Electrical Storm in Patients With Brugada Syndrome Is Associated With Early Repolarization

• Published in: Circulation. Arrhythmia and electrophysiology Vol. 7; no. 6; pp. 1122 - 1128
• Main Authors: Kaneko, Yoshiaki, Horie, Minoru, Niwano, Shinichi, Kusano, Kengo F, Takatsuki, Seiji, Kurita, Takashi, Mitsuhashi, Takeshi, Nakajima, Tadashi, Irie, Tadanobu, Hasegawa, Kanae, Noda, Takashi, Kamakura, Shiro, Aizawa, Yoshiyasu, Yasuoka, Ryobun, Torigoe, Katsumi, Suzuki, Hiroshi, Ohe, Toru, Shimizu, Akihiko, Fukuda, Keiichi, Kurabayashi, Masahiko, Aizawa, Yoshifusa
• Format: Journal Article
• Language: English
• Published: United States American Heart Association, Inc 01.12.2014
• Subjects: 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; Japan; ventricular fibrillation; isoproterenol; Brugada syndrome; bepridil; electrocardiography
• ISSN: 1941-3149; 1941-3084
• DOI: 10.1161/CIRCEP.114.001806

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.