{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,27]],"date-time":"2026-09-27T16:22:04Z","timestamp":1790526124626,"version":"4.1.0"},"reference-count":46,"publisher":"Ovid Technologies (Wolters Kluwer Health)","issue":"6","content-domain":{"domain":["www.ahajournals.org"],"crossmark-restriction":true},"short-container-title":["Circ: Arrhythmia and Electrophysiology"],"published-print":{"date-parts":[[2011,12]]},"abstract":"<jats:sec>\n            <jats:title>Background\u2014<\/jats:title>\n            <jats:p>Recently, we and others reported that early repolarization (J wave) is associated with idiopathic ventricular fibrillation. However, its clinical and genetic characteristics are unclear.<\/jats:p>\n          <\/jats:sec>\n          <jats:sec>\n            <jats:title>Methods and Results\u2014<\/jats:title>\n            <jats:p>\n              This study included 50 patients (44 men; age, 45\u00b117 years) with idiopathic ventricular fibrillation associated with early repolarization, and 250 age- and sex-matched healthy controls. All of the patients had experienced arrhythmia events, and 8 (16%) had a family history of sudden death. Ventricular fibrillation was inducible by programmed electric stimulation in 15 of 29 patients (52%). The heart rate was slower and the PR interval and QRS duration were longer in patients with idiopathic ventricular fibrillation than in controls. We identified nonsynonymous variants in\n              <jats:italic>SCN5A<\/jats:italic>\n              (resulting in A226D, L846R, and R367H) in 3 unrelated patients. These variants occur at residues that are highly conserved across mammals. His-ventricular interval was prolonged in all of the patients carrying an\n              <jats:italic>SCN5A<\/jats:italic>\n              mutation. Sodium channel blocker challenge resulted in an augmentation of early repolarization or development of ventricular fibrillation in all of 3 patients, but none was diagnosed with Brugada syndrome. In heterologous expression studies, all of the mutant channels failed to generate any currents. Immunostaining revealed a trafficking defect in A226D channels and normal trafficking in R367H and L846R channels.\n            <\/jats:p>\n          <\/jats:sec>\n          <jats:sec>\n            <jats:title>Conclusions\u2014<\/jats:title>\n            <jats:p>\n              We found reductions in heart rate and cardiac conduction and loss-of-function mutations in\n              <jats:italic>SCN5A<\/jats:italic>\n              in patients with idiopathic ventricular fibrillation associated with early repolarization. These findings support the hypothesis that decreased sodium current enhances ventricular fibrillation susceptibility.\n            <\/jats:p>\n          <\/jats:sec>","DOI":"10.1161\/circep.111.963983","type":"journal-article","created":{"date-parts":[[2011,10,26]],"date-time":"2011-10-26T02:01:15Z","timestamp":1319594475000},"page":"874-881","update-policy":"https:\/\/doi.org\/10.1161\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":144,"title":["Electrocardiographic Characteristics and\n            <i>SCN5A<\/i>\n            Mutations in Idiopathic Ventricular Fibrillation Associated With Early Repolarization"],"prefix":"10.1161","volume":"4","author":[{"given":"Hiroshi","family":"Watanabe","sequence":"first","affiliation":[{"name":"From the Division of Cardiology (H.W., Y.H., S.N., N.Y., A.S., K.O., M.C., Y.A.), Niigata University School of Medicine, Niigata; Division of Heart Rhythm Management (A.N.), Yokohama Rosai Hospital, Yokohama; Division of Cardiology (K.O., I.W.), Department of Medicine, Nihon University School of Medicine, Tokyo; Division of Arrhythmia and Electrophysiology (H.K., S.K., W.S.), Department of Cardiovascular Medicine, National Cerebral and Cardiovascular Center, Suita; Department of Molecular..."