Brugada syndrome in the young: An assessment of risk factors predicting future events

Abstract

Brugada syndrome (BS), first described in 1992, is a disease that predisposes to sudden cardiac death (SCD) in healthy patients with the absence of structural heart disease.1 Patients present with a typical electrocardiographic (ECG) patterns characterized by coved-type ST-segment elevation in the right precordial leads (V1-V3), with or without right bundle branch block. These diagnostic ECG attributes may present spontaneously, or they may be undetectable until revealed by intravenous administration of a sodium channel blocker. Patients with BS typically develop symptoms in the fourth or fifth decade of life. Yet, BS has been described in children, as in the first article on this syndrome, and has been linked to SCD in younger cohorts. 1,2 In the last few years, growing evidence has suggested that BS can present in early childhood and manifest as ongoing rhythm abnormalities and SCD.3 - 6 Previous studies have identified an increased risk of sudden death in children affected by BS and described symptoms useful for prognosticating the disease.7 Additionally, our group has previously described the final outcomes of follow-up in patients of ,12 years of age (yo).8 The aim of the present study was to investigate risks factors related to BS in younger patient cohorts. To identify predictors for arrhythmic events and SCD, symptomatic and asymptomatic young BS patients were compared.