Characterization and Management of Arrhythmic Events in Young Patients With Brugada Syndrome
Journal
Journal of the American College of Cardiology
Journal Volume
73
Journal Issue
14
Pages
1756-1765
Date Issued
2019
Author(s)
Michowitz Y.
Milman A.
Andorin A.
Sarquella-Brugada G.
Gonzalez Corcia M.C.
Gourraud J.-B.
Conte G.
Sacher F.
Kim S.-H.
Leshem E.
Mabo P.
Postema P.G.
Hochstadt A.
Wijeyeratne Y.D.
Denjoy I.
Giustetto C.
Mizusawa Y.
Huang Z.
Jespersen C.H.
Maeda S.
Takahashi Y.
Kamakura T.
Aiba T.
Arbelo E.
Mazzanti A.
Allocca G.
Brugada R.
Casado-Arroyo R.
Champagne J.
Priori S.G.
Veltmann C.
Delise P.
Corrado D.
Brugada J.
Kusano K.F.
Hirao K.
Calo L.
Takagi M.
Tfelt-Hansen J.
Yan G.-X.
Gaita F.
Leenhardt A.
Behr E.R.
Wilde A.A.M.
Nam G.-B.
Brugada P.
Probst V.
Belhassen B.
Abstract
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. ? 2019 American College of Cardiology Foundation
Subjects
ablation; adolescence; Brugada syndrome; pediatric; quinidine; SCN5A mutation
SDGs
Other Subjects
quinidine; antiarrhythmic agent; adolescent; adult; Article; Brugada syndrome; cardiovascular risk; Caucasian; child; clinical feature; cohort analysis; controlled study; electrocardiogram; faintness; family history; female; fever; follow up; gene; gene mutation; groups by age; heart arrest; heart arrhythmia; heart atrium arrhythmia; heart electrophysiology; heart ventricle conduction; heart ventricle fibrillation; human; major clinical study; male; priority journal; recurrent disease; SCN5A gene; sinus node disease; sudden cardiac death; ablation therapy; anamnesis; Brugada syndrome; electrocardiography; faintness; genetics; heart arrest; heart arrhythmia; heart function test; implantable cardioverter defibrillator; pathophysiology; procedures; risk assessment; risk factor; secondary prevention; young adult; Ablation Techniques; Adolescent; Anti-Arrhythmia Agents; Arrhythmias, Cardiac; Brugada Syndrome; Child; Defibrillators, Implantable; Electrocardiography; Electrophysiologic Techniques, Cardiac; Female; Heart Arrest; Humans; Male; Medical History Taking; Quinidine; Risk Assessment; Risk Factors; Secondary Prevention; Syncope; Young Adult
Publisher
Elsevier USA
Type
journal article