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Akihiko","family":"Nogami","sequence":"additional","affiliation":[{"name":"From the Division of Cardiology (H.W., Y.H., S.N., N.Y., A.S., K.O., M.C., Y.A.), Niigata University School of Medicine, Niigata; Division of Heart Rhythm Management (A.N.), Yokohama Rosai Hospital, Yokohama; Division of Cardiology (K.O., I.W.), Department of Medicine, Nihon University School of Medicine, Tokyo; Division of Arrhythmia and Electrophysiology (H.K., S.K., W.S.), Department of Cardiovascular Medicine, National Cerebral and Cardiovascular Center, Suita; Department of Molecular..."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kimie","family":"Ohkubo","sequence":"additional","affiliation":[{"name":"From the Division of Cardiology (H.W., Y.H., S.N., N.Y., A.S., K.O., M.C., Y.A.), Niigata University School of Medicine, Niigata; Division of Heart Rhythm Management (A.N.), Yokohama Rosai Hospital, Yokohama; Division of Cardiology (K.O., I.W.), Department of Medicine, Nihon University School of Medicine, Tokyo; Division of Arrhythmia and Electrophysiology (H.K., S.K., W.S.), Department of Cardiovascular Medicine, National Cerebral and Cardiovascular Center, Suita; Department of Molecular..."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hiro","family":"Kawata","sequence":"additional","affiliation":[{"name":"From the Division of Cardiology (H.W., Y.H., S.N., N.Y., A.S., K.O., M.C., Y.A.), Niigata University School of Medicine, Niigata; Division of Heart Rhythm Management (A.N.), Yokohama Rosai Hospital, Yokohama; Division of Cardiology (K.O., I.W.), Department of Medicine, Nihon University School of Medicine, Tokyo; Division of Arrhythmia and Electrophysiology (H.K., S.K., W.S.), Department of Cardiovascular Medicine, National Cerebral and Cardiovascular Center, Suita; Department of Molecular..."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yuka","family":"Hayashi","sequence":"additional","affiliation":[{"name":"From the Division of Cardiology (H.W., Y.H., S.N., N.Y., A.S., K.O., M.C., Y.A.), Niigata University School of Medicine, Niigata; Division of Heart Rhythm Management (A.N.), Yokohama Rosai Hospital, Yokohama; Division of Cardiology (K.O., I.W.), Department of Medicine, Nihon University School of Medicine, Tokyo; Division of Arrhythmia and Electrophysiology (H.K., S.K., W.S.), Department of Cardiovascular Medicine, National Cerebral and Cardiovascular Center, Suita; Department of Molecular..."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Taisuke","family":"Ishikawa","sequence":"additional","affiliation":[{"name":"From the Division of Cardiology (H.W., Y.H., S.N., N.Y., A.S., K.O., M.C., Y.A.), Niigata University School of Medicine, Niigata; Division of Heart Rhythm Management (A.N.), Yokohama Rosai Hospital, Yokohama; Division of Cardiology (K.O., I.W.), Department of Medicine, Nihon University School of Medicine, Tokyo; Division of Arrhythmia and Electrophysiology (H.K., S.K., W.S.), Department of Cardiovascular Medicine, National Cerebral and Cardiovascular Center, Suita; Department of Molecular..."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Takeru","family":"Makiyama","sequence":"additional","affiliation":[{"name":"From the Division of Cardiology (H.W., Y.H., S.N., N.Y., A.S., K.O., M.C., Y.A.), Niigata University School of Medicine, Niigata; Division of Heart Rhythm Management (A.N.), Yokohama Rosai Hospital, Yokohama; Division of Cardiology (K.O., I.W.), Department of Medicine, Nihon University School of Medicine, Tokyo; Division of Arrhythmia and Electrophysiology (H.K., S.K., W.S.), Department of Cardiovascular Medicine, National Cerebral and Cardiovascular Center, Suita; Department of Molecular..."