Characterization and Management of Arrhythmic Events in Young Patients With Brugada Syndrome
Por:
Michowitz Y, Milman A, Andorin A, Sarquella-Brugada G, Gonzalez Corcia MC, Gourraud JB, Conte G, Sacher F, Juang JJM, Kim SH, 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-Terradellas J, Kusano KF, Hirao K, Calo L, Takagi M, Tfelt-Hansen J, Yan GX, Gaita F, Leenhardt A, Behr ER, Wilde AAM, Nam GB, Brugada P, Probst V and Belhassen B
Publicada:
16 abr 2019
Categoría:
Cardiology and cardiovascular medicine
Resumen:
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. (C) 2019 by the American College of Cardiology Foundation.
Filiaciones:
Michowitz Y:
Department of Cardiology, Tel Aviv Sourasky Medical Center, Tel Aviv, Israel
Sackler School of Medicine, Tel Aviv University, Tel Aviv, Israel
Milman A:
Sackler School of Medicine, Tel Aviv University, Tel Aviv, Israel
Department of Cardiology, Leviev Heart Institute, The Chaim Sheba Medical Center, Tel Hashomer, Israel
Andorin A:
L'institut du Thorax, Service de Cardiologie, CHU de Nantes, Nantes, France
European Reference Network for Rare and Low Prevalence Complex Diseases of the Heart
Sarquella-Brugada G:
Pediatric Arrhythmias, Electrophysiology and Sudden Death Unit Cardiology, Department Hospital Sant Joan de Déu, Barcelona-Universitat de Barcelona, Barcelona, Spain
Gonzalez Corcia MC:
Pediatric Electrophysiology, Cliniques Universitaires St. Luc, Brussels, Belgium
Gourraud JB:
L'institut du Thorax, Service de Cardiologie, CHU de Nantes, Nantes, France
European Reference Network for Rare and Low Prevalence Complex Diseases of the Heart
Conte G:
Heart Rhythm Management Centre, UZ-VUB, Brussels, Belgium
Sacher F:
Hôpital Cardiologique du Haut-Lévêque & Université Bordeaux, LIRYC Institute, Bordeaux, France
Juang JJM:
Cardiovascular Center and Division of Cardiology, Department of Internal Medicine, National Taiwan University Hospital and National Taiwan University College of Medicine, Taipei, Taiwan
Kim SH:
Division of Cardiology, Department of Internal Medicine, College of Medicine, The Catholic University of Korea, Seoul, Korea
Leshem E:
Department of Cardiology, Tel Aviv Sourasky Medical Center, Tel Aviv, Israel
Beth Israel Deaconess Medical Center, Boston, Massachusetts
Mabo P:
Cardiology and Vascular Disease Division, Rennes University Health Centre, Rennes, France
Postema PG:
European Reference Network for Rare and Low Prevalence Complex Diseases of the Heart
Heart Centre AMC, Department of Clinical and Experimental Cardiology, AMC, University of Amsterdam, Amsterdam, the Netherlands
Hochstadt A:
Department of Internal Medicine J, Tel-Aviv Medical Center, Tel Aviv, Israel
Wijeyeratne YD:
European Reference Network for Rare and Low Prevalence Complex Diseases of the Heart
Cardiovascular Sciences, St. George's University of London and Cardiology Clinical Academic Group St. George's University Hospitals NHS Foundation Trust, London, United Kingdom
Denjoy I:
European Reference Network for Rare and Low Prevalence Complex Diseases of the Heart
Service de Cardiologie et CNMR Maladies Cardiaques Héréditaires Rares, Hôpital Bichat, Paris, and Université Paris Diderot, Sorbonne, Paris, France
Giustetto C:
Division of Cardiology, University of Torino, Department of Medical Sciences, Città della Salute e della Scienza Hospital, Torino, Italy
Mizusawa Y:
European Reference Network for Rare and Low Prevalence Complex Diseases of the Heart
Heart Centre AMC, Department of Clinical and Experimental Cardiology, AMC, University of Amsterdam, Amsterdam, the Netherlands
Huang Z:
Department of Cardiology, the First Affiliated Hospital of Xiamen University, Xiamen, Fujian, China
Jespersen CH:
European Reference Network for Rare and Low Prevalence Complex Diseases of the Heart
The Department of Cardiology, The Heart Centre, Copenhagen University Hospital, Rigshospitalet, Copenhagen, Denmark
Department of Forensic Medicine, Faculty of Medical Sciences, University of Copenhagen, Copenhagen, Denmark
Maeda S:
Heart Rhythm Center, Tokyo Medical and Dental University, Tokyo, Japan
Takahashi Y:
