Trans-2,3-enoyl-CoA reductase-like-related catecholaminergic polymorphic ventricular tachycardia with regular ventricular tachycardia and response to flecainide

J Cardiovasc Electrophysiol. 2023 Sep;34(9):1996-2001. doi: 10.1111/jce.16011. Epub 2023 Jul 20.

Abstract

Introduction: We describe a unique case of TECRL-CPVT presented with cardiac arrest.

Methods: Post resuscitation, the patient developed regular ventricular tachycardia featuring a left purkinje system morphology.

Results: There was clear suppression of arrhythmia with the addition of flecainide and isolated ventricular ectopy causing secondary T-wave changes.

Conclusion: A high index of suspicion was required to eventually make the diagnosis through whole exome sequencing.

Keywords: CPVT; TECRL; cardiac arrest.

Publication types

  • Case Reports

MeSH terms

  • Anti-Arrhythmia Agents / therapeutic use
  • Flecainide / therapeutic use
  • Humans
  • Oxidoreductases
  • Ryanodine Receptor Calcium Release Channel
  • Tachycardia, Ventricular* / diagnosis
  • Tachycardia, Ventricular* / drug therapy
  • Tachycardia, Ventricular* / genetics
  • Ventricular Premature Complexes* / complications

Substances

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

Supplementary concepts

  • Polymorphic catecholergic ventricular tachycardia