Congenital long QT syndrome type 3

Yanfei Ruan, Nian Liu, Rong Bai, Silvia G. Priori, Carlo Napolitano

Research output: Contribution to journalArticlepeer-review


Long QT syndrome type 3 (LQT3) is caused by mutations of the SCN5A gene encoding the α-subunit of the human cardiac sodium channel. Specific ST-T wave patterns, triggers, and risk for cardiac events are associated with this LQTS variant. Bench studies have gathered enough knowledge to devise gene-specific therapies to specifically counteract the effects of the mutations. In this article, the authors delineate the LQT3 pathophysiology and epidemiology. They also discuss the clinical management with a focus on the appropriate use of gene-specific therapy.

Original languageEnglish
Pages (from-to)705-713
Number of pages9
JournalCardiac Electrophysiology Clinics
Issue number4
Publication statusPublished - Dec 1 2014


  • Electrophysiology
  • Genetics
  • Ion channel
  • Long QT syndrome
  • Pharmacology
  • Sodium

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine
  • Physiology (medical)


Dive into the research topics of 'Congenital long QT syndrome type 3'. Together they form a unique fingerprint.

Cite this