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(Circulation. 2008;117:2169.)
© 2008 American Heart Association, Inc.

Clinical Summaries

An extract of the first 250 words of the full text is provided, because this article has no abstract.

	Risk Factors for Aborted Cardiac Arrest and Sudden Cardiac Death in Children With the Congenital Long-QT Syndrome

 
The congenital long-QT syndrome (LQTS) is an important cause of sudden cardiac death in young individuals without structural heart disease. However, data on risk factors for life-threatening cardiac events in children with this genetic disorder are limited. The present study assessed the risk of aborted cardiac arrest or sudden cardiac death in a population of 3015 LQTS children 1 through 12 years of age who were enrolled in the International LQTS Registry. Using time-dependent multivariable analysis, we identified male gender, QTc duration, and a history of prior syncope as risk factors for life-threatening cardiac events in LQTS children. Furthermore, we demonstrate that significant interactions exist among these 3 clinical risk factors that may be used to identify risk subsets in this population. Notably, β-blocker therapy is shown to be associated with a significant reduction in the risk of life-threatening cardiac events during childhood, with a more pronounced benefit (73% risk reduction) in high-risk children who experience recent syncope. However, the rate of life-threatening cardiac events in high-risk children who are treated with this mode of medical therapy is still considerable, with an average annual event rate of 2% while on medical therapy. These findings suggest that careful follow-up is warranted in LQTS children because risk factors for life-threatening cardiac events in this population are time dependent and age specific, resulting in a substantial variability in the phenotypic expression of LQTS during long-term follow-up. See p 2184.

	Long-QT Syndrome After Age 40

 
The congenital long-QT syndrome (LQTS) is associated with increased risk for ventricular tachyarrhythmias . . . [Full Text of this Article]

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Risk Factors for Aborted Cardiac Arrest and Sudden Cardiac Death in Children With the Congenital Long-QT Syndrome

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