Profile of patients with Brugada syndrome presenting with their first documented arrhythmic event

Data from the Survey on Arrhythmic Events in BRUgada Syndrome (SABRUS)

Anat Milman, Antoine Andorin, Jean-Baptiste Gourraud, Pieter G Postema, Frederic Sacher, Philippe Mabo, Sung-Hwan Kim, Jimmy J M Juang, Shingo Maeda, Yoshihide Takahashi, Tsukasa Kamakura, Takeshi Aiba, Giulio Conte, Georgia Sarquella-Brugada, Eran Leshem, Michael Rahkovich, Aviram Hochstadt, Yuka Mizusawa, Elena Arbelo, Zhengrong Huang & 27 others Isabelle Denjoy, Carla Giustetto, Yanushi D Wijeyeratne, Carlo Napolitano, Yoav Michowitz, Ramon Brugada, Ruben Casado-Arroyo, Jean Champagne, Leonardo Calo, Jacob Tfelt-Hansen, Silvia G Priori, Masahiko Takagi, Christian Veltmann, Pietro Delise, Domenico Corrado, Elijah R Behr, Fiorenzo Gaita, Gan-Xin Yan, Josep Brugada, Antoine Leenhardt, Arthur A M Wilde, Pedro Brugada, Kengo F Kusano, Kenzo Hirao, Gi-Byoung Nam, Vincent Probst, Bernard Belhassen

Research output: Contribution to journalArticle

Abstract

BACKGROUND: Detailed information on the profile of patients with Brugada syndrome (BrS) presenting their first arrhythmic event (AE) after prophylactic implantation of an implantable cardioverter-defibrillator (ICD) is limited.

OBJECTIVES: The objectives of this study were (1) to compare clinical, electrocardiographic, electrophysiologic, and genetic profiles of patients who exhibited their first documented AE as aborted cardiac arrest (group A) with profiles of those in whom the AE was documented after prophylactic ICD implantation (group B) and (2) to characterize group B patients' profile using the class II indications for ICD implantation established by HRS/EHRA/APHRS expert consensus statement in 2013.

METHODS: A survey of 23 centers from 10 Western and 4 Asian countries enabled data collection of 678 patients with BrS who exhibited their AE (group A, n = 426; group B, n = 252).

RESULTS: The first AE occurred in group B patients 6.7 years later than in group A (mean age 46.1 ± 13.3 years vs 39.4 ± 15.1 years; P < .001). Group B patients had a higher incidence of family history of sudden cardiac death and SCN5A mutations. Of the 252 group B patients, 189 (75%) complied with the HRS/EHRA/APHRS indications whereas the remaining 63 (25%) did not.

CONCLUSION: Patients with BrS with the first AE documented after prophylactic ICD implantation exhibited their AE at a later age with a higher incidence of positive family history of sudden cardiac death and SCN5A mutations as compared with those presenting with aborted cardiac arrest. Only 75% of patients who exhibited an AE after receiving a prophylactic ICD complied with the 2013 class II indications, suggesting that efforts are still required for improving risk stratification.

Original languageEnglish
Pages (from-to)716-724
Number of pages9
JournalHeart Rhythm
Volume15
Issue number5
DOIs
Publication statusPublished - May 2018

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Brugada Syndrome
Implantable Defibrillators
Sudden Cardiac Death
Heart Arrest
Surveys and Questionnaires
Mutation
Incidence
Consensus

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Profile of patients with Brugada syndrome presenting with their first documented arrhythmic event : Data from the Survey on Arrhythmic Events in BRUgada Syndrome (SABRUS). / Milman, Anat; Andorin, Antoine; Gourraud, Jean-Baptiste; Postema, Pieter G; Sacher, Frederic; Mabo, Philippe; Kim, Sung-Hwan; Juang, Jimmy J M; Maeda, Shingo; Takahashi, Yoshihide; Kamakura, Tsukasa; Aiba, Takeshi; Conte, Giulio; Sarquella-Brugada, Georgia; Leshem, Eran; Rahkovich, Michael; Hochstadt, Aviram; Mizusawa, Yuka; Arbelo, Elena; Huang, Zhengrong; Denjoy, Isabelle; Giustetto, Carla; Wijeyeratne, Yanushi D; Napolitano, Carlo; Michowitz, Yoav; Brugada, Ramon; Casado-Arroyo, Ruben; Champagne, Jean; Calo, Leonardo; Tfelt-Hansen, Jacob; Priori, Silvia G; Takagi, Masahiko; Veltmann, Christian; Delise, Pietro; Corrado, Domenico; Behr, Elijah R; Gaita, Fiorenzo; Yan, Gan-Xin; Brugada, Josep; Leenhardt, Antoine; Wilde, Arthur A M; Brugada, Pedro; Kusano, Kengo F; Hirao, Kenzo; Nam, Gi-Byoung; Probst, Vincent; Belhassen, Bernard.

