The QT syndromes: long and short

Hiroshi Morita, Jiashin Wu, Douglas P. Zipes

研究成果査読

274 被引用数 (Scopus)

抄録

This Seminar presents the most recent information about the congenital long and short QT syndromes, emphasising the varied genotype-phenotype association in the ten different long QT syndromes and the five different short QT syndromes. Although uncommon, these syndromes serve as a Rosetta stone for the understanding of inherited ion-channel disorders leading to life-threatening cardiac arrhythmias. Ionic abnormal changes mainly affecting K+, Na+, or Ca2+ currents, which either prolong or shorten ventricular repolarisation, can create a substrate of electrophysiological heterogeneity that predisposes to the development of ventricular tachyarrhythmias and sudden death. The understanding of the genetic basis of the syndromes is hoped to lead to genetic therapy that can restore repolarisation. Presently, symptomatic individuals are generally best treated with an implantable cardioverter defibrillator. Clinicians should be aware of these syndromes and realise that drugs, ischaemia, exercise, and emotions can precipitate sudden death in susceptible individuals.

本文言語English
ページ(範囲)750-763
ページ数14
ジャーナルThe Lancet
372
9640
DOI
出版ステータスPublished - 2008
外部発表はい

ASJC Scopus subject areas

  • 医学一般

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