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

Afdeling for Retspatologi

18 Citationer (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.

Originalsprog	Engelsk
Tidsskrift	Journal of the American College of Cardiology
Vol/bind	73
Udgave nummer	14
Sider (fra-til)	1756-1765
ISSN	0735-1097
DOI	https://doi.org/10.1016/j.jacc.2019.01.048
Status	Udgivet - 16 apr. 2019

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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, 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, bind 73, nr. 14, s. 1756-1765. https://doi.org/10.1016/j.jacc.2019.01.048

@article{4302a6c07d474aeb95620021f106298e,

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.",

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,

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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",

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number = "14",

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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.

U2 - 10.1016/j.jacc.2019.01.048

DO - 10.1016/j.jacc.2019.01.048

M3 - Journal article

C2 - 30975291

SN - 0735-1097

VL - 73

SP - 1756

EP - 1765

JO - Journal of the American College of Cardiology

JF - Journal of the American College of Cardiology

IS - 14

ER -

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

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