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Europace Advance Access originally published online on March 29, 2008
Europace 2008 10(7):884-887; doi:10.1093/europace/eun065
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Published on behalf of the European Society of Cardiology. All rights reserved. © The Author 2008. For permissions please email: journals.permissions@oxfordjournals.org

CASE REPORTS

Electrical storm in a patient with arrhythmogenic right ventricular cardiomyopathy and SCN5A mutation

Damir Erkapic*, Thomas Neumann, Jörn Schmitt, Johannes Sperzel, Alexander Berkowitsch, Malte Kuniss, Christian W. Hamm and Heinz-Friedrich Pitschner

Department of Cardiology, Kerckhoff Heart Center, Benekestr. 2–8, Bad Nauheim 61231, Germany

We described a case of a 58-year-old man with organic changes consistent with right ventricular cardiomyopathy. He also had a loss-of-function mutation in the cardiac sodium channel gene SCN5A, described in Brugada syndrome. He first presented with non-sustained ventricular tachycardia and was implanted with an implantable cardioverter defibrillator. He remained asymptomatic for 8 years until he developed recurrent episodes of ventricular tachyarrhythmias, which required multiple shocks. The patient was treated with a combination of quinidine and verapamil and since then remained free of arrhythmias.

Key Words: ARVC, SCN5A, Ion channel mutation, Brugada syndrome, Ventricular arrhythmias, Electrical storming, ICD

* Corresponding author. Tel: +49 6032 9962240; fax: +49 6032 9962367. E-mail address: d.erkapic{at}kerckhoff-klinik.de

Manuscript submitted 29 October 2007. Accepted after revision 28 February 2008.


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