Research progress of congenital long QT syndrome
10.3760/cma.j.issn.1673-4408.2011.04.014
- VernacularTitle:先天性长QT综合征的研究进展
- Author:
Xia YI
- Publication Type:Journal Article
- Keywords:
Congenital long QT syndrome;
Arhythmia;
Mutable site;
Gene diagnosis
- From:
International Journal of Pediatrics
2011;38(4):353-356
- CountryChina
- Language:Chinese
-
Abstract:
Congenital long QT syndrome ( LQTS) is a cardiac ion channel dysfunction, leading to prolonged myocardial repolarization time. It is characterized by the typical ECG QT interval prolongation and torsades de pointes. It shows clinical recurrence of cardiogenic syncope and even lead to sudden death. Molecular genetic studies have revealed a total of 12 forms of congenital LQTS caused by mutations in genes of the potassium, sodium and calcium channels or membrane adapter located on chromosomes 3, 4, 7, 11, 12, 17, 20 and 21. This review summarized the studies of the pathogenesis of LQTS and gene-related treatments.
