Referenced in: none

Automatically associated channels: Nav1.5

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

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

Journal, date & volume: , 2008 Jul , 10, 884-7

PubMed link: http://www.ncbi.nlm.nih.gov/pubmed/18375968

Abstract
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.