Sudden cardiac death in Andersen-Tawil syndrome

被引:18
作者
Peters, Stefan
Schulze-Bahr, Eric
Etheridge, Susan P.
Tristani-Firouzi, Martin
机构
[1] Univ Hosp Magdeburg, Acad Teaching Hosp, Klinikum Dorothea Christiane Erxleben Quedlinburg, D-06484 Quedlinburg, Germany
[2] Univ Munster, Inst Arteriosclerosis Res, Mol Cardiol Sect, D-4400 Munster, Germany
[3] Univ Utah, Sch Med, Dept Pediat, Div Cardiol, Salt Lake City, UT 84112 USA
[4] Univ Utah, Sch Med, Nora Eccles Harrison Cardiovasc Res & Training In, Salt Lake City, UT 84112 USA
来源
EUROPACE | 2007年 / 9卷 / 03期
关键词
cardiac arrest; bidirectional ventricular tachycardia; polymorphic ventricular tachycardia; genetic disorder; KCNJ2 mutation negative;
D O I
10.1093/europace/eul188
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
Andersen-Tawil syndrome (ATS) is an autosomal dominant or sporadic disorder characterized by periodic paralysis, dysmorphic features, and ventricular arrhythmias. Although ventricular tachycardia burden is quite high sudden cardiac death in ATS is rare. We describe a case with sudden cardiac death due to electrical storm a few days after ICD implantation in KCNJ2 mutation-negative ATS.
引用
收藏
页码:162 / 166
页数:5
相关论文
共 16 条
[1]   Polymorphic ventricular tachycardia and KCNJ2 mutations [J].
Chun, TUH ;
Epstein, MR ;
Dick, M ;
Andelfinger, G ;
Ballester, L ;
Vanoye, CG ;
George, AL ;
Benson, DW .
HEART RHYTHM, 2004, 1 (02) :235-241
[2]   PIP2 binding residues of Kir2.1 are common targets of mutations causing Andersen syndrome [J].
Donaldson, MR ;
Jensen, JL ;
Tristani-Firouzi, M ;
Tawil, R ;
Bendahhou, S ;
Suarez, WA ;
Cobo, AM ;
Poza, JJ ;
Behr, E ;
Wagstaff, J ;
Szepetowski, P ;
Pereira, S ;
Mozaffar, T ;
Escolar, DM ;
Fu, YH ;
Ptácek, LJ .
NEUROLOGY, 2003, 60 (11) :1811-1816
[3]   Drug-induced Torsades de Pointes and implications for drug development [J].
Fenichel, RR ;
Malik, M ;
Antzelevitch, C ;
Sanguinetti, M ;
Roden, DM ;
Priori, SG ;
Ruskin, JN ;
Lipicky, RJ ;
Cantilena, LR .
JOURNAL OF CARDIOVASCULAR ELECTROPHYSIOLOGY, 2004, 15 (04) :475-495
[4]   Andersen mutations of KCNJ2 suppress the native inward rectifier current IK1 in a dominant-negative fashion [J].
Lange, PS ;
Er, F ;
Gassanov, N ;
Hoppe, UC .
CARDIOVASCULAR RESEARCH, 2003, 59 (02) :321-327
[5]   Gene therapy -: Biological pacemaker created by gene transfer [J].
Miake, J ;
Marbán, E ;
Nuss, HB .
NATURE, 2002, 419 (6903) :132-133
[6]   Mutations in Kir2.1 cause the developmental and episodic electrical phenotypes of Andersen's syndrome [J].
Plaster, NM ;
Tawil, R ;
Tristani-Firouzi, M ;
Canún, S ;
Bendahhou, S ;
Tsunoda, A ;
Donaldson, MR ;
Iannaccone, ST ;
Brunt, E ;
Barohn, R ;
Clark, J ;
Deymeer, F ;
George, AL ;
Fish, FA ;
Hahn, A ;
Nitu, A ;
Ozdemir, C ;
Serdaroglu, P ;
Subramony, SH ;
Wolfe, G ;
Fu, YH ;
Ptácek, LJ .
CELL, 2001, 105 (04) :511-519
[7]   The genetic basis of variability in drug responses [J].
Roden, DM ;
George, AL .
NATURE REVIEWS DRUG DISCOVERY, 2002, 1 (01) :37-44
[8]  
SEEMANN G, 2006, AM J PHYSL HEART CIR
[9]   Mechanism of pacemaking in IK1-downregulated myocytes [J].
Silva, J ;
Rudy, Y .
CIRCULATION RESEARCH, 2003, 92 (03) :261-263
[10]  
Stöllberger C, 2005, INT CLIN PSYCHOPHARM, V20, P243