ArticleJournal of the American College of Cardiology2019
Characterization and Management of Arrhythmic Events in Young Patients With Brugada Syndrome.
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Article in Journal of the American College of Cardiology, 2019. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 30 papers, 5 of them syntheses that pooled it.
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30citing papers in PubMed, 5 pooled it
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30 citing papers in PubMed, 5 syntheses or guidelines pooled it, 68 citations in OpenAlex.
- Diagnosis and management of very rare primary arrhythmia syndromes in children and adults: a Clinical Consensus Statement of the European Heart Rhythm Association of the ESC and the Association of Cardiovascular Nursing & Allied Professions of the ESC, endorsed by the Association for European Paediatric and Congenital Cardiology.Europace : European pacing, arrhythmias, and cardiac electrophysiology : journal of the working groups on cardiac pacing, arrhythmias, and cardiac cellular electrophysiology of the European Society of Cardiology · 2026Guideline
- The diagnostic role of pharmacological provocation testing in cardiac electrophysiology: a clinical consensus statement of the European Heart Rhythm Association and the European Association of Percutaneous Cardiovascular Interventions (EAPCI) of the ESC, the ESC Working Group on Cardiovascular Pharmacotherapy, the Association of European Paediatric and Congenital Cardiology (AEPC), the Paediatric & Congenital Electrophysiology Society (PACES), the Heart Rhythm Society (HRS), the Asia Pacific Heart Rhythm Society (APHRS), and the Latin American Heart Rhythm Society (LAHRS).Europace : European pacing, arrhythmias, and cardiac electrophysiology : journal of the working groups on cardiac pacing, arrhythmias, and cardiac cellular electrophysiology of the European Society of Cardiology · 2025Guideline
- Brazilian Guideline for Exercise Testing in Children and Adolescents - 2024.Arquivos brasileiros de cardiologia · 2024Guideline
- Electrocardiographic Markers Indicating Right Ventricular Outflow Tract Conduction Delay as a Predictor of Major Arrhythmic Events in Patients With Brugada Syndrome: A Systematic Review and Meta-Analysis.Frontiers in cardiovascular medicine · 2022Pooled it
- Standard ECG in Brugada Syndrome as a Marker of Prognosis: From Risk Stratification to Pathophysiological Insights.Journal of the American Heart Association · 2021Pooled it
- The History of the Precordial Early Repolarization and Sudden Death Syndrome, Lately Named Brugada Syndrome.Journal of clinical medicine · 2026Review
- Contemporary Perspectives on J-Wave Syndromes: An Expert Consensus Statement.Journal of arrhythmia · 2026Article
- Epidemiology of paediatric inherited arrhythmogenic diseases under "Real World" conditions: findings from a 10-year longitudinal study in Eastern Austria.European journal of pediatrics · 2025Article
- Beyond the type 1 pattern: comprehensive risk stratification in Brugada syndrome.Journal of interventional cardiac electrophysiology : an international journal of arrhythmias and pacing · 2025Review
- Fever, Syncope, and the Brugada Dilemma: Navigating the Complexities of ICD Decision-Making in an Atypical Presentation.Case reports in cardiology · 2025Article
- Brugada Syndrome: Focus for the General Pediatrician.Children (Basel, Switzerland) · 2024Review
- Contributions of Israel to the field of clinical cardiac electrophysiology and implantable devices.Heart rhythm O2 · 2024Article
- Inherited Arrhythmias in the Pediatric Population: An Updated Overview.Medicina (Kaunas, Lithuania) · 2024Review
- Assessment of Sudden Cardiac Death Risk in Pediatric Primary Electrical Disorders: A Comprehensive Overview.Diagnostics (Basel, Switzerland) · 2023Review
- Use, misuse, and pitfalls of the drug challenge test in the diagnosis of the Brugada syndrome.European heart journal · 2023Review
- Genetic testing in children with Brugada syndrome: results from a large prospective registry.Europace : European pacing, arrhythmias, and cardiac electrophysiology : journal of the working groups on cardiac pacing, arrhythmias, and cardiac cellular electrophysiology of the European Society of Cardiology · 2023Article
- Screening, diagnosis and follow-up of Brugada syndrome in children: a Dutch expert consensus statement.Netherlands heart journal : monthly journal of the Netherlands Society of Cardiology and the Netherlands Heart Foundation · 2023Review
- Risk stratification of sudden cardiac death in asymptomatic female Brugada syndrome patients: A literature review.Annals of noninvasive electrocardiology : the official journal of the International Society for Holter and Noninvasive Electrocardiology, Inc · 2023Review
- Ethnic and racial differences in Asian populations with ion channelopathies associated with sudden cardiac death.Frontiers in cardiovascular medicine · 2023Review
- Clinical Genetics of Inherited Arrhythmogenic Disease in the Pediatric Population.Biomedicines · 2022Review
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Authors and funding
49 authors at 20 institutions in 16 countries.