Heart Rhythm Center, Tokyo Medical and Dental University, Tokyo, Japan
Kamakura T:
Division of Arrhythmia and Electrophysiology, Department of Cardiovascular Medicine, National Cerebral and Cardiovascular Center, Osaka, Japan
Aiba T:
Division of Arrhythmia and Electrophysiology, Department of Cardiovascular Medicine, National Cerebral and Cardiovascular Center, Osaka, Japan
Arbelo E:
Cardiology Department, Cardiovascular Institute, Hospital Clinic and IDIBAPS, Barcelona, Catalonia, Spain
Mazzanti A:
European Reference Network for Rare and Low Prevalence Complex Diseases of the Heart
Molecular Cardiology, Istituti Clinici Scientifici Maugeri IRCCS, Pavia, Italy
Allocca G:
Division of Cardiology, Hospital of Peschiera del Garda, Veneto, Italy
Brugada R:
Cardiovascular Genetics Center, Medical Science Department, University of Girona-IDIBGI (CIBERCV) Cardiology Service, Hospital Josep Trueta, Girona, Spain
Casado-Arroyo R:
Department of Cardiology, Erasme University Hospital, Université Libre de Bruxelles, Brussels, Belgium
Champagne J:
Quebec Heart and Lung Institute, Quebec City, Quebec, Canada
Priori SG:
European Reference Network for Rare and Low Prevalence Complex Diseases of the Heart
Molecular Cardiology, Istituti Clinici Scientifici Maugeri IRCCS, Pavia, Italy
Veltmann C:
Rhythmology and Electrophysiology, Department of Cardiology, Hannover Medical School, Hannover, Germany
Delise P:
Division of Cardiology, Hospital of Peschiera del Garda, Veneto, Italy
Corrado D:
European Reference Network for Rare and Low Prevalence Complex Diseases of the Heart
Department of Cardiac, Thoracic and Vascular Sciences University of Padova, Padova, Italy
Brugada-Terradellas J:
Cardiology Department, Cardiovascular Institute, Hospital Clinic and IDIBAPS, Barcelona, Catalonia, Spain
Kusano KF:
Division of Arrhythmia and Electrophysiology, Department of Cardiovascular Medicine, National Cerebral and Cardiovascular Center, Osaka, Japan
Hirao K:
Heart Rhythm Center, Tokyo Medical and Dental University, Tokyo, Japan
Calo L:
Division of Cardiology, Policlinico Casilino, Roma, Italy
Takagi M:
Division of Cardiac Arrhythmia, Kansai Medical University Medical Center, Moriguchi, Japan
Tfelt-Hansen J:
European Reference Network for Rare and Low Prevalence Complex Diseases of the Heart
The Department of Cardiology, The Heart Centre, Copenhagen University Hospital, Rigshospitalet, Copenhagen, Denmark
Department of Forensic Medicine, Faculty of Medical Sciences, University of Copenhagen, Copenhagen, Denmark
Yan GX:
Lankenau Medical Center, Wynnewood, Pennsylvania
Gaita F:
Division of Cardiology, University of Torino, Department of Medical Sciences, Città della Salute e della Scienza Hospital, Torino, Italy
Leenhardt A:
European Reference Network for Rare and Low Prevalence Complex Diseases of the Heart
Service de Cardiologie et CNMR Maladies Cardiaques Héréditaires Rares, Hôpital Bichat, Paris, and Université Paris Diderot, Sorbonne, Paris, France
Behr ER:
European Reference Network for Rare and Low Prevalence Complex Diseases of the Heart
Cardiovascular Sciences, St. George's University of London and Cardiology Clinical Academic Group St. George's University Hospitals NHS Foundation Trust, London, United Kingdom
Wilde AAM:
European Reference Network for Rare and Low Prevalence Complex Diseases of the Heart
Heart Centre AMC, Department of Clinical and Experimental Cardiology, AMC, University of Amsterdam, Amsterdam, the Netherlands
Nam GB:
Division of Cardiology, Department of Internal Medicine, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea
Brugada P:
European Reference Network for Rare and Low Prevalence Complex Diseases of the Heart
Heart Rhythm Management Centre, UZ-VUB, Brussels, Belgium
Probst V:
L'institut du Thorax, Service de Cardiologie, CHU de Nantes, Nantes, France
European Reference Network for Rare and Low Prevalence Complex Diseases of the Heart
Belhassen B:
Department of Cardiology, Tel Aviv Sourasky Medical Center, Tel Aviv, Israel
Sackler School of Medicine, Tel Aviv University, Tel Aviv, Israel
Green Submitted, Bronze
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