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Satomi","family":"Nagao","sequence":"additional","affiliation":[{"name":"From the Division of Cardiology (H.W., Y.H., S.N., N.Y., A.S., K.O., M.C., Y.A.), Niigata University School of Medicine, Niigata; Division of Heart Rhythm Management (A.N.), Yokohama Rosai Hospital, Yokohama; Division of Cardiology (K.O., I.W.), Department of Medicine, Nihon University School of Medicine, Tokyo; Division of Arrhythmia and Electrophysiology (H.K., S.K., W.S.), Department of Cardiovascular Medicine, National Cerebral and Cardiovascular Center, Suita; Department of Molecular..."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nobue","family":"Yagihara","sequence":"additional","affiliation":[{"name":"From the Division of Cardiology (H.W., Y.H., S.N., N.Y., A.S., K.O., M.C., Y.A.), Niigata University School of Medicine, Niigata; Division of Heart Rhythm Management (A.N.), Yokohama Rosai Hospital, Yokohama; Division of Cardiology (K.O., I.W.), Department of Medicine, Nihon University School of Medicine, Tokyo; Division of Arrhythmia and Electrophysiology (H.K., S.K., W.S.), Department of Cardiovascular Medicine, National Cerebral and Cardiovascular Center, Suita; Department of Molecular..."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Naofumi","family":"Takehara","sequence":"additional","affiliation":[{"name":"From the Division of Cardiology (H.W., Y.H., S.N., N.Y., A.S., K.O., M.C., Y.A.), Niigata University School of Medicine, Niigata; Division of Heart Rhythm Management (A.N.), Yokohama Rosai Hospital, Yokohama; Division of Cardiology (K.O., I.W.), Department of Medicine, Nihon University School of Medicine, Tokyo; Division of Arrhythmia and Electrophysiology (H.K., S.K., W.S.), Department of Cardiovascular Medicine, National Cerebral and Cardiovascular Center, Suita; Department of Molecular..."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yuichiro","family":"Kawamura","sequence":"additional","affiliation":[{"name":"From the Division of Cardiology (H.W., Y.H., S.N., N.Y., A.S., K.O., M.C., Y.A.), Niigata University School of Medicine, Niigata; Division of Heart Rhythm Management (A.N.), Yokohama Rosai Hospital, Yokohama; Division of Cardiology (K.O., I.W.), Department of Medicine, Nihon University School of Medicine, Tokyo; Division of Arrhythmia and Electrophysiology (H.K., S.K., W.S.), Department of Cardiovascular Medicine, National Cerebral and Cardiovascular Center, Suita; Department of Molecular..."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Akinori","family":"Sato","sequence":"additional","affiliation":[{"name":"From the Division of Cardiology (H.W., Y.H., S.N., N.Y., A.S., K.O., M.C., Y.A.), Niigata University School of Medicine, Niigata; Division of Heart Rhythm Management (A.N.), Yokohama Rosai Hospital, Yokohama; Division of Cardiology (K.O., I.W.), Department of Medicine, Nihon University School of Medicine, Tokyo; Division of Arrhythmia and Electrophysiology (H.K., S.K., W.S.), Department of Cardiovascular Medicine, National Cerebral and Cardiovascular Center, Suita; Department of Molecular..."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kazuki","family":"Okamura","sequence":"additional","affiliation":[{"name":"From the Division of Cardiology (H.W., Y.H., S.N., N.Y., A.S., K.O., M.C., Y.A.), Niigata University School of Medicine, Niigata; Division of Heart Rhythm Management (A.N.), Yokohama Rosai Hospital, Yokohama; Division of Cardiology (K.O., I.W.), Department of Medicine, Nihon University School of Medicine, Tokyo; Division of Arrhythmia and Electrophysiology (H.K., S.K., W.S.), Department of Cardiovascular Medicine, National Cerebral and Cardiovascular Center, Suita; Department of Molecular..."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yukio","family":"Hosaka","sequence":"additional","affiliation":[{"name":"From the Division of Cardiology (H.W., Y.H., S.N., N.Y., A.S., K.O., M.C., Y.A.), Niigata University School of Medicine, Niigata; Division of Heart Rhythm Management (A.N.), Yokohama Rosai Hospital, Yokohama; Division of Cardiology (K.O., I.W.), Department of Medicine, Nihon University School of Medicine, Tokyo; Division of Arrhythmia and Electrophysiology (H.K., S.K., W.S.), Department of Cardiovascular Medicine, National Cerebral and Cardiovascular Center, Suita; Department of Molecular..."