Author + information
- Received November 2, 2018
- Revision received December 29, 2018
- Accepted January 8, 2019
- Published online April 8, 2019.
- Yoav Michowitz, MDa,b,
- Anat Milman, MD, PhDb,c,
- Antoine Andorin, MDd,e,
- Georgia Sarquella-Brugada, MD, PhDf,
- M. Cecilia Gonzalez Corcia, MDg,
- Jean-Baptiste Gourraud, MD, PhDd,e,
- Giulio Conte, MD, PhDh,
- Frederic Sacher, MDi,
- Jimmy J.M. Juang, MD, PhDj,
- Sung-Hwan Kim, MDk,
- Eran Leshem, MDa,l,
- Philippe Mabo, MDm,
- Pieter G. Postema, MD, PhDe,n,
- Aviram Hochstadt, MDo,
- Yanushi D. Wijeyeratne, MDe,p,
- Isabelle Denjoy, MDe,q,
- Carla Giustetto, MDr,
- Yuka Mizusawa, MDe,n,
- Zhengrong Huang, MD, PhDs,
- Camilla H. Jespersen, MDe,t,u,
- Shingo Maeda, MD, PhDv,
- Yoshihide Takahashi, MD, PhDv,
- Tsukasa Kamakura, MD, PhDw,
- Takeshi Aiba, MD, PhDw,
- Elena Arbelo, MD, PhDx,
- Andrea Mazzanti, MDe,y,
- Giuseppe Allocca, MDz,
- Ramon Brugada, MD, PhDaa,
- Ruben Casado-Arroyo, MD, PhDbb,
- Jean Champagne, MDcc,
- Silvia G. Priori, MD, PhDe,y,
- Christian Veltmann, MDdd,
- Pietro Delise, MDz,
- Domenico Corrado, MD, PhDe,ee,
- Josep Brugada, MD, PhDx,
- Kengo F. Kusano, MD, PhDw,
- Kenzo Hirao, MD, PhDv,
- Leonardo Calo, MDff,
- Masahiko Takagi, MD, PhDgg,
- Jacob Tfelt-Hansen, MD, DMSce,t,u,
- Gan-Xin Yan, MD, PhDhh,
- Fiorenzo Gaita, MDr,
- Antoine Leenhardt, MDe,q,
- Elijah R. Behr, MDe,p,
- Arthur A.M. Wilde, MD, PhDe,n,
- Gi-Byoung Nam, MD, PhDii,
- Pedro Brugada, MD, PhDe,h,
- Vincent Probst, MD, PhDd,e and
- Bernard Belhassen, MDa,b,∗ (, )@SouraskyMedCtr
- aDepartment of Cardiology, Tel Aviv Sourasky Medical Center, Tel Aviv, Israel
- bSackler School of Medicine, Tel Aviv University, Tel Aviv, Israel
- cDepartment of Cardiology, Leviev Heart Institute, The Chaim Sheba Medical Center, Tel Hashomer, Israel
- dL'institut du Thorax, Service de Cardiologie, CHU de Nantes, Nantes, France
- eEuropean Reference Network for Rare and Low Prevalence Complex Diseases of the Heart
- fPediatric Arrhythmias, Electrophysiology and Sudden Death Unit Cardiology, Department Hospital Sant Joan de Déu, Barcelona-Universitat de Barcelona, Barcelona, Spain
- gPediatric Electrophysiology, Cliniques Universitaires St. Luc, Brussels, Belgium
- hHeart Rhythm Management Centre, UZ-VUB, Brussels, Belgium
- iHôpital Cardiologique du Haut-Lévêque & Université Bordeaux, LIRYC Institute, Bordeaux, France
- jCardiovascular Center and Division of Cardiology, Department of Internal Medicine, National Taiwan University Hospital and National Taiwan University College of Medicine, Taipei, Taiwan
- kDivision of Cardiology, Department of Internal Medicine, College of Medicine, The Catholic University of Korea, Seoul, Korea
- lBeth Israel Deaconess Medical Center, Boston, Massachusetts
- mCardiology and Vascular Disease Division, Rennes University Health Centre, Rennes, France
- nHeart Centre AMC, Department of Clinical and Experimental Cardiology, AMC, University of Amsterdam, Amsterdam, the Netherlands
- oDepartment of Internal Medicine J, Tel-Aviv Medical Center, Tel Aviv, Israel
- pCardiovascular Sciences, St. George's University of London and Cardiology Clinical Academic Group St. George's University Hospitals NHS Foundation Trust, London, United Kingdom
- qService de Cardiologie et CNMR Maladies Cardiaques Héréditaires Rares, Hôpital Bichat, Paris, and Université Paris Diderot, Sorbonne, Paris, France
- rDivision of Cardiology, University of Torino, Department of Medical Sciences, Città della Salute e della Scienza Hospital, Torino, Italy
- sDepartment of Cardiology, the First Affiliated Hospital of Xiamen University, Xiamen, Fujian, China
- tThe Department of Cardiology, The Heart Centre, Copenhagen University Hospital, Rigshospitalet, Copenhagen, Denmark
- uDepartment of Forensic Medicine, Faculty of Medical Sciences, University of Copenhagen, Copenhagen, Denmark
- vHeart Rhythm Center, Tokyo Medical and Dental University, Tokyo, Japan
- wDivision of Arrhythmia and Electrophysiology, Department of Cardiovascular Medicine, National Cerebral and Cardiovascular Center, Osaka, Japan
- xCardiology Department, Cardiovascular Institute, Hospital Clinic and IDIBAPS, Barcelona, Catalonia, Spain
- yMolecular Cardiology, Istituti Clinici Scientifici Maugeri IRCCS, Pavia, Italy
- zDivision of Cardiology, Hospital of Peschiera del Garda, Veneto, Italy
- aaCardiovascular Genetics Center, Medical Science Department, University of Girona-IDIBGI (CIBERCV) Cardiology Service, Hospital Josep Trueta, Girona, Spain
- bbDepartment of Cardiology, Erasme University Hospital, Université Libre de Bruxelles, Brussels, Belgium
- ccQuebec Heart and Lung Institute, Quebec City, Quebec, Canada
- ddRhythmology and Electrophysiology, Department of Cardiology, Hannover Medical School, Hannover, Germany
- eeDepartment of Cardiac, Thoracic and Vascular Sciences University of Padova, Padova, Italy
- ffDivision of Cardiology, Policlinico Casilino, Roma, Italy
- ggDivision of Cardiac Arrhythmia, Kansai Medical University Medical Center, Moriguchi, Japan
- hhLankenau Medical Center, Wynnewood, Pennsylvania
- iiDivision of Cardiology, Department of Internal Medicine, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea
- ↵∗Address for correspondence:
Dr. Bernard Belhassen, Department of Cardiology, Tel-Aviv Sourasky Medical Center, Weizman St 6, Tel-Aviv 64239, Israel.
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.
Dr. Arbelo has served on the Speakers Bureau of Biosense Webster. Dr. P. Brugada has served as a consultant for Biotronik. All other authors have reported that they have no relationships relevant to the contents of this paper to disclose.
Listen to this manuscript's audio summary by Editor-in-Chief Dr. Valentin Fuster on JACC.org.
- Received November 2, 2018.
- Revision received December 29, 2018.
- Accepted January 8, 2019.
- 2019 American College of Cardiology Foundation
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