• Yoshiaki Kaneko, Minoru Horie, Shinichi Niwano, Kengo F Kusano, Seiji Takatsuki, Takashi Kurita, Takeshi Mitsuhashi, Tadashi Nakajima, Tadanobu Irie, Kanae Hasegawa, Takashi Noda, Shiro Kamakura, Yoshiyasu Aizawa, Ryobun Yasuoka, Katsumi Torigoe, Hiroshi Suzuki, Toru Ohe, Akihiko Shimizu, Keiichi Fukuda, Masahiko Kurabayashi, and Yoshifusa Aizawa.
• From the Department of Medicine and Biological Science, Gunma University Graduate School of Medicine, Maebashi, Gunma, Japan (Y.K., T.N., T.I., M.K.); Department of Cardiovascular and Respiratory Medicine, Shiga University of Medical Science, Otsu, Shiga, Japan (M.H., K.H.); Department of Cardio-Angiology, Kitasato University School of Medicine, Sagamihara, Kanagawa, Japan (S.N.); Department of Cardiovascular Medicine, National Cerebral and Cardiovascular Center, Osaka, Japan (S.N.); (K.F.K., T.N., S.K.); Department of Cardiology, Keio University School of Medicine, Tokyo, Japan (S.T., Yoshiyasu A., K.F.); Department of Medicine, Division of Cardiology, Faculty of Medicine, Kinki University, Osaka (T.K., R.Y.); Division of Cardiovascular Medicine, Department of Medicine, Jichi Medical University School of Medicine, Tochigi, Japan (T.M.); Department of Pediatrics, Nagaoka Red Cross Hospital, Nagaoka, Japan (K.T.); Department of Pediatrics, Niigata University School of Medicine, Niigata, Japan (H.S.); Research Division, The Sakakibara Heart Institute of Okayama, Okayama, Japan (T.O.); Faculty of Health Sciences, Yamaguchi University Graduate School of Medicine, Yamaguchi, Japan (A.S.); Department of Research and Development, Tachikawa Medical Center, Nagaoka, Niigata, Japan (Yoshifusa A.). kanekoy@gunma-u.ac.jp.
• Circ Arrhythm Electrophysiol. 2014 Dec 1; 7 (6): 1122-8.

BackgroundElectrical storms (ESs) in patients with Brugada syndrome (BrS) are rare though potentially lethal.Methods And ResultsWe 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.ConclusionsA 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.© 2014 American Heart Association, Inc.

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