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

Eur Heart J. 2017 Jun 7;38(22):1756-1763. doi: 10.1093/eurheartj/ehx119.


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.

Keywords: Clinical characterization; Prognosis; Risk stratification; Sudden cardiac death; Brugada syndrome.

MeSH terms

  • Adolescent
  • Adult
  • Aged
  • Aged, 80 and over
  • Brugada Syndrome / complications*
  • Brugada Syndrome / mortality
  • Brugada Syndrome / therapy
  • 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
  • Sick Sinus Syndrome / mortality
  • Syncope / etiology
  • Syncope / mortality
  • Young Adult