A score model to predict risk of events in patients with Brugada Syndrome

Juan Sieira, Giulio Conte, Giuseppe Ciconte, Gian-Battista Chierchia, Ruben Casado-Arroyo, Giannis Baltogiannis, Giacomo Di Giovanni, Yukio Saitoh, Justo Juliá, Giacomo Mugnai, Mark La Meir, Francis Wellens, Jens Czapla, Gudrun Pappaert, Carlo de Asmundis, Pedro Brugada

Onderzoeksoutput: Article

79 Citaten (Scopus)


Aims: Risk stratification in Brugada Syndrome (BS) remains challenging. Arrhythmic events can occur life-long and studies with long follow-ups are sparse. The aim of our study was to investigate long-term prognosis and risk stratification of BS patients.

Methods and results: A single centre consecutive cohort of 400 BS patients was included and analysed. Mean age was 41.1 years, 78 patients (19.5%) had a spontaneous type I electrocardiogram (ECG). Clinical presentation was aborted sudden cardiac death (SCD) in 20 patients (5.0%), syncope in 111 (27.8%) and asymptomatic in 269 (67.3%). Familial antecedents of SCD were found in 184 individuals (46.0%), in 31 (7.8%) occurred in first-degree relatives younger than 35 years. An implantable cardioverter defibrillator (ICD) was placed in 176 (44.0%). During a mean follow-up of 80.7 months, 34 arrhythmic events occurred (event rate: 1.4% year). Variables significantly associated to events were: presentation as aborted SCD (Hazard risk [HR] 20.0), syncope (HR 3.7), spontaneous type I (HR 2.7), male gender (HR 2.7), early SCD in first-degree relatives (HR 2.9), SND (HR 5.0), inducible VA (HR 4.7) and proband status (HR 2.1). A score including ECG pattern, early familial SCD antecedents, inducible electrophysiological study, presentation as syncope or as aborted SCD and SND had a predictive performance of 0.82. A score greater than 2 conferred a 5-year event probability of 9.2%.

Conclusions: BS patients remain at risk many years after diagnosis. Early SCD in first-degree relatives and SND are risk factors for arrhythmic events. A simple risk score might help in the stratification and management of BS patients.

Originele taal-2English
Pagina's (van-tot)1756-1763
Aantal pagina's8
TijdschriftEuropean Heart Journal
Nummer van het tijdschrift22
StatusPublished - 7 jun 2017

Bibliografische nota

Published on behalf of the European Society of Cardiology. All rights reserved. © The Author 2017. For permissions, please email: journals.permissions@oup.com.


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