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

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

Brugada syndrome SCN5A mutation ablation adolescence pediatric quinidine

Journal

Journal of the American College of Cardiology

ISSN: 1558-3597

Titre abrégé: J Am Coll Cardiol

Pays: United States

ID NLM: 8301365

Informations de publication

Date de publication:
16 04 2019

Historique:

received: 02 11 2018

revised: 29 12 2018

accepted: 08 01 2019

entrez: 13 4 2019

pubmed: 13 4 2019

medline: 25 2 2020

Statut: ppublish

Résumé

Information on young patients with Brugada syndrome (BrS) and arrhythmic events (AEs) is limited. The purpose of this study was to describe their characteristics and management as well as risk factors for AE recurrence. 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). 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. 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.

Sections du résumé

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.

Identifiants

DOI: 10.1016/j.jacc.2019.01.048 PMID: 30975291

pubmed: 30975291

pii: S0735-1097(19)30590-X

doi: 10.1016/j.jacc.2019.01.048

pii:

doi:

Substances chimiques

Anti-Arrhythmia Agents 0

Quinidine ITX08688JL

Types de publication

Journal Article

Langues

eng

Sous-ensembles de citation

Pagination

1756-1765

Commentaires et corrections

Type : CommentIn