The QT syndromes: long and short

Hiroshi Morita, Jiashin Wu, Douglas P. Zipes

Research output: Contribution to journalReview article

240 Citations (Scopus)

Abstract

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.

Original languageEnglish
Pages (from-to)750-763
Number of pages14
JournalThe Lancet
Volume372
Issue number9640
DOIs
Publication statusPublished - Aug 29 2008
Externally publishedYes

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ASJC Scopus subject areas

  • Medicine(all)

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