title: Electrical storm in a patient with arrhythmogenic right ventricular cardiomyopathy and SCN5A mutation.
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.
authors: Department of Cardiology, Kerckhoff Heart Center, Benekestr. 2-8, Bad Nauheim 61231, Germany. d.erkapic@kerckhoff-klinik.de
authors: Damir
authors: D
authors: Erkapic
authors: Thomas
authors: T
authors: Neumann
authors: Jörn
authors: J
authors: Schmitt
authors: Johannes
authors: J
authors: Sperzel
authors: Alexander
authors: A
authors: Berkowitsch
authors: Malte
authors: M
authors: Kuniss
authors: Christian W
authors: CW
authors: Hamm
authors: Heinz-Friedrich
authors: HF
authors: Pitschner
mesh_terms: D000889:Anti-Arrhythmia Agents / Q000627:therapeutic use; D019571:Arrhythmogenic Right Ventricular Dysplasia / Q000188:drug therapy / Q000235:genetics* / Q000503:physiopathology*; D004562:Electrocardiography; D006801:Humans; D008297:Male; D008875:Middle Aged; D009124:Muscle Proteins / Q000235:genetics*; D009154:Mutation / Q000235:genetics*; D062554:NAV1.5 Voltage-Gated Sodium Channel; D011802:Quinidine / Q000627:therapeutic use; D015222:Sodium Channels / Q000235:genetics*; D014700:Verapamil / Q000627:therapeutic use
chemical_list: D000889:Anti-Arrhythmia Agents; D009124:Muscle Proteins; D062554:NAV1.5 Voltage-Gated Sodium Channel; C568320:SCN5A protein, human; D015222:Sodium Channels; D014700:Verapamil; D011802:Quinidine