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. Jespersen & 29 others 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, Arthur A.M. Wilde, Gi Byoung Nam, Pedro Brugada, Vincent Probst, Bernard Belhassen

Research output: Contribution to journalArticle

2 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 - Apr 16 2019

Fingerprint

Brugada Syndrome
Pediatrics
Electrocardiography
Fever
Cardiac Arrhythmias
Sick Sinus Syndrome
Recurrence
Mutation
Quinidine
Defibrillators
Sudden Cardiac Death
Syncope
Ventricular Fibrillation
Complementary Therapies
Heart Arrest

Keywords

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

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine

Cite this

Michowitz, Y., Milman, A., Andorin, A., Sarquella-Brugada, G., Gonzalez Corcia, M. C., Gourraud, J. B., ... Belhassen, B. (2019). Characterization and Management of Arrhythmic Events in Young Patients With Brugada Syndrome. Journal of the American College of Cardiology, 73(14), 1756-1765. https://doi.org/10.1016/j.jacc.2019.01.048

Characterization and Management of Arrhythmic Events in Young Patients With Brugada Syndrome. / Michowitz, Yoav; Milman, Anat; Andorin, Antoine; Sarquella-Brugada, Georgia; Gonzalez Corcia, M. Cecilia; Gourraud, Jean Baptiste; Conte, Giulio; Sacher, Frederic; Juang, Jimmy J.M.; Kim, Sung Hwan; Leshem, Eran; Mabo, Philippe; Postema, Pieter G.; Hochstadt, Aviram; Wijeyeratne, Yanushi D.; Denjoy, Isabelle; Giustetto, Carla; Mizusawa, Yuka; Huang, Zhengrong; Jespersen, Camilla H.; Maeda, Shingo; Takahashi, Yoshihide; Kamakura, Tsukasa; Aiba, Takeshi; Arbelo, Elena; Mazzanti, Andrea; Allocca, Giuseppe; Brugada, Ramon; Casado-Arroyo, Ruben; Champagne, Jean; Priori, Silvia G.; Veltmann, Christian; Delise, Pietro; Corrado, Domenico; Brugada, Josep; Kusano, Kengo F.; Hirao, Kenzo; Calo, Leonardo; Takagi, Masahiko; Tfelt-Hansen, Jacob; Yan, Gan Xin; Gaita, Fiorenzo; Leenhardt, Antoine; Behr, Elijah R.; Wilde, Arthur A.M.; Nam, Gi Byoung; Brugada, Pedro; Probst, Vincent; Belhassen, Bernard.

In: Journal of the American College of Cardiology, Vol. 73, No. 14, 16.04.2019, p. 1756-1765.

