title: A score model to predict risk of events in patients with Brugada Syndrome.
abstract: OBJECTIVE
  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.
  
  
  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.
authors.affiliation: Heart Rhythm Management Centre, UZ Brussel-VUB, Laarbeeklaan 101, 1090, Brussels, Belgium.
authors.forename: Juan
authors.initials: J
authors.lastname: Sieira
authors.affiliation: Heart Rhythm Management Centre, UZ Brussel-VUB, Laarbeeklaan 101, 1090, Brussels, Belgium.
authors.forename: Giulio
authors.initials: G
authors.lastname: Conte
authors.affiliation: Heart Rhythm Management Centre, UZ Brussel-VUB, Laarbeeklaan 101, 1090, Brussels, Belgium.
authors.forename: Giuseppe
authors.initials: G
authors.lastname: Ciconte
authors.affiliation: Heart Rhythm Management Centre, UZ Brussel-VUB, Laarbeeklaan 101, 1090, Brussels, Belgium.
authors.forename: Gian-Battista
authors.initials: GB
authors.lastname: Chierchia
authors.affiliation: Heart Rhythm Management Centre, UZ Brussel-VUB, Laarbeeklaan 101, 1090, Brussels, Belgium.
authors.forename: Ruben
authors.initials: R
authors.lastname: Casado-Arroyo
authors.affiliation: Heart Rhythm Management Centre, UZ Brussel-VUB, Laarbeeklaan 101, 1090, Brussels, Belgium.
authors.forename: Giannis
authors.initials: G
authors.lastname: Baltogiannis
authors.affiliation: Heart Rhythm Management Centre, UZ Brussel-VUB, Laarbeeklaan 101, 1090, Brussels, Belgium.
authors.forename: Giacomo
authors.initials: G
authors.lastname: Di Giovanni
authors.affiliation: Heart Rhythm Management Centre, UZ Brussel-VUB, Laarbeeklaan 101, 1090, Brussels, Belgium.
authors.forename: Yukio
authors.initials: Y
authors.lastname: Saitoh
authors.affiliation: Heart Rhythm Management Centre, UZ Brussel-VUB, Laarbeeklaan 101, 1090, Brussels, Belgium.
authors.forename: Justo
authors.initials: J
authors.lastname: Juliá
authors.affiliation: Heart Rhythm Management Centre, UZ Brussel-VUB, Laarbeeklaan 101, 1090, Brussels, Belgium.
authors.forename: Giacomo
authors.initials: G
authors.lastname: Mugnai
authors.affiliation: Cardiac Surgery Department, UZ Brussel-VUB, Laarbeeklaan 101, 1090, Brussels, Belgium.
authors.forename: Mark
authors.initials: M
authors.lastname: La Meir
authors.affiliation: Cardiac Surgery Department, UZ Brussel-VUB, Laarbeeklaan 101, 1090, Brussels, Belgium.
authors.forename: Francis
authors.initials: F
authors.lastname: Wellens
authors.affiliation: Cardiac Surgery Department, UZ Brussel-VUB, Laarbeeklaan 101, 1090, Brussels, Belgium.
authors.forename: Jens
authors.initials: J
authors.lastname: Czapla
authors.affiliation: Heart Rhythm Management Centre, UZ Brussel-VUB, Laarbeeklaan 101, 1090, Brussels, Belgium.
authors.forename: Gudrun
authors.initials: G
authors.lastname: Pappaert
authors.affiliation: Heart Rhythm Management Centre, UZ Brussel-VUB, Laarbeeklaan 101, 1090, Brussels, Belgium.
authors.forename: Carlo
authors.initials: C
authors.lastname: de Asmundis
authors.affiliation: Heart Rhythm Management Centre, UZ Brussel-VUB, Laarbeeklaan 101, 1090, Brussels, Belgium.
authors.forename: Pedro
authors.initials: P
authors.lastname: Brugada
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D053840:Brugada Syndrome / Q000150:complications* / Q000401:mortality / Q000628:therapy; D002648:Child; D002675:Child, Preschool; D016757:Death, Sudden, Cardiac / Q000209:etiology; D017147:Defibrillators, Implantable; D018572:Disease-Free Survival; D004562:Electrocardiography; D022062:Electrophysiologic Techniques, Cardiac; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D008875:Middle Aged; D010375:Pedigree; D011379:Prognosis; D011446:Prospective Studies; D018570:Risk Assessment / Q000379:methods; D012307:Risk Factors; D017678:Sex Distribution; D012804:Sick Sinus Syndrome / Q000209:etiology / Q000401:mortality; D013575:Syncope / Q000209:etiology / Q000401:mortality; D055815:Young Adult
keywords: Clinical characterization ; Prognosis ; Risk stratification ; Sudden cardiac death;  Brugada syndrome 