Document Detail


Short and long QT syndromes: does QT length really matter?
MedLine Citation:
PMID:  20728018     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
The short and long QT syndromes are inherited diseases associated with an increased risk for life-threatening arrhythmias. The first case of long QT syndrome (LQTS) was reported more than 150 years ago, and the study of this disease led to crucial advancement of our understanding of channelopathies and associated ventricular arrhythmias. Ten years ago, Gussak et al. reported four cases of idiopathic ventricular fibrillation in individuals from a family with a history of sudden cardiac death exhibited very short QT interval and labeled the disease: short QT syndrome (SQTS). Over this decade, the SQTS was found to be a rare inherited syndrome with the potential to provide novel insights into the main mechanisms of cardiac arrhythmogenicity. In this review, we discuss these mechanisms and provocatively question the role of the QT interval duration as a surrogate marker of increased risk for arrhythmia in both the LQTS and the SQTS.
Authors:
Jean-Philippe Couderc; Coeli M Lopes
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Publication Detail:
Type:  Journal Article; Research Support, N.I.H., Extramural; Review    
Journal Detail:
Title:  Journal of electrocardiology     Volume:  43     ISSN:  1532-8430     ISO Abbreviation:  J Electrocardiol     Publication Date:    2010 Sep-Oct
Date Detail:
Created Date:  2010-08-23     Completed Date:  2011-01-11     Revised Date:  2011-09-13    
Medline Journal Info:
Nlm Unique ID:  0153605     Medline TA:  J Electrocardiol     Country:  United States    
Other Details:
Languages:  eng     Pagination:  396-9     Citation Subset:  IM    
Copyright Information:
Copyright 2010 Elsevier Inc. All rights reserved.
Affiliation:
Center for Quantitative Electrocardiography and Cardiac Safety, Department of Medicine, University of Rochester Medical Center, Rochester, NY 14642, USA. jean-philippe.couderc@thew-project.org
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MeSH Terms
Descriptor/Qualifier:
Arrhythmias, Cardiac / complications,  genetics,  physiopathology*
Channelopathies / complications,  genetics,  physiopathology*
Death, Sudden, Cardiac / etiology
Electrocardiography
Humans
Long QT Syndrome / complications,  genetics,  physiopathology
Mutation
Potassium Channels / genetics
Risk Factors
Grant Support
ID/Acronym/Agency:
5U24HL096556-03/HL/NHLBI NIH HHS; U24 HL096556-03/HL/NHLBI NIH HHS
Chemical
Reg. No./Substance:
0/Potassium Channels
Comments/Corrections

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