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Masahito","family":"Sato","sequence":"additional","affiliation":[{"name":"From the Division of Cardiology (H.W., Y.H., S.N., N.Y., A.S., K.O., M.C., Y.A.), Niigata University School of Medicine, Niigata; Division of Heart Rhythm Management (A.N.), Yokohama Rosai Hospital, Yokohama; Division of Cardiology (K.O., I.W.), Department of Medicine, Nihon University School of Medicine, Tokyo; Division of Arrhythmia and Electrophysiology (H.K., S.K., W.S.), Department of Cardiovascular Medicine, National Cerebral and Cardiovascular Center, Suita; Department of Molecular..."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Satoki","family":"Fukae","sequence":"additional","affiliation":[{"name":"From the Division of Cardiology (H.W., Y.H., S.N., N.Y., A.S., K.O., M.C., Y.A.), Niigata University School of Medicine, Niigata; Division of Heart Rhythm Management (A.N.), Yokohama Rosai Hospital, Yokohama; Division of Cardiology (K.O., I.W.), Department of Medicine, Nihon University School of Medicine, Tokyo; Division of Arrhythmia and Electrophysiology (H.K., S.K., W.S.), Department of Cardiovascular Medicine, National Cerebral and Cardiovascular Center, Suita; Department of Molecular..."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Masaomi","family":"Chinushi","sequence":"additional","affiliation":[{"name":"From the Division of Cardiology (H.W., Y.H., S.N., N.Y., A.S., K.O., M.C., Y.A.), Niigata University School of Medicine, Niigata; Division of Heart Rhythm Management (A.N.), Yokohama Rosai Hospital, Yokohama; Division of Cardiology (K.O., I.W.), Department of Medicine, Nihon University School of Medicine, Tokyo; Division of Arrhythmia and Electrophysiology (H.K., S.K., W.S.), Department of Cardiovascular Medicine, National Cerebral and Cardiovascular Center, Suita; Department of Molecular..."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hirotaka","family":"Oda","sequence":"additional","affiliation":[{"name":"From the Division of Cardiology (H.W., Y.H., S.N., N.Y., A.S., K.O., M.C., Y.A.), Niigata University School of Medicine, Niigata; Division of Heart Rhythm Management (A.N.), Yokohama Rosai Hospital, Yokohama; Division of Cardiology (K.O., I.W.), Department of Medicine, Nihon University School of Medicine, Tokyo; Division of Arrhythmia and Electrophysiology (H.K., S.K., W.S.), Department of Cardiovascular Medicine, National Cerebral and Cardiovascular Center, Suita; Department of Molecular..."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Masaaki","family":"Okabe","sequence":"additional","affiliation":[{"name":"From the Division of Cardiology (H.W., Y.H., S.N., N.Y., A.S., K.O., M.C., Y.A.), Niigata University School of Medicine, Niigata; Division of Heart Rhythm Management (A.N.), Yokohama Rosai Hospital, Yokohama; Division of Cardiology (K.O., I.W.), Department of Medicine, Nihon University School of Medicine, Tokyo; Division of Arrhythmia and Electrophysiology (H.K., S.K., W.S.), Department of Cardiovascular Medicine, National Cerebral and Cardiovascular Center, Suita; Department of Molecular..."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Akinori","family":"Kimura","sequence":"additional","affiliation":[{"name":"From the Division of Cardiology (H.W., Y.H., S.N., N.Y., A.S., K.O., M.C., Y.A.), Niigata University School of Medicine, Niigata; Division of Heart Rhythm Management (A.N.), Yokohama Rosai Hospital, Yokohama; Division of Cardiology (K.O., I.W.), Department of Medicine, Nihon University School of Medicine, Tokyo; Division of Arrhythmia and Electrophysiology (H.K., S.K., W.S.), Department of Cardiovascular Medicine, National Cerebral and Cardiovascular Center, Suita; Department of Molecular..."