Update on long QT syndrome

J Cardiovasc Electrophysiol. 2019 Dec;30(12):3068-3078. doi: 10.1111/jce.14227. Epub 2019 Oct 14.


Long QT syndrome (LQTS) is an inherited disorder characterized by a prolonged QT interval in the 12-lead electrocardiogram and increased risk of malignant arrhythmias in patients with a structurally normal heart. Since its first description in the 1950s, advances in molecular genetics have greatly improved our understanding of the cause and mechanisms of this disease. Sixteen genes linked to LQTS have been described and genetic testing had become an integral part of the diagnosis and risk stratification. This article provides an updated review of the genetic basis, diagnosis, and clinical management of LQTS.

Keywords: QT interval; electrocardiography; long QT; torsades de pointes.

Publication types

  • Review

MeSH terms

  • Action Potentials* / drug effects
  • Action Potentials* / genetics
  • Genetic Predisposition to Disease
  • Heart Conduction System / physiopathology*
  • Heart Rate* / drug effects
  • Heart Rate* / genetics
  • Humans
  • Long QT Syndrome / chemically induced*
  • Long QT Syndrome / genetics*
  • Long QT Syndrome / physiopathology
  • Long QT Syndrome / therapy
  • Phenotype
  • Prognosis
  • Risk Factors