title: Must every child with long QT syndrome take a beta blocker?
abstract: Long QT syndrome is the most commonly recognised cause of sudden cardiac death in children. With a prevalence of 1 in 2000, family screening is identifying large numbers of hitherto asymptomatic gene carriers in the community, about a third of whom have a normal QT interval. The mainstay of treatment is long term uninterrupted beta blocker therapy, a treatment with many potential side effects. This article reviews the evidence and suggests a cohort who may, after assessment in a specialised cardiac-genetic clinic, be spared this treatment because of very low baseline risk. These are asymptomatic boys and prepubertal girls with a heart rate corrected QT interval persistently less than 470 ms who do not indulge in high risk activities (especially swimming) and do not have a missense mutation in the c-loop region of the KCNQ1 (long QT 1) gene.
authors.affiliation: Green Lane Paediatric and Congenital Cardiac Services, Starship Children's Hospital, Auckland, New Zealand Department of Cardiology, University of Auckland, Auckland, New Zealand Department of Child Health, University of Auckland, Auckland, New Zealand.
authors.forename: Kathryn E
authors.initials: KE
authors.lastname: Waddell-Smith
authors.affiliation: Department of Cardiology, University of Auckland, Auckland, New Zealand Department of Child Health, University of Auckland, Auckland, New Zealand.
authors.forename: Nikki
authors.initials: N
authors.lastname: Earle
authors.affiliation: Green Lane Paediatric and Congenital Cardiac Services, Starship Children's Hospital, Auckland, New Zealand Department of Cardiology, University of Auckland, Auckland, New Zealand Department of Child Health, University of Auckland, Auckland, New Zealand Cardiac Inherited Disease Group, Auckland City Hospital, Auckland, New Zealand.
authors.forename: Jonathan R
authors.initials: JR
authors.lastname: Skinner
mesh_terms: D000319:Adrenergic beta-Antagonists / Q000627:therapeutic use*; D002648:Child; D002675:Child, Preschool; D005260:Female; D005838:Genotype; D006801:Humans; D008133:Long QT Syndrome / Q000188:drug therapy* / Q000235:genetics; D008297:Male; D009154:Mutation
chemical_list: D000319:Adrenergic beta-Antagonists
keywords: Genetics; beta blockers; children; long QT syndrome; sudden cardiac death