In: Heart Rhythm, Vol. 15, No. 5, 05.2018, p. 716-724.

Research output: Contribution to journalArticle

Milman, A, Andorin, A, Gourraud, J-B, Postema, PG, Sacher, F, Mabo, P, Kim, S-H, Juang, JJM, Maeda, S, Takahashi, Y, Kamakura, T, Aiba, T, Conte, G, Sarquella-Brugada, G, Leshem, E, Rahkovich, M, Hochstadt, A, Mizusawa, Y, Arbelo, E, Huang, Z, Denjoy, I, Giustetto, C, Wijeyeratne, YD, Napolitano, C, Michowitz, Y, Brugada, R, Casado-Arroyo, R, Champagne, J, Calo, L, Tfelt-Hansen, J, Priori, SG, Takagi, M, Veltmann, C, Delise, P, Corrado, D, Behr, ER, Gaita, F, Yan, G-X, Brugada, J, Leenhardt, A, Wilde, AAM, Brugada, P, Kusano, KF, Hirao, K, Nam, G-B, Probst, V & Belhassen, B 2018, 'Profile of patients with Brugada syndrome presenting with their first documented arrhythmic event: Data from the Survey on Arrhythmic Events in BRUgada Syndrome (SABRUS)', Heart Rhythm, vol. 15, no. 5, pp. 716-724. https://doi.org/10.1016/j.hrthm.2018.01.014
Milman, Anat ; Andorin, Antoine ; Gourraud, Jean-Baptiste ; Postema, Pieter G ; Sacher, Frederic ; Mabo, Philippe ; Kim, Sung-Hwan ; Juang, Jimmy J M ; Maeda, Shingo ; Takahashi, Yoshihide ; Kamakura, Tsukasa ; Aiba, Takeshi ; Conte, Giulio ; Sarquella-Brugada, Georgia ; Leshem, Eran ; Rahkovich, Michael ; Hochstadt, Aviram ; Mizusawa, Yuka ; Arbelo, Elena ; Huang, Zhengrong ; Denjoy, Isabelle ; Giustetto, Carla ; Wijeyeratne, Yanushi D ; Napolitano, Carlo ; Michowitz, Yoav ; Brugada, Ramon ; Casado-Arroyo, Ruben ; Champagne, Jean ; Calo, Leonardo ; Tfelt-Hansen, Jacob ; Priori, Silvia G ; Takagi, Masahiko ; Veltmann, Christian ; Delise, Pietro ; Corrado, Domenico ; Behr, Elijah R ; Gaita, Fiorenzo ; Yan, Gan-Xin ; Brugada, Josep ; Leenhardt, Antoine ; Wilde, Arthur A M ; Brugada, Pedro ; Kusano, Kengo F ; Hirao, Kenzo ; Nam, Gi-Byoung ; Probst, Vincent ; Belhassen, Bernard. / Profile of patients with Brugada syndrome presenting with their first documented arrhythmic event : Data from the Survey on Arrhythmic Events in BRUgada Syndrome (SABRUS). In: Heart Rhythm. 2018 ; Vol. 15, No. 5. pp. 716-724.
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title = "Profile of patients with Brugada syndrome presenting with their first documented arrhythmic event: Data from the Survey on Arrhythmic Events in BRUgada Syndrome (SABRUS)",