Yoav MichowitzDepartment of Cardiology, Tel Aviv Sourasky Medical Center, Tel Aviv, Israel; Sackler School of Medicine, Tel Aviv University, Tel Aviv, Israel.
Anat MilmanSackler School of Medicine, Tel Aviv University, Tel Aviv, Israel; Department of Cardiology, Leviev Heart Institute, The Chaim Sheba Medical Center, Tel Hashomer, Israel.
Antoine AndorinL'institut du Thorax, Service de Cardiologie, CHU de Nantes, Nantes, France; European Reference Network for Rare and Low Prevalence Complex Diseases of the Heart.
Georgia Sarquella-BrugadaPediatric Arrhythmias, Electrophysiology and Sudden Death Unit Cardiology, Department Hospital Sant Joan de Déu, Barcelona-Universitat de Barcelona, Barcelona, Spain.
M Cecilia Gonzalez CorciaPediatric Electrophysiology, Cliniques Universitaires St. Luc, Brussels, Belgium.
Jean-Baptiste GourraudL'institut du Thorax, Service de Cardiologie, CHU de Nantes, Nantes, France; European Reference Network for Rare and Low Prevalence Complex Diseases of the Heart.
Giulio ConteHeart Rhythm Management Centre, UZ-VUB, Brussels, Belgium.
Frederic SacherHôpital Cardiologique du Haut-Lévêque & Université Bordeaux, LIRYC Institute, Bordeaux, France.
Jimmy J M JuangCardiovascular Center and Division of Cardiology, Department of Internal Medicine, National Taiwan University Hospital and National Taiwan University College of Medicine, Taipei, Taiwan.
Sung-Hwan KimDivision of Cardiology, Department of Internal Medicine, College of Medicine, The Catholic University of Korea, Seoul, Korea.
Eran LeshemDepartment of Cardiology, Tel Aviv Sourasky Medical Center, Tel Aviv, Israel; Beth Israel Deaconess Medical Center, Boston, Massachusetts.
Philippe MaboCardiology and Vascular Disease Division, Rennes University Health Centre, Rennes, France.
Pieter G PostemaEuropean 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.
Aviram HochstadtDepartment of Internal Medicine J, Tel-Aviv Medical Center, Tel Aviv, Israel.
Yanushi D WijeyeratneEuropean 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.
Isabelle DenjoyEuropean 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.
Carla GiustettoDivision of Cardiology, University of Torino, Department of Medical Sciences, Città della Salute e della Scienza Hospital, Torino, Italy.
Yuka MizusawaEuropean 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.
Zhengrong HuangDepartment of Cardiology, the First Affiliated Hospital of Xiamen University, Xiamen, Fujian, China.
Camilla H JespersenEuropean 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.
Shingo MaedaHeart Rhythm Center, Tokyo Medical and Dental University, Tokyo, Japan.
Yoshihide TakahashiHeart Rhythm Center, Tokyo Medical and Dental University, Tokyo, Japan.
Tsukasa KamakuraDivision of Arrhythmia and Electrophysiology, Department of Cardiovascular Medicine, National Cerebral and Cardiovascular Center, Osaka, Japan.
Takeshi AibaDivision of Arrhythmia and Electrophysiology, Department of Cardiovascular Medicine, National Cerebral and Cardiovascular Center, Osaka, Japan.
Elena ArbeloCardiology Department, Cardiovascular Institute, Hospital Clinic and IDIBAPS, Barcelona, Catalonia, Spain.
Andrea MazzantiEuropean Reference Network for Rare and Low Prevalence Complex Diseases of the Heart; Molecular Cardiology, Istituti Clinici Scientifici Maugeri IRCCS, Pavia, Italy.
Giuseppe AlloccaDivision of Cardiology, Hospital of Peschiera del Garda, Veneto, Italy.
