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Europace Advance Access originally published online on February 1, 2007
Europace 2007 9(3):162-166; doi:10.1093/europace/eul188
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© The European Society of Cardiology 2007. All rights reserved. For Permissions, please e-mail: journals.permissions@oxfordjournals.org

VENTRICULAR ARRHYTHMIA

Sudden cardiac death in Andersen–Tawil syndrome

Stefan Peters1,*, Eric Schulze-Bahr2, Susan P. Etheridge3 and Martin Tristani-Firouzi3,4

1 Klinikum Dorothea Christiane Erxleben Quedlinburg, Academic Teaching Hospital of the University Hospital Magdeburg, Innere Medizin II-Kardiologie, Ditfurter Weg 24, 06484 Quedlinburg, Germany; 2 Genetics of Arrhythmias, Molecular Cardiology Section, Institute for Arteriosclerosis Research, University of Münster, Münster, Germany; 3 Department of Pediatrics, Division of Cardiology, University of Utah School of Medicine, Salt Lake City, UT, USA; 4 Nora Eccles Harrision Cardiovascular Research and Training Institute, University of Utah School of Medicine, Salt Lake City, UT, USA

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.

Key Words: Cardiac arrest, Bidirectional ventricular tachycardia, Polymorphic ventricular tachycardia, Genetic disorder, KCNJ2 mutation-negative

* Corresponding author. Tel: +49 3946 909 1361. E-mail address: s.peters{at}klinikum-quedlinburg.de

Manuscript submitted 14 September 2006. Accepted after revision 19 November 2006.


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