abstract = "BACKGROUND: Detailed information on the profile of patients with Brugada syndrome (BrS) presenting their first arrhythmic event (AE) after prophylactic implantation of an implantable cardioverter-defibrillator (ICD) is limited.OBJECTIVES: The objectives of this study were (1) to compare clinical, electrocardiographic, electrophysiologic, and genetic profiles of patients who exhibited their first documented AE as aborted cardiac arrest (group A) with profiles of those in whom the AE was documented after prophylactic ICD implantation (group B) and (2) to characterize group B patients' profile using the class II indications for ICD implantation established by HRS/EHRA/APHRS expert consensus statement in 2013.METHODS: A survey of 23 centers from 10 Western and 4 Asian countries enabled data collection of 678 patients with BrS who exhibited their AE (group A, n = 426; group B, n = 252).RESULTS: The first AE occurred in group B patients 6.7 years later than in group A (mean age 46.1 ± 13.3 years vs 39.4 ± 15.1 years; P < .001). Group B patients had a higher incidence of family history of sudden cardiac death and SCN5A mutations. Of the 252 group B patients, 189 (75{\%}) complied with the HRS/EHRA/APHRS indications whereas the remaining 63 (25{\%}) did not.CONCLUSION: Patients with BrS with the first AE documented after prophylactic ICD implantation exhibited their AE at a later age with a higher incidence of positive family history of sudden cardiac death and SCN5A mutations as compared with those presenting with aborted cardiac arrest. Only 75{\%} of patients who exhibited an AE after receiving a prophylactic ICD complied with the 2013 class II indications, suggesting that efforts are still required for improving risk stratification.",
author = "Anat Milman and Antoine Andorin and Jean-Baptiste Gourraud and Postema, {Pieter G} and Frederic Sacher and Philippe Mabo and Sung-Hwan Kim and Juang, {Jimmy J M} and Shingo Maeda and Yoshihide Takahashi and Tsukasa Kamakura and Takeshi Aiba and Giulio Conte and Georgia Sarquella-Brugada and Eran Leshem and Michael Rahkovich and Aviram Hochstadt and Yuka Mizusawa and Elena Arbelo and Zhengrong Huang and Isabelle Denjoy and Carla Giustetto and Wijeyeratne, {Yanushi D} and Carlo Napolitano and Yoav Michowitz and Ramon Brugada and Ruben Casado-Arroyo and Jean Champagne and Leonardo Calo and Jacob Tfelt-Hansen and Priori, {Silvia G} and Masahiko Takagi and Christian Veltmann and Pietro Delise and Domenico Corrado and Behr, {Elijah R} and Fiorenzo Gaita and Gan-Xin Yan and Josep Brugada and Antoine Leenhardt and Wilde, {Arthur A M} and Pedro Brugada and Kusano, {Kengo F} and Kenzo Hirao and Gi-Byoung Nam and Vincent Probst and Bernard Belhassen",
note = "Copyright {\circledC} 2018 Heart Rhythm Society. Published by Elsevier Inc. All rights reserved.",
year = "2018",
month = "5",
doi = "10.1016/j.hrthm.2018.01.014",
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TY - JOUR

