Characterization and Management of Arrhythmic Events in Young Patients With Brugada Syndrome |
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| Authors: | 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 Bernard Belhassen |
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| Affiliation: | 1. Department of Cardiology, Tel Aviv Sourasky Medical Center, Tel Aviv, Israel;2. Sackler School of Medicine, Tel Aviv University, Tel Aviv, Israel;3. Department of Cardiology, Leviev Heart Institute, The Chaim Sheba Medical Center, Tel Hashomer, Israel;4. L''institut du Thorax, Service de Cardiologie, CHU de Nantes, Nantes, France;5. European Reference Network for Rare and Low Prevalence Complex Diseases of the Heart;6. Pediatric Arrhythmias, Electrophysiology and Sudden Death Unit Cardiology, Department Hospital Sant Joan de Déu, Barcelona-Universitat de Barcelona, Barcelona, Spain;7. Pediatric Electrophysiology, Cliniques Universitaires St. Luc, Brussels, Belgium;8. Heart Rhythm Management Centre, UZ-VUB, Brussels, Belgium;9. Hôpital Cardiologique du Haut-Lévêque & Université Bordeaux, LIRYC Institute, Bordeaux, France;10. Cardiovascular Center and Division of Cardiology, Department of Internal Medicine, National Taiwan University Hospital and National Taiwan University College of Medicine, Taipei, Taiwan;11. Division of Cardiology, Department of Internal Medicine, College of Medicine, The Catholic University of Korea, Seoul, Korea;12. Beth Israel Deaconess Medical Center, Boston, Massachusetts;13. Cardiology and Vascular Disease Division, Rennes University Health Centre, Rennes, France;14. Heart Centre AMC, Department of Clinical and Experimental Cardiology, AMC, University of Amsterdam, Amsterdam, the Netherlands;15. Department of Internal Medicine J, Tel-Aviv Medical Center, Tel Aviv, Israel;p. Cardiovascular Sciences, St. George''s University of London and Cardiology Clinical Academic Group St. George''s University Hospitals NHS Foundation Trust, London, United Kingdom;q. Service de Cardiologie et CNMR Maladies Cardiaques Héréditaires Rares, Hôpital Bichat, Paris, and Université Paris Diderot, Sorbonne, Paris, France;r. Division of Cardiology, University of Torino, Department of Medical Sciences, Città della Salute e della Scienza Hospital, Torino, Italy;s. Department of Cardiology, the First Affiliated Hospital of Xiamen University, Xiamen, Fujian, China;t. The Department of Cardiology, The Heart Centre, Copenhagen University Hospital, Rigshospitalet, Copenhagen, Denmark;u. Department of Forensic Medicine, Faculty of Medical Sciences, University of Copenhagen, Copenhagen, Denmark;v. Heart Rhythm Center, Tokyo Medical and Dental University, Tokyo, Japan;w. Division of Arrhythmia and Electrophysiology, Department of Cardiovascular Medicine, National Cerebral and Cardiovascular Center, Osaka, Japan;x. Cardiology Department, Cardiovascular Institute, Hospital Clinic and IDIBAPS, Barcelona, Catalonia, Spain;y. Molecular Cardiology, Istituti Clinici Scientifici Maugeri IRCCS, Pavia, Italy;z. Division of Cardiology, Hospital of Peschiera del Garda, Veneto, Italy;11. Cardiovascular Genetics Center, Medical Science Department, University of Girona-IDIBGI (CIBERCV) Cardiology Service, Hospital Josep Trueta, Girona, Spain;22. Department of Cardiology, Erasme University Hospital, Université Libre de Bruxelles, Brussels, Belgium;33. Quebec Heart and Lung Institute, Quebec City, Quebec, Canada;44. Rhythmology and Electrophysiology, Department of Cardiology, Hannover Medical School, Hannover, Germany;55. Department of Cardiac, Thoracic and Vascular Sciences University of Padova, Padova, Italy;66. Division of Cardiology, Policlinico Casilino, Roma, Italy;77. Division of Cardiac Arrhythmia, Kansai Medical University Medical Center, Moriguchi, Japan;88. Lankenau Medical Center, Wynnewood, Pennsylvania;99. Division of Cardiology, Department of Internal Medicine, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea |
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| Abstract: | BackgroundInformation on young patients with Brugada syndrome (BrS) and arrhythmic events (AEs) is limited.ObjectivesThe purpose of this study was to describe their characteristics and management as well as risk factors for AE recurrence.MethodsA 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).ResultsPatients’ 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.ConclusionsYoung 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. |
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| Keywords: | ablation adolescence Brugada syndrome pediatric quinidine ACA aborted cardiac arrest AE arrhythmic event AFL atrial flutter AT atrial tachycardia BrS Brugada syndrome ECG electrocardiogram EPS electrophysiological study ICD implantable cardioverter-defibrillator SCD sudden cardiac death SND sinus node dysfunction VF ventricular fibrillation VT ventricular tachycardia |
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