Flecainide Reduces Ventricular Arrhythmias in Patients With Genotype RyR2-positive Catecholaminergic Polymorphic Ventricular Tachycardia

Rev Esp Cardiol (Engl Ed). 2018 Mar;71(3):185-191. doi: 10.1016/j.rec.2017.04.032. Epub 2017 Aug 5.
[Article in English, Spanish]

Abstract

Introduction and objectives: Catecholaminergic polymorphic ventricular tachycardia (CPVT) is an inherited disease characterized by polymorphic or bidirectional ventricular arrhythmias (VA) triggered by physical or emotional stress in young people with a structurally normal heart. Beta-blockers are the cornerstone of treatment, while flecainide has recently been incorporated into the therapeutic arsenal. The aim of this study was to report our experience with this drug.

Methods: The cohort included 174 genotype-positive CPVT-patients from 7 families. We collected data from patients who were receiving flecainide and analyzed the indications, adverse effects and dosage, clinical events, VA and arrhythmic window during exercise testing, and implantable cardioverter-defibrillator (ICD) shocks during follow-up.

Results: Eighteen patients (10.4%) received flecainide; 17 patients in combination with beta-blockers, and 1 patient as monotherapy due to beta-blocker intolerance. None of the patients presented side effects. In 13 patients (72.2%) the indication was the persistence of exercise-induced VA and in 5 patients (27.7%) persistent ICD-shocks, despite on beta-blockers. After flecainide initiation, the exercise-induced VA quantitative score was reduced by more than 50% in 66.7% of the members of family 1 (32.76 ± 84.06 vs 74.38 ± 153.86; P = .018). The arrhythmic window was reduced (5.8 ± 11.9 bpm vs 19.69 ± 21.27 bpm; P = .007), and 4 of 5 patients with appropriate ICD shocks experienced no further shocks in the follow-up.

Conclusions: In CPVT-patients flecainide reduces clinical events, exercise-induced VA, the arrhythmic window, and ICD shocks, with good tolerance.

Keywords: Arritmia ventricular; Catecholaminergic polymorphic ventricular tachycardia; Flecainida; Flecainide; Taquicardia ventricular polimórfica catecolaminérgica; Ventricular arrhythmia.

Publication types

  • Multicenter Study

MeSH terms

  • Adult
  • Anti-Arrhythmia Agents / administration & dosage
  • DNA Mutational Analysis
  • Death, Sudden, Cardiac / epidemiology
  • Death, Sudden, Cardiac / prevention & control
  • Electrocardiography
  • Female
  • Flecainide / therapeutic use*
  • Follow-Up Studies
  • Genotype
  • Humans
  • Male
  • Mutation*
  • Polymorphic Catecholaminergic Ventricular Tachycardia
  • Ryanodine Receptor Calcium Release Channel / genetics*
  • Ryanodine Receptor Calcium Release Channel / metabolism
  • Tachycardia, Ventricular / genetics*
  • Tachycardia, Ventricular / metabolism
  • Tachycardia, Ventricular / mortality

Substances

  • Anti-Arrhythmia Agents
  • Ryanodine Receptor Calcium Release Channel
  • Flecainide