Key result
BRUGADA-RISK score best predicts major arrhythmic events in Brugada syndrome with 0.81 AUC.
Why the study?
Risk stratification remains a major challenge in Brugada syndrome, particularly for predicting risk in asymptomatic or intermediate-risk individuals.
Although multivariable risk scores for Brugada syndrome improve risk stratification, they remain decision-support tools that do not replace clinical judgment or guideline-endorsed predictors like syncope and EPS inducibility.
May aid intermediate-risk stratification in Brugada syndrome; leaves open prospective validation against syncope and EPS.
Brugada Syndrome (BrS) is a rare but clinically significant inherited arrhythmia disorder characterized by a type 1 ECG pattern and an increased risk of sudden cardiac death (SCD). Since its first description in 1992, BrS has been the subject of intensive investigation, yet risk stratification remains one of its greatest challenges. While survivors of cardiac arrest and patients with documented ventricular fibrillation (VF) are clear candidates for implantable cardioverter-defibrillators (ICDs), predicting risk in asymptomatic or intermediate-risk individuals is less straightforward. Over the past two decades, multiple risk scores have been developed—including the Sieira, Shanghai, BRUGADA-RISK, and PAT—each integrating combinations of clinical, ECG, electrophysiological study (EPS), and genetic data. Performance metrics vary, with C-statistics ranging from 0.70 to 0.82 in derivation cohorts, but external validation has often been limited. Importantly, current ESC and AHA/ACC guidelines only endorse syncope and EPS inducibility as validated predictors, reflecting the cautious stance of expert panels in the face of heterogeneous data. Nonetheless, the emergence of structured risk models has improved our ability to stratify intermediate-risk patients and stimulated further innovation. Looking ahead, opportunities lie in integrating artificial intelligence applied to raw ECG waveforms, wearable technology for dynamic monitoring, advanced cardiac imaging biomarkers, and polygenic risk scores. Multinational collaboration and federated learning will be essential to overcome statistical fragility and ensure global applicability. Ultimately, BrS risk scores should be considered decision-support tools that enrich but do not replace clinical judgment. Shared decision-making remains central, particularly in asymptomatic patients where ICD implantation is not a clear-cut choice.
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Rattanawong et al. (2026) conducted a review in Brugada syndrome. Risk scores for Brugada syndrome provide incremental improvements in predictive performance, with the best performance demonstrated by the BRUGADA-RISK score (AUC 0.81).
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