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; 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

doi:10.1016/j.jacc.2019.01.048

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 journal › Article › peer-review

53 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 language	English
Pages (from-to)	1756-1765
Number of pages	10
Journal	Journal of the American College of Cardiology
Volume	73
Issue number	14
DOIs	https://doi.org/10.1016/j.jacc.2019.01.048
Publication status	Published - 16 Apr 2019

Bibliographical note

Keywords

Brugada syndrome
SCN5A mutation
ablation
adolescence
pediatric
quinidine

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10.1016/j.jacc.2019.01.048

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Michowitz, Y., Milman, A., Andorin, A., Sarquella-Brugada, G., Gonzalez Corcia, M. C., Gourraud, J-B., Conte, G., Sacher, F., Juang, J. J. M., Kim, S-H., Leshem, E., Mabo, P., Postema, P. G., Hochstadt, A., Wijeyeratne, Y. D., Denjoy, I., Giustetto, C., Mizusawa, Y., Huang, Z., ... 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

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.

@article{06ac9ea3b23d4c6692b7e652e9a94e4f,

title = "Characterization and Management of Arrhythmic Events in Young Patients With Brugada Syndrome",

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.",

keywords = "Brugada syndrome, SCN5A mutation, ablation, adolescence, pediatric, quinidine",

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

year = "2019",

month = apr,

day = "16",

doi = "10.1016/j.jacc.2019.01.048",

language = "English",

volume = "73",

pages = "1756--1765",

journal = "Journal of the American College of Cardiology",

issn = "0735-1097",

publisher = "Elsevier USA",

number = "14",

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Michowitz, Y, Milman, A, Andorin, A, Sarquella-Brugada, G, Gonzalez Corcia, MC, Gourraud, J-B, Conte, G, Sacher, F, Juang, JJM, Kim, S-H, 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, G-X, Gaita, F, Leenhardt, A, Behr, ER, Wilde, AAM, Nam, G-B, 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

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 journal › Article › peer-review

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 - Brugada syndrome

KW - SCN5A mutation

KW - ablation

KW - adolescence

KW - pediatric

KW - quinidine

U2 - 10.1016/j.jacc.2019.01.048

DO - 10.1016/j.jacc.2019.01.048

M3 - Article

C2 - 30975291

VL - 73

SP - 1756

EP - 1765

JO - Journal of the American College of Cardiology

JF - Journal of the American College of Cardiology

SN - 0735-1097

IS - 14

ER -

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

Abstract

Bibliographical note

Keywords

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