Is the Sieira Risk Score Useful for Predicting Sudden Death in Brugada Syndrome?

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Researchers conducted an evaluation to validate whether the Sieira risk score model was effective for predicting risk of sudden cardiac death in Brugada syndrome.

An independent study failed to validate the clinical utility of a multivariate risk score for sudden cardiac death in Brugada syndrome. The findings were published in the American Journal of Cardiology.

For the study, researchers used the Sieira risk score to retrospectively assess patients (N=192) with Brugada syndrome, who were cared for at Imperial College Healthcare NHS Trust and University Hospital of Wales in the United Kingdom between 2004 and 2019. Clinical outcomes at an average follow-up of 5.1±2.76 years were compared with predicted risk.

Patients from the Imperial (n=124) and Wales (n=68) cohorts had a mean age of 48.1 and 45.5 years; 59.7% and 55.9% were men; 46.0% and 41.2% had a family history of sudden cardiac death; 31.5% and 35.3% had syncope; 60.5% and 48.5% had proband status; and 35.5% and 35.3% had an implantable cardio-verter defibrillator (ICD) device, respectively.

Compared with the original Sieira study, this current patient population differed significantly by age (P <.01), proportion of family history with sudden cardiac death at <35 years (P <.01), electrophysiologic inducibility (P <.01), proband status (P <.01), spontaneous type 1 Brugada pattern (P <.01), and previous aborted sudden cardiac death (P =.01).

The overall Sieira scores did not differ significantly between this study cohort (1.77) compared with the original Sieira study (1.57; P =.12).

Sudden cardiac death occurred among 20 individuals. Among the present study cohort, the investigators found a high number of false positives and negatives, giving the Sieira score a sensitivity of 25.0% (95% CI, 0.63%-80.6%) and specificity of 58.3% (95% CI, 47.0%-69.0%).

This analysis was limited by the differential patient characteristics between the original and current studies. However, these differences did not impact the average Sieira score.

This independent analysis failed to validate the clinical utility of the Sieira risk score for predicting sudden cardiac death among patients with Brugada syndrome.

“Patients and clinicians must be well-counseled on the performance of modern risk stratification before planning ICD implantation. Risk stratification in Brugada syndrome continues to be a challenge,” the researchers said.


Chow J-J, Leong KMW, Yazdani M, et al. A Multicenter external validation of a score model to predict risk of events in patients with Brugada syndrome. Am J Cardiol. Published online October 2, 2021. doi:10.1016/j.amjcard.2021.08.035