Outcome after implantation of a cardioverter-defibrillator in patients with Brugada syndrome: a multicenter study-part 2

Circulation. 2013 Oct 15;128(16):1739-47. doi: 10.1161/CIRCULATIONAHA.113.001941. Epub 2013 Aug 30.


Background: Implantable cardioverter-defibrillator indications in Brugada syndrome remain controversial, especially in asymptomatic patients. Previous outcome data are limited by relatively small numbers of patients or short follow-up durations. We report the outcome of patients with Brugada syndrome implanted with an implantable cardioverter-defibrillator in a large multicenter registry.

Methods and results: A total of 378 patients (310 male; age, 46±13 years) with a type 1 Brugada ECG pattern implanted with an implantable cardioverter-defibrillator (31 for aborted sudden cardiac arrest, 181 for syncope, and 166 asymptomatic) were included. Fifteen patients (4%) were lost to follow-up. During a mean follow-up of 77±42 months, 7 patients (2%) died (1 as a result of an inappropriate shock), and 46 patients (12%) had appropriate device therapy (5±5 shocks per patient). Appropriate device therapy rates at 10 years were 48% for patients whose implantable cardioverter-defibrillator indication was aborted sudden cardiac arrest, 19% for those whose indication was syncope, and 12% for the patients who were asymptomatic at implantation. At 10 years, rates of inappropriate shock and lead failure were 37% and 29%, respectively. Inappropriate shock occurred in 91 patients (24%; 4±4 shocks per patient) because of lead failure (n=38), supraventricular tachycardia (n=20), T-wave oversensing (n=14), or sinus tachycardia (n=12). Importantly, introduction of remote monitoring, programming a high single ventricular fibrillation zone (>210-220 bpm), and a long detection time were associated with a reduced risk of inappropriate shock.

Conclusions: Appropriate therapies are more prevalent in symptomatic Brugada syndrome patients but are not insignificant in asymptomatic patients (1%/y). Optimal implantable cardioverter-defibrillator programming and follow-up dramatically reduce inappropriate shock. However, lead failure remains a major problem in this population.

Keywords: Brugada syndrome; death, sudden, cardiac; defibrillators, implantable.

Publication types

  • Multicenter Study

MeSH terms

  • Adult
  • Aged
  • Brugada Syndrome / diagnosis
  • Brugada Syndrome / mortality*
  • Brugada Syndrome / therapy*
  • Death, Sudden, Cardiac / epidemiology*
  • Death, Sudden, Cardiac / prevention & control*
  • Defibrillators, Implantable / adverse effects
  • Defibrillators, Implantable / statistics & numerical data*
  • Electrocardiography
  • Female
  • Follow-Up Studies
  • Humans
  • Kaplan-Meier Estimate
  • Male
  • Middle Aged
  • Prevalence
  • Risk Factors
  • Treatment Outcome