Key result
Beta blocker therapy may be spared in asymptomatic boys and prepubertal girls with long QT syndrome and QTc <470 ms who avoid high-risk activities and lack KCNQ1 c-loop missense mutations.
Why the study?
Can a specific low-risk cohort of children with long QT syndrome be safely spared from long-term beta blocker therapy?
Can a specific low-risk cohort of children with long QT syndrome be safely spared from long-term beta blocker therapy?
A highly specific, low-risk cohort of children with long QT syndrome may safely avoid the potential side effects of long-term beta blocker therapy.
Does not yet justify sparing beta blockers in pediatric LQTS; leaves open targeted omission in low-risk subgroups pending prospective validation.
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.
No takes yet. Share an insight, caveat, or question.
Waddell-Smith et al. (2014) conducted a review in Long QT syndrome. Beta blocker therapy was evaluated. Beta blocker therapy may be spared in asymptomatic boys and prepubertal girls with long QT syndrome and QTc <470 ms who avoid high-risk activities and lack KCNQ1 c-loop missense mutations.
Synapse has enriched 5 closely related papers on similar clinical questions. Consider them for comparative context: