title: Congenital and acquired long QT syndromes.
abstract: Exploration into the underlying genetic causes of congenital long QT syndrome (LQTS) has opened the door to our understanding of repolarization disorders. Expression of LQTS mutations has led to an improved understanding of the mechanisms of arrhythmogenesis, clinical diagnostic tools and channel specific therapy. Further insight into the mechanisms underlying the more common acquired LQTS is emerging from gene and channel studies that have used the congenital syndrome as a springboard for directing research to improve understanding. This review summarizes the clinical, genetic and electrophysiological understanding of congenital and acquired LQTS.
authors.affiliation: Division of Cardiology and the Blackburn Genetics Laboratory, Robarts Research Institute, University of Western Ontario, London.
authors.forename: Bruce D
authors.initials: BD
authors.lastname: Walker
authors.forename: Andrew D
authors.initials: AD
authors.lastname: Krahn
authors.forename: George J
authors.initials: GJ
authors.lastname: Klein
authors.forename: Allan C
authors.initials: AC
authors.lastname: Skanes
authors.forename: Jian
authors.initials: J
authors.lastname: Wang
authors.forename: Robert A
authors.initials: RA
authors.lastname: Hegele
authors.forename: Raymond
authors.initials: R
authors.lastname: Yee
mesh_terms: D000200:Action Potentials / Q000502:physiology; D000889:Anti-Arrhythmia Agents / Q000009:adverse effects / Q000627:therapeutic use; D006328:Cardiac Catheterization; D004594:Electrophysiology; D008040:Genetic Linkage; D006329:Heart Conduction System / Q000002:abnormalities / Q000187:drug effects*; D006801:Humans; D008133:Long QT Syndrome / Q000151:congenital* / Q000235:genetics* / Q000628:therapy; D015222:Sodium Channels / Q000502:physiology; D016171:Torsades de Pointes / Q000209:etiology
chemical_list: D000889:Anti-Arrhythmia Agents; D015222:Sodium Channels