Beyond the type 1 pattern: comprehensive risk stratification in Brugada syndrome

Abstract

Brugada Syndrome (BrS) is an inherited cardiac ion channelopathy associated with an elevated risk of sudden cardiac death, particularly due to ventricular arrhythmias in structurally normal hearts. Affecting approximately 1 in 2,000 individuals, BrS is most prevalent among middle-aged males of Asian descent. Although diagnosis is based on the presence of a Type 1 electrocardiographic (ECG) pattern, either spontaneous or induced, accurately stratifying risk in asymptomatic and borderline patients remains a major clinical challenge. This review explores current and emerging approaches to BrS risk stratification, focusing on electrocardiographic, electrophysiological, imaging, and computational markers. Non-invasive ECG indicators such as the β-angle, fragmented QRS, S wave in lead I, early repolarisation, aVR sign, and transmural dispersion of repolarisation have demonstrated predictive value for arrhythmic events. Adjunctive tools like signal-averaged ECG, Holter monitoring, and exercise stress testing enhance diagnostic yield by capturing dynamic electrophysiological changes. In parallel, imaging modalities, particularly speckle-tracking echocardiography and cardiac magnetic resonance have revealed subclinical structural abnormalities in the right ventricular outflow tract and atria, challenging the paradigm of BrS as a purely electrical disorder. Invasive electrophysiological studies and substrate mapping have further clarified the anatomical basis of arrhythmogenesis, while risk scoring systems (e.g., Sieira, BRUGADA-RISK, PAT) and machine learning models offer new avenues for personalised risk assessment. Together, these advances underscore the importance of an integrated, multimodal approach to BrS risk stratification. Optimising these strategies is essential to guide implantable cardioverter-defibrillator decisions and improve outcomes in patients vulnerable to life-threatening arrhythmias.

Keywords: BrS, ventricular arrhythmia; Brugada syndrome; ICD; SCD, implantable cardioverter defibrillator; VA, sudden cardiac death.