title: Successful treatment of catecholaminergic polymorphic ventricular tachycardia with flecainide: a case report and review of the current literature.
abstract: Catecholaminergic polymorphic ventricular tachycardia (CPVT) is an inherited arrhythmogenic disease that can cause sudden cardiac death due to ventricular fibrillation (VF). While pharmacological therapy with beta-blockers and/or Ca(2)(+) antagonists is often unreliable, a recent study has demonstrated that flecainide can effectively suppress arrhythmia in a murine model of CPVT as well as clinically in two human subjects suffering from CPVT. We here present the case of an 11-year-old boy suffering from CPVT-1 as well as a review of the current relevant literature. After resuscitation due to VF at age 9, an automated implantable cardioverter-defibrillator (ICD) was implanted in 2007. Under beta-blocker therapy, repeated shocks were delivered due to either fast ventricular tachycardia (VT) or VF. This persisted under additional therapy with verapamil. Implantable cardioverter-defibrillator routine interrogations showed frequent non-sustained VT with an average of 8.8 per day. Additionally, the patient suffered from impaired physical performance due to decreased chronotropic competence. In July 2009, flecainide was added to the beta-blocker/verapamil regimen, resulting in a plasma level of 0.20 mg/L. No ICD shock or sustained VT occurred until December 2010. Genetic testing revealed an RyR2 receptor mutation. The case demonstrates the challenge of diagnosis and management of CPVT. It furthermore supports recent experimental evidence that the class 1 antiarrhythmic drug flecainide can suppress CPVT. The presented case supports a novel strategy in treating CPVT with the class I antiarrhythmic agent flecainide.
authors.affiliation: Department of Cardiology and Angiology, University Hospital Münster, Albert-Schweitzer Strasse 33, 48149 Münster, Germany. christian.pott@ukmuenster.de
authors.forename: Christian
authors.initials: C
authors.lastname: Pott
authors.forename: Dirk G
authors.initials: DG
authors.lastname: Dechering
authors.forename: Florian
authors.initials: F
authors.lastname: Reinke
authors.forename: Adam
authors.initials: A
authors.lastname: Muszynski
authors.forename: Stephan
authors.initials: S
authors.lastname: Zellerhoff
authors.forename: Alex
authors.initials: A
authors.lastname: Bittner
authors.forename: Julia
authors.initials: J
authors.lastname: Köbe
authors.forename: Kristina
authors.initials: K
authors.lastname: Wasmer
authors.forename: Eric
authors.initials: E
authors.lastname: Schulze-Bahr
authors.forename: Gerold
authors.initials: G
authors.lastname: Mönnig
authors.forename: Stefan
authors.initials: S
authors.lastname: Kotthoff
authors.forename: Lars
authors.initials: L
authors.lastname: Eckardt
mesh_terms: D000319:Adrenergic beta-Antagonists / Q000627:therapeutic use; D000889:Anti-Arrhythmia Agents / Q000627:therapeutic use*; D002121:Calcium Channel Blockers / Q000627:therapeutic use; D002648:Child; D016757:Death, Sudden, Cardiac / Q000209:etiology / Q000517:prevention & control; D017147:Defibrillators, Implantable; D004359:Drug Therapy, Combination; D004562:Electrocardiography; D005424:Flecainide / Q000627:therapeutic use*; D006801:Humans; D008297:Male; D009154:Mutation / Q000235:genetics; D019837:Ryanodine Receptor Calcium Release Channel / Q000235:genetics; D017180:Tachycardia, Ventricular / Q000150:complications / Q000188:drug therapy* / Q000235:genetics*; D016896:Treatment Outcome; D014700:Verapamil / Q000627:therapeutic use
chemical_list: D000319:Adrenergic beta-Antagonists; D000889:Anti-Arrhythmia Agents; D002121:Calcium Channel Blockers; D019837:Ryanodine Receptor Calcium Release Channel; D014700:Verapamil; D005424:Flecainide