Yoshiaki Kaneko1, Minoru Horie2, Shinichi Niwano2, Kengo F Kusano2, Seiji Takatsuki2, Takashi Kurita2, Takeshi Mitsuhashi2, Tadashi Nakajima2, Tadanobu Irie2, Kanae Hasegawa2, Takashi Noda2, Shiro Kamakura2, Yoshiyasu Aizawa2, Ryobun Yasuoka2, Katsumi Torigoe2, Hiroshi Suzuki2, Toru Ohe2, Akihiko Shimizu2, Keiichi Fukuda2, Masahiko Kurabayashi2, Yoshifusa Aizawa2. 1. 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. 2. 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.).
Abstract
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.
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.
Authors: Charles Antzelevitch; Gan-Xin Yan; Michael J Ackerman; Martin Borggrefe; Domenico Corrado; Jihong Guo; Ihor Gussak; Can Hasdemir; Minoru Horie; Heikki Huikuri; Changsheng Ma; Hiroshi Morita; Gi-Byoung Nam; Frederic Sacher; Wataru Shimizu; Sami Viskin; Arthur A M Wilde Journal: Europace Date: 2017-04-01 Impact factor: 5.214
Authors: Mu Chen; Dong-Zhu Xu; Adonis Z Wu; Shuai Guo; Juyi Wan; Dechun Yin; Shien-Fong Lin; Zhenhui Chen; Michael Rubart-von der Lohe; Thomas H Everett; Zhilin Qu; James N Weiss; Peng-Sheng Chen Journal: JCI Insight Date: 2018-11-15
Authors: Giulio Conte; Maria Luce Caputo; François Regoli; Tiziano Moccetti; Pedro Brugada; Angelo Auricchio Journal: Arrhythm Electrophysiol Rev Date: 2016-08