Short QT syndrome. Update on a recent entity

Arch Cardiovasc Dis. Nov-Dec 2008;101(11-12):779-86. doi: 10.1016/j.acvd.2008.08.009. Epub 2008 Nov 18.

Abstract

The short QT syndrome, a recently discovered ion channel disorder, combines shortened repolarization, a predisposition to atrial and ventricular fibrillatory arrhythmias, and a risk of sudden death. Few cases have been reported, but the prevalence may be underestimated. This syndrome might account for some cases of unexplained ventricular fibrillation in patients with otherwise healthy hearts. Patients have abnormally short QT intervals and refractory periods, and atrial/ventricular fibrillation can be triggered during investigations. Gain-of-function mutations have been detected in three genes encoding potassium channels. Treatment is based on defibrillator implantation, sometimes as a preventive measure. Quinidine may be beneficial in certain cases.

Publication types

  • Review

MeSH terms

  • Action Potentials
  • Anti-Arrhythmia Agents / therapeutic use
  • Arrhythmias, Cardiac / complications
  • Arrhythmias, Cardiac / diagnosis
  • Arrhythmias, Cardiac / genetics
  • Arrhythmias, Cardiac / metabolism*
  • Arrhythmias, Cardiac / therapy
  • Atrial Fibrillation / etiology
  • Atrial Fibrillation / metabolism
  • Death, Sudden, Cardiac
  • Defibrillators, Implantable
  • Electrocardiography
  • Heart Conduction System / metabolism*
  • Humans
  • Mutation
  • Potassium / metabolism*
  • Potassium Channels / genetics
  • Potassium Channels / metabolism*
  • Syndrome
  • Time Factors
  • Treatment Outcome
  • Ventricular Fibrillation / genetics
  • Ventricular Fibrillation / metabolism

Substances

  • Anti-Arrhythmia Agents
  • Potassium Channels
  • Potassium