The molecular basis of long QT syndrome and prospects for therapy

Mol Med Today. 1998 Sep;4(9):382-8. doi: 10.1016/s1357-4310(98)01320-3.

Abstract

Long QT syndrome (LQT) is a cardiac disorder that causes sudden death from ventricular tachyarrhythmias, specifically torsade de pointes. Two types of LQT have been reported, autosomal-dominant LQT (Romano-Ward syndrome) and autosomal-recessive LQT (Jervell and Lange-Nielsen syndrome); Jervell and Lange-Nielsen syndrome is also associated with deafness. Four LQT genes have been identified for autosomal-dominant LQT: K+ channel genes KVLQT1 on chromosome 11p15.5, HERG on 7q35-36 and minK on 21q22, and the cardiac Na+ channel gene SCN5A on chromosome 3p21-24. Two genes, KVLQT1 and minK, have been identified for Jervell and Lange-Nielsen syndrome. Genetic testing and gene-specific therapies are available for some LQT patients.

Publication types

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

MeSH terms

  • Electrophysiology
  • Genes, Dominant
  • Genetic Testing
  • Genetic Therapy*
  • Humans
  • Ion Channels / genetics*
  • Long QT Syndrome / genetics*
  • Long QT Syndrome / physiopathology
  • Long QT Syndrome / therapy*
  • Potassium Channels / genetics
  • Sodium Channels / genetics

Substances

  • Ion Channels
  • Potassium Channels
  • Sodium Channels