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Must every child with long QT syndrome take a beta blocker?
  1. Kathryn E Waddell-Smith1,2,3,
  2. Nikki Earle2,3,
  3. Jonathan R Skinner1,2,3,4
  1. 1Green Lane Paediatric and Congenital Cardiac Services, Starship Children's Hospital, Auckland, New Zealand
  2. 2Department of Cardiology, University of Auckland, Auckland, New Zealand
  3. 3Department of Child Health, University of Auckland, Auckland, New Zealand
  4. 4Cardiac Inherited Disease Group, Auckland City Hospital, Auckland, New Zealand
  1. Correspondence to Dr Jonathan Skinner, Green Lane Paediatric and Congenital Cardiac Services, Starship Children's’ Hospital, Private Bag 92024, Auckland 1142, New Zealand; jskinner{at}adhb.govt.nz

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.

  • Genetics
  • sudden cardiac death
  • long QT syndrome
  • beta blockers
  • children

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