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; 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

doi:10.1016/j.hrthm.2018.01.014

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 HuangIsabelle 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^*

^*Corresponding author for this work

Department of Clinical Medicine

24 Citations (Scopus)

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 language	English
Journal	Heart Rhythm
Volume	15
Issue number	5
Pages (from-to)	716-724
Number of pages	9
ISSN	1547-5271
DOIs	https://doi.org/10.1016/j.hrthm.2018.01.014
Publication status	Published - 1 May 2018

Keywords

Arrhythmic risk stratification
Electrophysiologic study
Genetics
ICD
Sudden cardiac death
Prognosis
Humans
Middle Aged
Death, Sudden, Cardiac/epidemiology
Israel/epidemiology
Male
Incidence
Young Adult
Survival Rate/trends
United States/epidemiology
Europe/epidemiology
Time Factors
Defibrillators, Implantable
Republic of Korea/epidemiology
Electrocardiography
Adult
Female
Surveys and Questionnaires
China/epidemiology
Japan/epidemiology
Risk Assessment
Brugada Syndrome/complications
Quebec/epidemiology
Adolescent
Aged

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10.1016/j.hrthm.2018.01.014

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Milman, A., Andorin, A., Gourraud, J. B., Postema, P. G., Sacher, F., Mabo, P., Kim, S. H., Juang, J. J. M., Maeda, S., Takahashi, Y., Kamakura, T., Aiba, T., Conte, G., Sarquella-Brugada, G., Leshem, E., Rahkovich, M., Hochstadt, A., Mizusawa, Y., Arbelo, E., ... 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, 15(5), 716-724. https://doi.org/10.1016/j.hrthm.2018.01.014

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 et al.
In: Heart Rhythm, Vol. 15, No. 5, 01.05.2018, p. 716-724.

Research output: Contribution to journal › Journal article › Research › peer-review

Milman, A, Andorin, A, Gourraud, JB, Postema, PG, Sacher, F, Mabo, P, Kim, SH, 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, GX, Brugada, J, Leenhardt, A, Wilde, AAM, Brugada, P, Kusano, KF, Hirao, K, Nam, GB, 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

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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.",

keywords = "Arrhythmic risk stratification, Electrophysiologic study, Genetics, ICD, Sudden cardiac death, Prognosis, Humans, Middle Aged, Death, Sudden, Cardiac/epidemiology, Israel/epidemiology, Male, Incidence, Young Adult, Survival Rate/trends, United States/epidemiology, Europe/epidemiology, Time Factors, Defibrillators, Implantable, Republic of Korea/epidemiology, Electrocardiography, Adult, Female, Surveys and Questionnaires, China/epidemiology, Japan/epidemiology, Risk Assessment, Brugada Syndrome/complications, Quebec/epidemiology, Adolescent, Aged",

author = "Anat Milman and Antoine Andorin and Gourraud, {Jean Baptiste} and Postema, {Pieter G.} and Frederic Sacher and Philippe Mabo and Kim, {Sung Hwan} 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 Yan, {Gan Xin} and Josep Brugada and Antoine Leenhardt and Wilde, {Arthur A.M.} and Pedro Brugada and Kusano, {Kengo F.} and Kenzo Hirao and Nam, {Gi Byoung} and Vincent Probst and Bernard Belhassen",

year = "2018",

month = may,

day = "1",

doi = "10.1016/j.hrthm.2018.01.014",

language = "English",

volume = "15",

pages = "716--724",

journal = "Heart Rhythm",

issn = "1547-5271",

publisher = "Elsevier",

number = "5",

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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

PY - 2018/5/1

Y1 - 2018/5/1

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.

KW - Arrhythmic risk stratification

KW - Electrophysiologic study

KW - Genetics

KW - ICD

KW - Sudden cardiac death

KW - Prognosis

KW - Humans

KW - Middle Aged

KW - Death, Sudden, Cardiac/epidemiology

KW - Israel/epidemiology

KW - Male

KW - Incidence

KW - Young Adult

KW - Survival Rate/trends

KW - United States/epidemiology

KW - Europe/epidemiology

KW - Time Factors

KW - Defibrillators, Implantable

KW - Republic of Korea/epidemiology

KW - Electrocardiography

KW - Adult

KW - Female

KW - Surveys and Questionnaires

KW - China/epidemiology

KW - Japan/epidemiology

KW - Risk Assessment

KW - Brugada Syndrome/complications

KW - Quebec/epidemiology

KW - Adolescent

KW - Aged

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U2 - 10.1016/j.hrthm.2018.01.014

DO - 10.1016/j.hrthm.2018.01.014

M3 - Journal article

C2 - 29325976

AN - SCOPUS:85043392645

SN - 1547-5271

VL - 15

SP - 716

EP - 724

JO - Heart Rhythm

JF - Heart Rhythm

IS - 5

ER -

Profile of patients with Brugada syndrome presenting with their first documented arrhythmic event: Data from the Survey on Arrhythmic Events in BRUgada Syndrome (SABRUS)

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