101. Electrical storm in a patient with arrhythmogenic right ventricular cardiomyopathy and SCN5A mutation
- Author
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Malte Kuniss, H. F. Pitschner, Johannes Sperzel, Jörn Schmitt, Christian W. Hamm, Damir Erkapic, Thomas Neumann, and Alexander Berkowitsch
- Subjects
Quinidine ,Male ,medicine.medical_specialty ,medicine.medical_treatment ,Muscle Proteins ,Ventricular tachycardia ,Asymptomatic ,Right ventricular cardiomyopathy ,Sodium Channels ,NAV1.5 Voltage-Gated Sodium Channel ,Electrocardiography ,Physiology (medical) ,Internal medicine ,medicine ,Humans ,cardiovascular diseases ,Arrhythmogenic Right Ventricular Dysplasia ,Brugada syndrome ,medicine.diagnostic_test ,business.industry ,Middle Aged ,Implantable cardioverter-defibrillator ,medicine.disease ,Verapamil ,Mutation ,cardiovascular system ,Cardiology ,medicine.symptom ,Cardiology and Cardiovascular Medicine ,business ,Anti-Arrhythmia Agents ,medicine.drug - 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.
- Published
- 2008