Successful treatment of catecholaminergic polymorphic ventricular tachycardia with flecainide: a case report and review of the current literature

Europace. 2011 Jun;13(6):897-901. doi: 10.1093/europace/euq517. Epub 2011 Feb 2.

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.

Publication types

  • Case Reports
  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Adrenergic beta-Antagonists / therapeutic use
  • Anti-Arrhythmia Agents / therapeutic use*
  • Calcium Channel Blockers / therapeutic use
  • Child
  • Death, Sudden, Cardiac / etiology
  • Death, Sudden, Cardiac / prevention & control
  • Defibrillators, Implantable
  • Drug Therapy, Combination
  • Electrocardiography
  • Flecainide / therapeutic use*
  • Humans
  • Male
  • Mutation / genetics
  • Ryanodine Receptor Calcium Release Channel / genetics
  • Tachycardia, Ventricular / complications
  • Tachycardia, Ventricular / drug therapy*
  • Tachycardia, Ventricular / genetics*
  • Treatment Outcome
  • Verapamil / therapeutic use

Substances

  • Adrenergic beta-Antagonists
  • Anti-Arrhythmia Agents
  • Calcium Channel Blockers
  • Ryanodine Receptor Calcium Release Channel
  • Verapamil
  • Flecainide