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Mol Med Today. 1998 Sep;4(9):382-8.

The molecular basis of long QT syndrome and prospects for therapy.

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1
Lillie Frank Abercrombie Section of Pediatric Cardiology, Baylor College of Medicine, Texas Children's Hospital, Houston 77030, USA.

Abstract

Long QT syndrome (LQT) is a cardiac disorder that causes sudden death from ventricular tachyarrhythmias, specifically torsade de pointes. Two types of LQT have been reported, autosomal-dominant LQT (Romano-Ward syndrome) and autosomal-recessive LQT (Jervell and Lange-Nielsen syndrome); Jervell and Lange-Nielsen syndrome is also associated with deafness. Four LQT genes have been identified for autosomal-dominant LQT: K+ channel genes KVLQT1 on chromosome 11p15.5, HERG on 7q35-36 and minK on 21q22, and the cardiac Na+ channel gene SCN5A on chromosome 3p21-24. Two genes, KVLQT1 and minK, have been identified for Jervell and Lange-Nielsen syndrome. Genetic testing and gene-specific therapies are available for some LQT patients.

PMID:
9791861
[Indexed for MEDLINE]
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