title: Genetics of long-QT syndrome.
abstract: Congenital long QT syndrome (LQTS) is an inherited arrhythmia syndrome characterized by a prolonged QT interval in the 12-lead ECG, torsades de pointes and not negligible prevalence of sudden cardiac death. The genetic testing plays an important role in the diagnosis of LQTS. A total of 15 genes have been reported for autosomal-dominant forms of Romano-Ward-type congenital LQTS and 2 genes for autosomal-recessive forms of the Jervell and Lange-Nielsen syndrome. In this review, we summarize the recent advances in genetics of LQTS and briefly describe forward perspectives of LQTS investigation.
authors.affiliation: Programs for Biomedical Research Graduate School of Biomedical Science, Division of Frontier Medical Science, Department of Cardiovascular Medicine, Hiroshima University, Hiroshima, Japan.
authors.forename: Yukiko
authors.initials: Y
authors.lastname: Nakano
authors.affiliation: Department of Cardiovascular Medicine, Graduate School of Medicine, Nippon Medical School, Tokyo, Japan.
authors.forename: Wataru
authors.initials: W
authors.lastname: Shimizu
mesh_terms: D006801:Humans; D007473:Ion Channels / Q000235:genetics; D008133:Long QT Syndrome / Q000175:diagnosis / Q000235:genetics*; D009154:Mutation
chemical_list: D007473:Ion Channels