A Multicenter External Validation of a Score Model to Predict Risk of Events in Patients With Brugada Syndrome

Area of Science:

• Cardiology
• Electrophysiology
• Medical Informatics

Background:

• Brugada syndrome poses a risk of sudden cardiac death.
• A multivariate risk score by Sieira et al. (2017) showed promise but requires independent validation.

Purpose of the Study:

• To independently validate the Sieira risk score model for predicting sudden death in Brugada syndrome patients.
• To assess the clinical utility of the Sieira score for primary prevention strategies.

Main Methods:

• Retrospective analysis of 192 Brugada syndrome patient records from two UK centers.
• Patients' data were scored using the Sieira et al. model.
• Scores were compared against actual outcomes, including sudden cardiac death and cardiac arrest events.

Main Results:

• The Sieira score demonstrated low predictive performance with a C-index of 0.58.
• Sensitivity was 22.7% and specificity was 57.6%.
• An estimated 75% of cardiac arrest survivors in the cohort would have been ineligible for a defibrillator based on this score prior to their event.

Conclusions:

• The Sieira risk score model exhibits limited utility for primary prevention of sudden death in Brugada syndrome.
• Current risk stratification strategies may be insufficient, potentially leading to under-treatment.
• Independent validation is crucial for assessing the real-world applicability of risk prediction tools.