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1 Catecholaminergic polymorphic ventricular tachycardia Department of Pediatrics, Nihon University1), Department of Cardiology, Aichi Children's Health and Medical Center2), The Second Department of Pediatrics, Japanese Red Cross Wakayama Medical Center3), Department of Pediatrics, Kurashiki Central Hospital4), Department of Cardiology, Tokyo Metropolitan Hiroo Hospital5), Departnemt of Cardiology, Fukuoka Children's Hospital and Medical Center for Infectious Diseases6), Department of Cardiology, Shizuoka Children's Hospital7), Department of Pediatrics, Hiroshima University8), Department of Pediatrics, Kyushu Kosei Nenkin Hospital9), Department of Pediatrics, University of Occupational and Environmental Health10), Department of Pediatrics, Tokushima City Hospital11), Department of Pediatrics, Kyushu University12), Department of Pediatrics, Seirei Mikatahara Hospital13), Department of Cardiology, Yokohama Minami Kyosai Hospital14), Department of Cardiology, Tokyo Metropolitan Kiyose Children's Hospital15), Department of Pediatrics, Yokohama City University16) Naokata Sumitomo,Kensuke Harada,Masami Nagashima,Yoshihide Nakamura,Yoshio Aragaki, Harumizu Sakurada, Seiji Fukamizu, Hiroya Ushinohama, Kenichi Kurosaki, Toshiaki Yasuda, Shiro Konishi, Kunitaka Jouo, Masumi Koujiro, Suguru Matsuoka, Takuro Ohno, Satoru Hayakawa, Mitsuhiro Nishizaki, Masaru Miura, Toshimitsu Shibata, Ichiro Niimura

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6 1) Leenhardt A, Lucet V, Den joy I, et al : Catecholaminergic polymorphic ventricular tachycardia in children. Circulation 1995 ; 91 : ) Berg KJ : IVIultifocal ventricular extrasystoles with Adams-Stokes syndrome in sibling. Am Heart J 1960 ; episodic unconsciousness. Arch Dis Child 1962 ; 37 : : ) Horan IVI, Venables AW : Paroxysmal tachycardia with 4) Wennewold A, Melchior JC, Sandoe E : Adams-Stokes syndrome in children without organic heat disease : electrocardiogram after exercise as a diagnostic tool. Acta Med Scand 1965 ; 177 : ) Dik T, Janukowicz C : Bidirectional tachycardia in a case of recurrent paroxysmal tachycardia with ventricular fibrillation. Cardiologia 1968 ; 52 : ) Reid DS, Tynan M, Braidwood L, et al : Bidirectional tachycardia in a child : a study using His bundle electrography. Br Heart J : ) Shaw TR : Recurrent ventricular fibrillation associated with normal QT interval. QJ Med 1981 ; 200 : ) Wennevolcl A, Sandoe E : Paroxysmal ventricular fibrillation in children: long term follow-up of three cases treated with beta-blocking agents. Acta Med Scand 1977 ; 202 :

7 9) Benson DW Jr, Gallagher JJ, Sterba R et al : Catecholamine induced double tachycardia: case report in a child PACE 1980 ; 3 : ) von Bernuth G, Bernsau U, Gutheil H, et al : Tachyarrhymic syncope in children with structurally normal hearts with and without QT-prolongation in the electrocardiogram. Eur J Pediatr 1982 ; 138 : ) Coumel P, Fidelle J, Lucet V, et al : Catecholaminergicinduced severe ventricular arrhythmias with Adams- Stokes syndrome in children: report of four cases. Br Heart J 1978 ; 40 (supple) : ) Cohen TJ, Liem LB, Hancock EW : Association of bidirectional ventricular tachycardia with familial sudden death syndrome. Am J Cardiol 1989 ; 64 : ) Eisenberg 5, Scheinman M, Dullet N, et al : Sudden cardiac death and polymorphous ventricular tachycardia in patients with normal QT intervals and normal systolic cardiac function. Am J Cardiol 1995 ; 75 : ) Myrianthefs M, Cariolou M, Eldar M, et al : Exerciseinduced ventricular arrhythmias and sudden cardiac death in a family. Chest 1997 ; 111 : ) Priori SG, Napolitano C, Memmi M, et al : Clinical and molecular characterization of patients with catecholaminergic polymorphic ventricular tachycardia. Circulation 2002 ; 106 : ) Sumitomo N, Harada K, Nagashima M, et al : Catecholaminergic polymorphic ventricular tachycardia : electrocardiographic characteristics and optimal therapeutic strategies to prevent sudden death. Heart 2003 ; 89 : ) Brugada P, Brugada J : Right bundle branch block, persistent ST segment elevation and sudden cardiac death : A distinct clinical and electrocardiographic syndrome, A multicenter report. J Am Coll Cardiol 1992 ; 20 : ) Priori SG, Napolitano C, Tiso N, et al : Mutations in the cardiac ryanodine receptor gene (hryr2) underlie catecholaminergic polymorphic ventricular tachycardia. Circulation 2001 ; 103 : ) Laitinen PJ, Brown KM, Piippo K, et al : Mutations of the cardiac Ryanodine receptor (RyR2) gene in familial polymorphic ventricular tachycardia. Circulation 2001 ; 103 : ) Nakajima T, Kaneko Y, Taniguchi Y, et al : The mechanism of catecholaminergic polymorphic ventricular tachycardia may be triggered activity due to delayed afterdepolarization. Eur Heart J 1997 ; 18 : ) Zygmunt AC, Goodrow RJ, Weigel CM : INaCa and ICI (Ca) contribute to isoproterenol-induced delayed after depolarizations in midmyocardial cells. Am J Physiol 1998 ; 275 : H ) Katra RP, Laurita KR. Cellular mechanism of calciummediated triggered activity in the heart. Circ Res 2005 ; 96 : ) Nam GB, Burashnikov A, Antzelevitch C : Cellular mechanisms underlying the development of catecholaminergic ventricular tachycardia. Circulation 2005 ; 111 : ) Sedarat F, Xu L, Moore et al : Localization of dihydropyridine and ryanodine receptors in neonate rabbit heart using confocal microscopy. Am J Physiol Heart Circ Physiol 2000 ; 279 : ) Lahat H, Eldar M, Levy-Nissenbaum E, et al : Autosomal recessive catecholamine- or exercise-induced polymorphic ventricular tachycardia : clinical features and assignment of the disease gene to chromosome 1p Circulation 2001 ; 103 : ) Laitinen PJ, Swan I-I, Kontula K : Molecular genetics of exercise-induced polymorphic ventricular tachycardia : identification of three novel cardiac ryanodine receptor mutations and two common calsequestrin 2 amino-acid polymorplaisms. Eur J Hum Genet 2003 ; 11 : ) Eldar M, Pras E, Lahat 11 : A missense mutation in the CASQ2 gene is associated with autosomal-recessive catecholamine-induced polymorphic ventricular tachycardia. Trends Cardiovasc Med 2003 ; 13 : ) Lahat H, Pras E, ()lender T, et al : A missense mutation in a highly conserved region of CASQ2 is associated with autosomal recessive catecholamine-induced polymorphic ventricular tachycardia in Bedouin families from Israel. Am J Hum Genet 2001 ; 69 :

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