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Arch Dis Child. 2015 Mar;100(3):279-82. doi: 10.1136/archdischild-2014-306864. Epub 2014 Sep 30.

Must every child with long QT syndrome take a beta blocker?

Author information

1
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.
2
Department of Cardiology, University of Auckland, Auckland, New Zealand Department of Child Health, University of Auckland, Auckland, New Zealand.
3
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.

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.

KEYWORDS:

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

[Indexed for MEDLINE]

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