Clinical management of patients with the long QT syndrome: drugs, devices, and gene-specific therapy

Pacing Clin Electrophysiol. 1997 Aug;20(8 Pt 2):2058-60. doi: 10.1111/j.1540-8159.1997.tb03627.x.


The familial long QT syndrome (LQTS) is now recognized as a genetic channelopathy with a propensity to arrhythmogenic syncope and sudden death. Three genetic mutations have been identified that involve the slow and fast delayed potassium rectifier currents and the sodium current. Distinctive ECG-T wave phenotypes are associated with each of the three genotypes. Current day therapy includes: beta-adrenergic blocking drugs; pacemakers; left cervicothoracic sympathetic ganglionectomy; implanted cardioverter defibrillators; and possibly, drugs that improve mutant ionic channel dysfunction. LQTS has provided unique insight into the complex relationship between ionic channel dysfunction and ventricular tachyarrhythmias.

Publication types

  • Research Support, U.S. Gov't, P.H.S.
  • Review

MeSH terms

  • Adrenergic beta-Antagonists / therapeutic use
  • Death, Sudden, Cardiac / etiology
  • Defibrillators, Implantable
  • Ganglionectomy
  • Genetic Therapy
  • Genotype
  • Humans
  • Ion Channels / genetics
  • Long QT Syndrome / drug therapy
  • Long QT Syndrome / genetics
  • Long QT Syndrome / surgery
  • Long QT Syndrome / therapy*
  • Mutation / genetics
  • Pacemaker, Artificial
  • Phenotype
  • Potassium Channels / genetics
  • Sodium Channels / genetics
  • Syncope, Vasovagal / etiology
  • Tachycardia, Ventricular / etiology


  • Adrenergic beta-Antagonists
  • Ion Channels
  • Potassium Channels
  • Sodium Channels