Ramon BrugadaCardiovascular Genetics Center, Medical Science Department, University of Girona-IDIBGI (CIBERCV) Cardiology Service, Hospital Josep Trueta, Girona, Spain.
Ruben Casado-ArroyoDepartment of Cardiology, Erasme University Hospital, Université Libre de Bruxelles, Brussels, Belgium.
Jean ChampagneQuebec Heart and Lung Institute, Quebec City, Quebec, Canada.
Silvia G PrioriEuropean Reference Network for Rare and Low Prevalence Complex Diseases of the Heart; Molecular Cardiology, Istituti Clinici Scientifici Maugeri IRCCS, Pavia, Italy.
Christian VeltmannRhythmology and Electrophysiology, Department of Cardiology, Hannover Medical School, Hannover, Germany.
Pietro DeliseDivision of Cardiology, Hospital of Peschiera del Garda, Veneto, Italy.
Domenico CorradoEuropean Reference Network for Rare and Low Prevalence Complex Diseases of the Heart; Department of Cardiac, Thoracic and Vascular Sciences University of Padova, Padova, Italy.
Josep BrugadaCardiology Department, Cardiovascular Institute, Hospital Clinic and IDIBAPS, Barcelona, Catalonia, Spain.
Kengo F KusanoDivision of Arrhythmia and Electrophysiology, Department of Cardiovascular Medicine, National Cerebral and Cardiovascular Center, Osaka, Japan.
Kenzo HiraoHeart Rhythm Center, Tokyo Medical and Dental University, Tokyo, Japan.
Leonardo CaloDivision of Cardiology, Policlinico Casilino, Roma, Italy.
Masahiko TakagiDivision of Cardiac Arrhythmia, Kansai Medical University Medical Center, Moriguchi, Japan.
Jacob Tfelt-HansenEuropean 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.
Gan-Xin YanLankenau Medical Center, Wynnewood, Pennsylvania.
Fiorenzo GaitaDivision of Cardiology, University of Torino, Department of Medical Sciences, Città della Salute e della Scienza Hospital, Torino, Italy.
Antoine LeenhardtEuropean 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.
Elijah R BehrEuropean 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.
Arthur A M WildeEuropean 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.
Gi-Byoung NamDivision of Cardiology, Department of Internal Medicine, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea.
Pedro BrugadaEuropean Reference Network for Rare and Low Prevalence Complex Diseases of the Heart; Heart Rhythm Management Centre, UZ-VUB, Brussels, Belgium.
Vincent ProbstL'institut du Thorax, Service de Cardiologie, CHU de Nantes, Nantes, France; European Reference Network for Rare and Low Prevalence Complex Diseases of the Heart.
Bernard BelhassenDepartment of Cardiology, Tel Aviv Sourasky Medical Center, Tel Aviv, Israel; Sackler School of Medicine, Tel Aviv University, Tel Aviv, Israel. Electronic address: bblhass@gmail.com.
Hospital Sant Joan de Déu Barcelona · ESNational Cerebral and Cardiovascular Center · JPSt George's, University of London · GBTel Aviv University · ILTokyo Medical and Dental University · JPCasa di Cura Villa Garda · ITCentre Hospitalier Universitaire de Nantes · FRERN GUARD-Heart · NLIstituti Clinici Scientifici Maugeri · ITTel Aviv Sourasky Medical Center · ILUniversity of Copenhagen · DKArt Research Centre of the Slovak Academy of Sciences · SKBeth Israel Deaconess Medical Center · USCatholic University of Korea · KRCliniques Universitaires Saint-Luc · BEConsorci Institut D'Investigacions Biomediques August Pi I Sunyer · ESDélégation Paris 7 · FRElectrophysiology and Heart Modeling Institute · FRFirst Affiliated Hospital of Xiamen University · CNKansai Medical University · JP
Funding
No grant is acknowledged in the PubMed record.
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.
Indexed as
Arrhythmias, CardiacBrugada SyndromeHeart ArrestAblation TechniquesAdolescentAnti-Arrhythmia AgentsChildDefibrillators, ImplantableElectrocardiographyElectrophysiologic Techniques, CardiacFemaleHumansMaleMedical History TakingQuinidineRisk AssessmentAnti-Arrhythmia AgentsQuinidineablationadolescenceBrugada syndromepediatricquinidineSCN5A mutation
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