The molecular basis of long QT syndrome and prospects for therapy.
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.
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Lillie Frank Abercrombie Section of Pediatric Cardiology, Baylor College of Medicine, Texas Children's Hospital, Houston 77030, USA.
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Towbin
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D004594:Electrophysiology; D005799:Genes, Dominant; D005820:Genetic Testing; D015316:Genetic Therapy; D006801:Humans; D007473:Ion Channels / Q000235:genetics*; D008133:Long QT Syndrome / Q000235:genetics* / Q000503:physiopathology / Q000628:therapy*; D015221:Potassium Channels / Q000235:genetics; D015222:Sodium Channels / Q000235:genetics
D007473:Ion Channels; D015221:Potassium Channels; D015222:Sodium Channels