Benign premature ventricular complexes from the right ventricular outflow tract triggered polymorphic ventricular tachycardia in a latent type 2 LQTS patient

Intern Med. 2012;51(23):3261-5. doi: 10.2169/internalmedicine.51.8565. Epub 2012 Dec 1.

Abstract

A 57-year-old woman showed frequent premature ventricular complexes (PVCs) originating from the right ventricular outflow tract (RVOT), and some of the PVCs triggered polymorphic ventricular tachycardia (PVT). Structural heart diseases were ruled out by conventional cardiac examinations. Radiofrequency catheter ablation was successful in eliminating the PVCs and subsequent PVT. However, epinephrine infusion unmasked her prolonged QT interval, and a genetic analysis revealed a KCNH2 mutation (R694H) as the cause of latent type-2 long QT syndrome (LQTS). This case suggests that latent LQTS may work as an arrhythmogenic substrate of PVT triggered by a benign form of RVOT-PVCs in patients with a structurally normal heart.

Publication types

  • Case Reports

MeSH terms

  • Amino Acid Substitution
  • Catheter Ablation
  • ERG1 Potassium Channel
  • Electrocardiography
  • Epinephrine
  • Ether-A-Go-Go Potassium Channels / genetics
  • Female
  • Humans
  • Long QT Syndrome / diagnosis
  • Long QT Syndrome / genetics
  • Long QT Syndrome / physiopathology*
  • Middle Aged
  • Mutation, Missense
  • Tachycardia, Ventricular / genetics
  • Tachycardia, Ventricular / physiopathology*
  • Tachycardia, Ventricular / therapy
  • Ventricular Premature Complexes / genetics
  • Ventricular Premature Complexes / physiopathology*
  • Ventricular Premature Complexes / therapy

Substances

  • ERG1 Potassium Channel
  • Ether-A-Go-Go Potassium Channels
  • KCNH2 protein, human
  • Epinephrine

Supplementary concepts

  • Long Qt Syndrome 2
  • Polymorphic catecholergic ventricular tachycardia