  • Brugada syndrome
  • SCN5A mutation
  • ablation
  • adolescence
  • pediatric
  • quinidine

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