T1 - Profile of patients with Brugada syndrome presenting with their first documented arrhythmic event

T2 - Data from the Survey on Arrhythmic Events in BRUgada Syndrome (SABRUS)

AU - Milman, Anat

AU - Andorin, Antoine

AU - Gourraud, Jean-Baptiste

AU - Postema, Pieter G

AU - Sacher, Frederic

AU - Mabo, Philippe

AU - Kim, Sung-Hwan

AU - Juang, Jimmy J M

AU - Maeda, Shingo

AU - Takahashi, Yoshihide

AU - Kamakura, Tsukasa

AU - Aiba, Takeshi

AU - Conte, Giulio

AU - Sarquella-Brugada, Georgia

AU - Leshem, Eran

AU - Rahkovich, Michael

AU - Hochstadt, Aviram

AU - Mizusawa, Yuka

AU - Arbelo, Elena

AU - Huang, Zhengrong

AU - Denjoy, Isabelle

AU - Giustetto, Carla

AU - Wijeyeratne, Yanushi D

AU - Napolitano, Carlo

AU - Michowitz, Yoav

AU - Brugada, Ramon

AU - Casado-Arroyo, Ruben

AU - Champagne, Jean

AU - Calo, Leonardo

AU - Tfelt-Hansen, Jacob

AU - Priori, Silvia G

AU - Takagi, Masahiko

AU - Veltmann, Christian

AU - Delise, Pietro

AU - Corrado, Domenico

AU - Behr, Elijah R

AU - Gaita, Fiorenzo

AU - Yan, Gan-Xin

AU - Brugada, Josep

AU - Leenhardt, Antoine

AU - Wilde, Arthur A M

AU - Brugada, Pedro

AU - Kusano, Kengo F

AU - Hirao, Kenzo

AU - Nam, Gi-Byoung

AU - Probst, Vincent

AU - Belhassen, Bernard

N1 - Copyright © 2018 Heart Rhythm Society. Published by Elsevier Inc. All rights reserved.

PY - 2018/5

Y1 - 2018/5

N2 - BACKGROUND: Detailed information on the profile of patients with Brugada syndrome (BrS) presenting their first arrhythmic event (AE) after prophylactic implantation of an implantable cardioverter-defibrillator (ICD) is limited.OBJECTIVES: The objectives of this study were (1) to compare clinical, electrocardiographic, electrophysiologic, and genetic profiles of patients who exhibited their first documented AE as aborted cardiac arrest (group A) with profiles of those in whom the AE was documented after prophylactic ICD implantation (group B) and (2) to characterize group B patients' profile using the class II indications for ICD implantation established by HRS/EHRA/APHRS expert consensus statement in 2013.METHODS: A survey of 23 centers from 10 Western and 4 Asian countries enabled data collection of 678 patients with BrS who exhibited their AE (group A, n = 426; group B, n = 252).RESULTS: The first AE occurred in group B patients 6.7 years later than in group A (mean age 46.1 ± 13.3 years vs 39.4 ± 15.1 years; P < .001). Group B patients had a higher incidence of family history of sudden cardiac death and SCN5A mutations. Of the 252 group B patients, 189 (75%) complied with the HRS/EHRA/APHRS indications whereas the remaining 63 (25%) did not.CONCLUSION: Patients with BrS with the first AE documented after prophylactic ICD implantation exhibited their AE at a later age with a higher incidence of positive family history of sudden cardiac death and SCN5A mutations as compared with those presenting with aborted cardiac arrest. Only 75% of patients who exhibited an AE after receiving a prophylactic ICD complied with the 2013 class II indications, suggesting that efforts are still required for improving risk stratification.

AB - BACKGROUND: Detailed information on the profile of patients with Brugada syndrome (BrS) presenting their first arrhythmic event (AE) after prophylactic implantation of an implantable cardioverter-defibrillator (ICD) is limited.OBJECTIVES: The objectives of this study were (1) to compare clinical, electrocardiographic, electrophysiologic, and genetic profiles of patients who exhibited their first documented AE as aborted cardiac arrest (group A) with profiles of those in whom the AE was documented after prophylactic ICD implantation (group B) and (2) to characterize group B patients' profile using the class II indications for ICD implantation established by HRS/EHRA/APHRS expert consensus statement in 2013.METHODS: A survey of 23 centers from 10 Western and 4 Asian countries enabled data collection of 678 patients with BrS who exhibited their AE (group A, n = 426; group B, n = 252).RESULTS: The first AE occurred in group B patients 6.7 years later than in group A (mean age 46.1 ± 13.3 years vs 39.4 ± 15.1 years; P < .001). Group B patients had a higher incidence of family history of sudden cardiac death and SCN5A mutations. Of the 252 group B patients, 189 (75%) complied with the HRS/EHRA/APHRS indications whereas the remaining 63 (25%) did not.CONCLUSION: Patients with BrS with the first AE documented after prophylactic ICD implantation exhibited their AE at a later age with a higher incidence of positive family history of sudden cardiac death and SCN5A mutations as compared with those presenting with aborted cardiac arrest. Only 75% of patients who exhibited an AE after receiving a prophylactic ICD complied with the 2013 class II indications, suggesting that efforts are still required for improving risk stratification.

U2 - 10.1016/j.hrthm.2018.01.014

DO - 10.1016/j.hrthm.2018.01.014

M3 - Article

VL - 15

SP - 716

EP - 724

JO - Heart Rhythm

JF - Heart Rhythm

SN - 1547-5271

IS - 5

ER -