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

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 impla...

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Bibliographic Details
Published in:Europace (London, England) England), 2008-07, Vol.10 (7), p.884-887
Main Authors: Erkapic, Damir, Neumann, Thomas, Schmitt, Jörn, Sperzel, Johannes, Berkowitsch, Alexander, Kuniss, Malte, Hamm, Christian W., Pitschner, Heinz-Friedrich
Format: Article
Language:English
Subjects:
Anti-Arrhythmia Agents - therapeutic use
Arrhythmogenic Right Ventricular Dysplasia - drug therapy
Arrhythmogenic Right Ventricular Dysplasia - genetics
Arrhythmogenic Right Ventricular Dysplasia - physiopathology
Electrocardiography
Humans
Male
Middle Aged
Muscle Proteins - genetics
Mutation - genetics
NAV1.5 Voltage-Gated Sodium Channel
Quinidine - therapeutic use
Sodium Channels - genetics
Verapamil - therapeutic use
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Summary: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.
ISSN:1099-5129
1532-2092
DOI:10.1093/europace/eun065