Research output: Contribution to journalArticle

Michowitz, Y, Milman, A, Andorin, A, Sarquella-Brugada, G, Gonzalez Corcia, MC, Gourraud, JB, Conte, G, Sacher, F, Juang, JJM, Kim, SH, Leshem, E, Mabo, P, Postema, PG, Hochstadt, A, Wijeyeratne, YD, Denjoy, I, Giustetto, C, Mizusawa, Y, Huang, Z, Jespersen, CH, Maeda, S, Takahashi, Y, Kamakura, T, Aiba, T, Arbelo, E, Mazzanti, A, Allocca, G, Brugada, R, Casado-Arroyo, R, Champagne, J, Priori, SG, Veltmann, C, Delise, P, Corrado, D, Brugada, J, Kusano, KF, Hirao, K, Calo, L, Takagi, M, Tfelt-Hansen, J, Yan, GX, Gaita, F, Leenhardt, A, Behr, ER, Wilde, AAM, Nam, GB, Brugada, P, Probst, V & Belhassen, B 2019, 'Characterization and Management of Arrhythmic Events in Young Patients With Brugada Syndrome', Journal of the American College of Cardiology, vol. 73, no. 14, pp. 1756-1765. https://doi.org/10.1016/j.jacc.2019.01.048
Michowitz Y, Milman A, Andorin A, Sarquella-Brugada G, Gonzalez Corcia MC, Gourraud JB et al. Characterization and Management of Arrhythmic Events in Young Patients With Brugada Syndrome. Journal of the American College of Cardiology. 2019 Apr 16;73(14):1756-1765. https://doi.org/10.1016/j.jacc.2019.01.048
Michowitz, Yoav ; Milman, Anat ; Andorin, Antoine ; Sarquella-Brugada, Georgia ; Gonzalez Corcia, M. Cecilia ; Gourraud, Jean Baptiste ; Conte, Giulio ; Sacher, Frederic ; Juang, Jimmy J.M. ; Kim, Sung Hwan ; Leshem, Eran ; Mabo, Philippe ; Postema, Pieter G. ; Hochstadt, Aviram ; Wijeyeratne, Yanushi D. ; Denjoy, Isabelle ; Giustetto, Carla ; Mizusawa, Yuka ; Huang, Zhengrong ; Jespersen, Camilla H. ; Maeda, Shingo ; Takahashi, Yoshihide ; Kamakura, Tsukasa ; Aiba, Takeshi ; Arbelo, Elena ; Mazzanti, Andrea ; Allocca, Giuseppe ; Brugada, Ramon ; Casado-Arroyo, Ruben ; Champagne, Jean ; Priori, Silvia G. ; Veltmann, Christian ; Delise, Pietro ; Corrado, Domenico ; Brugada, Josep ; Kusano, Kengo F. ; Hirao, Kenzo ; Calo, Leonardo ; Takagi, Masahiko ; Tfelt-Hansen, Jacob ; Yan, Gan Xin ; Gaita, Fiorenzo ; Leenhardt, Antoine ; Behr, Elijah R. ; Wilde, Arthur A.M. ; Nam, Gi Byoung ; Brugada, Pedro ; Probst, Vincent ; Belhassen, Bernard. / Characterization and Management of Arrhythmic Events in Young Patients With Brugada Syndrome. In: Journal of the American College of Cardiology. 2019 ; Vol. 73, No. 14. pp. 1756-1765.
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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.",
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TY - JOUR

T1 - Characterization and Management of Arrhythmic Events in Young Patients With Brugada Syndrome

AU - Michowitz, Yoav

AU - Milman, Anat

AU - Andorin, Antoine

AU - Sarquella-Brugada, Georgia

AU - Gonzalez Corcia, M. Cecilia

AU - Gourraud, Jean Baptiste

AU - Conte, Giulio

AU - Sacher, Frederic

AU - Juang, Jimmy J.M.

AU - Kim, Sung Hwan

AU - Leshem, Eran

AU - Mabo, Philippe

AU - Postema, Pieter G.

AU - Hochstadt, Aviram

AU - Wijeyeratne, Yanushi D.

AU - Denjoy, Isabelle

AU - Giustetto, Carla

AU - Mizusawa, Yuka

AU - Huang, Zhengrong

AU - Jespersen, Camilla H.

AU - Maeda, Shingo

AU - Takahashi, Yoshihide

AU - Kamakura, Tsukasa

AU - Aiba, Takeshi

AU - Arbelo, Elena

AU - Mazzanti, Andrea

AU - Allocca, Giuseppe

AU - Brugada, Ramon

AU - Casado-Arroyo, Ruben

AU - Champagne, Jean

AU - Priori, Silvia G.

AU - Veltmann, Christian

AU - Delise, Pietro

AU - Corrado, Domenico

AU - Brugada, Josep

AU - Kusano, Kengo F.

AU - Hirao, Kenzo

AU - Calo, Leonardo

AU - Takagi, Masahiko

AU - Tfelt-Hansen, Jacob

AU - Yan, Gan Xin

AU - Gaita, Fiorenzo

AU - Leenhardt, Antoine

AU - Behr, Elijah R.

AU - Wilde, Arthur A.M.

AU - Nam, Gi Byoung

AU - Brugada, Pedro

AU - Probst, Vincent

AU - Belhassen, Bernard

PY - 2019/4/16

Y1 - 2019/4/16

N2 - 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.

AB - 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.

KW - ablation

KW - adolescence

KW - Brugada syndrome

KW - pediatric

KW - quinidine

KW - SCN5A mutation

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