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Sudden cardiac death in Andersen-Tawil syndrome
Stefan Peters1, Eric Schulze-Bahr, Susan P Etheridge
1Klinikum Dorothea Christiane Erxleben Quedlinburg, Academic Teaching Hospital of the University Hospital Magdeburg, Innere Medizin II-Kardiologie, Ditfurter Weg 24, 06484 Quedlinburg, Germany. s.peters@klinikum-quedlinburg.de
Abstract:
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.
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