title: Long-term prognosis of patients with Brugada syndrome and an implanted cardioverter-defibrillator.
abstract: BACKGROUND
  Risk stratification of Brugada syndrome (BrS) remains controversial and recommendations for an implantable cardioverter-defibrillator (ICD) are not well established. The objective of this study was to assess the long-term prognosis of BrS patients with an ICD.
  
  
  RESULTS
  Of 55 consecutive patients with BrS assessed between April 2002 and October 2012, 36 (mean age 41.7 ± 13.8 years; 81.8% male) underwent ICD implantation. Nineteen (52.8%) were asymptomatic, 11 (30.6%) had previous history of syncope (arrhythmic cause suspected in eight) and six (16.7%) had aborted sudden cardiac death (SCD). Spontaneous type 1 electrocardiographic (ECG) pattern was present in 25 (69.4%) patients and electrophysiological study (EPS), performed in 26 (72.2%), was positive in 22 (84.6%). During a mean follow-up of 74 ± 40 months (>5 years in 72% of cases), seven (19.4%) patients had appropriate shocks (annual event rate 2.8%). These patients most frequently had aborted SCD (54.1% vs. 6.9%; p=0.008) and nonsustained ventricular tachycardia (57.1% vs. 10.3%; p=0.016) during follow-up. Spontaneous type 1 ECG pattern, syncope and positive EPS were not significantly associated with appropriate shocks. Multivariate analysis revealed that aborted SCD was an independent predictor of appropriate shocks (HR 8.07, 95% CI 1.58-41.2; p=0.012). ROC curve analysis demonstrated that aborted SCD had moderate discriminatory power to predict appropriate shocks (AUC 0.751), with sensitivity of 57% and specificity of 93%. In terms of ICD-related complications, eight (22.2%) patients had inappropriate shocks during the follow-up period, mainly due to sinus tachycardia (five patients); one patient had lead infection and another had a lead fracture.
  
  
  CONCLUSIONS
  In this population of BrS patients with ICD, the long-term rate of appropriate shocks was 2.8%/year. Aborted SCD was associated with a higher risk of appropriate shocks, whereas syncope and spontaneous type I ECG pattern did not predict this event.
authors.affiliation: Serviço de Cardiologia, Hospital de Santa Cruz, Centro Hospitalar de Lisboa Ocidental, Carnaxide, Portugal. Electronic address: heldores@hotmail.com.
authors.forename: Hélder
authors.initials: H
authors.lastname: Dores
authors.affiliation: Centro Cardiovascular, Hospital da Luz, Lisboa, Portugal.
authors.forename: Katya
authors.initials: K
authors.lastname: Reis Santos
authors.affiliation: Serviço de Cardiologia, Hospital de Santa Cruz, Centro Hospitalar de Lisboa Ocidental, Carnaxide, Portugal.
authors.forename: Pedro
authors.initials: P
authors.lastname: Adragão
authors.affiliation: Serviço de Cardiologia, Hospital de Santa Cruz, Centro Hospitalar de Lisboa Ocidental, Carnaxide, Portugal.
authors.forename: Francisco
authors.initials: F
authors.lastname: Moscoso Costa
authors.affiliation: Serviço de Cardiologia, Hospital de Santa Cruz, Centro Hospitalar de Lisboa Ocidental, Carnaxide, Portugal.
authors.forename: Pedro
authors.initials: P
authors.lastname: Galvão Santos
authors.affiliation: Serviço de Cardiologia, Hospital de Santa Cruz, Centro Hospitalar de Lisboa Ocidental, Carnaxide, Portugal.
authors.forename: Pedro
authors.initials: P
authors.lastname: Carmo
authors.affiliation: Serviço de Cardiologia, Hospital de Santa Cruz, Centro Hospitalar de Lisboa Ocidental, Carnaxide, Portugal.
authors.forename: Diogo
authors.initials: D
authors.lastname: Cavaco
authors.affiliation: Serviço de Cardiologia, Hospital de Santa Cruz, Centro Hospitalar de Lisboa Ocidental, Carnaxide, Portugal.
authors.forename: Francisco
authors.initials: F
authors.lastname: Bello Morgado
authors.affiliation: Serviço de Cardiologia, Hospital de Santa Cruz, Centro Hospitalar de Lisboa Ocidental, Carnaxide, Portugal.
authors.forename: Miguel
authors.initials: M
authors.lastname: Mendes
mesh_terms: D000328:Adult; D053840:Brugada Syndrome / Q000150:complications / Q000628:therapy*; D016757:Death, Sudden, Cardiac / Q000209:etiology / Q000517:prevention & control*; D017147:Defibrillators, Implantable; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D012189:Retrospective Studies; D013997:Time Factors; D055815:Young Adult
keywords: Appropriate shocks; Brugada syndrome; Cardiodesfibrilhador; Choques apropriados; Implantable cardioverter-defibrillator; Morte súbita; Prognosis; Prognóstico; Sudden cardiac death; Syncope; Síncope; Síndrome de Brugada