Electrical storm in patients with brugada syndrome is associated with early repolarization

Circ Arrhythm Electrophysiol. 2014 Dec;7(6):1122-8. doi: 10.1161/CIRCEP.114.001806. Epub 2014 Sep 14.


Background: Electrical storms (ESs) in patients with Brugada syndrome (BrS) are rare though potentially lethal.

Methods and results: We studied 22 men with BrS and ES, defined as ≥3 episodes/d of ventricular fibrillation (VF) and compared their characteristics with those of 110 age-matched, control men with BrS without ES. BrS was diagnosed by a spontaneous or drug-induced type 1 pattern on the ECG in the absence of structural heart disease. Early repolarization (ER) was diagnosed by J waves, ie, >0.1 mV notches or slurs of the terminal portion of the QRS complex. The BrS ECG pattern was provoked with pilsicainide. A spontaneous type I ECG pattern, J waves, and horizontal/descending ST elevation were found, respectively, in 77%, 36%, and 88% of patients with ES, versus 28% (P<0.0001), 9% (P=0.003), and 60% (P=0.06) of controls. The J-wave amplitude was significantly higher in patients with than without ES (P=0.03). VF occurred during undisturbed sinus rhythm in 14 of 19 patients (74%), and ES were controlled by isoproterenol administration. All patients with ES received an implantable cardioverter defibrillator and over a 6.0±5.4 years follow-up, the prognosis of patients with ES was significantly worse than that of patients without ES. Bepridil was effective in preventing VF in 6 patients.

Conclusions: A high prevalence of ER was found in a subgroup of patients with BrS associated with ES. ES appeared to be suppressed by isoproterenol or quinidine, whereas bepridil and quinidine were effective in the long-term prevention of VF in the highest-risk patients.

Keywords: Brugada syndrome; bepridil; electrocardiography; isoproterenol; ventricular fibrillation.

Publication types

  • Multicenter Study

MeSH terms

  • Action Potentials
  • Adult
  • Aged
  • Anti-Arrhythmia Agents / therapeutic use
  • Brugada Syndrome / complications*
  • Brugada Syndrome / diagnosis
  • Brugada Syndrome / physiopathology
  • Brugada Syndrome / therapy
  • Defibrillators, Implantable
  • Disease-Free Survival
  • Electric Countershock / instrumentation
  • Electrocardiography
  • Heart Conduction System / drug effects
  • Heart Conduction System / physiopathology*
  • Humans
  • Infant
  • Japan
  • Kaplan-Meier Estimate
  • Male
  • Middle Aged
  • Predictive Value of Tests
  • Recurrence
  • Retrospective Studies
  • Risk Factors
  • Time Factors
  • Treatment Outcome
  • Ventricular Fibrillation / diagnosis
  • Ventricular Fibrillation / etiology*
  • Ventricular Fibrillation / physiopathology
  • Ventricular Fibrillation / prevention & control
  • Young Adult


  • Anti-Arrhythmia Agents