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

J Am Coll Cardiol. 2019 Apr 16;73(14):1756-1765. doi: 10.1016/j.jacc.2019.01.048.


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.

Keywords: Brugada syndrome; SCN5A mutation; ablation; adolescence; pediatric; quinidine.

MeSH terms

  • Ablation Techniques / methods
  • Adolescent
  • Anti-Arrhythmia Agents / therapeutic use
  • Arrhythmias, Cardiac* / diagnosis
  • Arrhythmias, Cardiac* / epidemiology
  • Arrhythmias, Cardiac* / genetics
  • Arrhythmias, Cardiac* / prevention & control
  • Brugada Syndrome* / diagnosis
  • Brugada Syndrome* / epidemiology
  • Brugada Syndrome* / physiopathology
  • Brugada Syndrome* / therapy
  • Child
  • Defibrillators, Implantable / statistics & numerical data
  • Electrocardiography / methods
  • Electrophysiologic Techniques, Cardiac / methods
  • Female
  • Heart Arrest* / diagnosis
  • Heart Arrest* / prevention & control
  • Humans
  • Male
  • Medical History Taking / statistics & numerical data
  • Quinidine / therapeutic use*
  • Risk Assessment / methods*
  • Risk Factors
  • Secondary Prevention / methods*
  • Syncope / diagnosis
  • Syncope / epidemiology
  • Syncope / etiology
  • Young Adult


  • Anti-Arrhythmia Agents
  • Quinidine