Characterization and Management of Arrhythmic Events in Young Patients With Brugada Syndrome

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 w...

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Detalles Bibliográficos
Autores: 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 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, Belhassen B
Tipo de recurso: artículo
Estado:Versión publicada
Fecha de publicación:2019
País:España
Institución:Fundació Sant Joan de Déu
Repositorio:r-FSJD. Repositorio Institucional de Producción Científica de la Fundació Sant Joan de Déu
OAI Identifier:oai:fsjd.fundanetsuite.com:p17444
Acceso en línea:https://fsjd.fundanetsuite.com/Publicaciones/ProdCientif/PublicacionFrw.aspx?id=17444
Access Level:acceso abierto
Palabra clave:ablation
adolescence
Brugada syndrome
pediatric
quinidine
SCN5A mutation
Descripción
Sumario: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.

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