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Koji","family":"Maemura","sequence":"additional","affiliation":[{"name":"From the Division of Cardiology (H.W., Y.H., S.N., N.Y., A.S., K.O., M.C., Y.A.), Niigata University School of Medicine, Niigata; Division of Heart Rhythm Management (A.N.), Yokohama Rosai Hospital, Yokohama; Division of Cardiology (K.O., I.W.), Department of Medicine, Nihon University School of Medicine, Tokyo; Division of Arrhythmia and Electrophysiology (H.K., S.K., W.S.), Department of Cardiovascular Medicine, National Cerebral and Cardiovascular Center, Suita; Department of Molecular..."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ichiro","family":"Watanabe","sequence":"additional","affiliation":[{"name":"From the Division of Cardiology (H.W., Y.H., S.N., N.Y., A.S., K.O., M.C., Y.A.), Niigata University School of Medicine, Niigata; Division of Heart Rhythm Management (A.N.), Yokohama Rosai Hospital, Yokohama; Division of Cardiology (K.O., I.W.), Department of Medicine, Nihon University School of Medicine, Tokyo; Division of Arrhythmia and Electrophysiology (H.K., S.K., W.S.), Department of Cardiovascular Medicine, National Cerebral and Cardiovascular Center, Suita; Department of Molecular..."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shiro","family":"Kamakura","sequence":"additional","affiliation":[{"name":"From the Division of Cardiology (H.W., Y.H., S.N., N.Y., A.S., K.O., M.C., Y.A.), Niigata University School of Medicine, Niigata; Division of Heart Rhythm Management (A.N.), Yokohama Rosai Hospital, Yokohama; Division of Cardiology (K.O., I.W.), Department of Medicine, Nihon University School of Medicine, Tokyo; Division of Arrhythmia and Electrophysiology (H.K., S.K., W.S.), Department of Cardiovascular Medicine, National Cerebral and Cardiovascular Center, Suita; Department of Molecular..."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Minoru","family":"Horie","sequence":"additional","affiliation":[{"name":"From the Division of Cardiology (H.W., Y.H., S.N., N.Y., A.S., K.O., M.C., Y.A.), Niigata University School of Medicine, Niigata; Division of Heart Rhythm Management (A.N.), Yokohama Rosai Hospital, Yokohama; Division of Cardiology (K.O., I.W.), Department of Medicine, Nihon University School of Medicine, Tokyo; Division of Arrhythmia and Electrophysiology (H.K., S.K., W.S.), Department of Cardiovascular Medicine, National Cerebral and Cardiovascular Center, Suita; Department of Molecular..."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yoshifusa","family":"Aizawa","sequence":"additional","affiliation":[{"name":"From the Division of Cardiology (H.W., Y.H., S.N., N.Y., A.S., K.O., M.C., Y.A.), Niigata University School of Medicine, Niigata; Division of Heart Rhythm Management (A.N.), Yokohama Rosai Hospital, Yokohama; Division of Cardiology (K.O., I.W.), Department of Medicine, Nihon University School of Medicine, Tokyo; Division of Arrhythmia and Electrophysiology (H.K., S.K., W.S.), Department of Cardiovascular Medicine, National Cerebral and Cardiovascular Center, Suita; Department of Molecular..."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Wataru","family":"Shimizu","sequence":"additional","affiliation":[{"name":"From the Division of Cardiology (H.W., Y.H., S.N., N.Y., A.S., K.O., M.C., Y.A.), Niigata University School of Medicine, Niigata; Division of Heart Rhythm Management (A.N.), Yokohama Rosai Hospital, Yokohama; Division of Cardiology (K.O., I.W.), Department of Medicine, Nihon University School of Medicine, Tokyo; Division of Arrhythmia and Electrophysiology (H.K., S.K., W.S.), Department of Cardiovascular Medicine, National Cerebral and Cardiovascular Center, Suita; Department of Molecular..."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Naomasa","family":"Makita","sequence":"additional","affiliation":[{"name":"From the Division of Cardiology (H.W., Y.H., S.N., N.Y., A.S., K.O., M.C., Y.A.), Niigata University School of Medicine, Niigata; 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