We recently observed a case of BrS characterized by prominent J waves in the inferolateral leads of the 12-lead ECG and electrical storms (ESs). 7 Case-control studies have described a close association between J waves, a sign of early repolarization (ER), and idiopathic VF. [8][9][10] The presence of J waves in patients presenting with BrS may also be a predictor of poor prognosis. 6,[11][12][13] The purpose of this multicenter study was to evaluate the characteristics of patients with BrS and ES, with a special attention to the presence of J waves.
Methods
Study PopulationWe retrospectively identified 22 men at 8 Japanese medical institutions, who presented with BrS and ES, defined as ≥3 episodes of VF/d. BrS was diagnosed according to the following currently accepted criteria 2,6,[11][12][13][14] : (1 Original ArticleBackground-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 followup, 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. (Circ Arrhythm Electrophysiol. 2014;7:1122-1128.)