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

Research output: Contribution to journalArticlepeer-review

105 Citations (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.

Original languageEnglish
Pages (from-to)1756-1763
Number of pages8
JournalEuropean Heart Journal
Issue number22
Publication statusPublished - 7 Jun 2017

Bibliographical note

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


  • Adolescent
  • Adult
  • Aged
  • Aged, 80 and over
  • Brugada Syndrome/complications
  • Child
  • Child, Preschool
  • Death, Sudden, Cardiac/etiology
  • Defibrillators, Implantable
  • Disease-Free Survival
  • Electrocardiography
  • Electrophysiologic Techniques, Cardiac
  • Female
  • Humans
  • Infant
  • Male
  • Middle Aged
  • Pedigree
  • Prognosis
  • Prospective Studies
  • Risk Assessment/methods
  • Risk Factors
  • Sex Distribution
  • Sick Sinus Syndrome/etiology
  • Syncope/etiology
  • Young Adult


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