Referenced in: none

Automatically associated channels: Kv7.1

Title: Congenital long-QT syndromes: a clinical and genetic update from infancy through adulthood.

Authors: Gregory Webster, Charles I Berul

Journal, date & volume: Trends Cardiovasc. Med., 2008 Aug , 18, 216-24

PubMed link: http://www.ncbi.nlm.nih.gov/pubmed/19185812

Abstract
Long-QT syndromes (LQTSs) have been described in all ages and are a significant cause of cardiovascular mortality, especially in structurally normal hearts. Abnormalities in transmembrane ion conduction channels and structural proteins produce these clinical syndromes, labeled LQT1-LQT12; however, genotype-positive patients still represent only about 70% of LQTSs. Future research will determine the etiology of the remaining cases, further risk-stratify the known genetic defects, improve current treatment options for these syndromes, and uncover novel therapies.