2009
DOI: 10.1161/circulationaha.108.772533
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High Efficacy of β-Blockers in Long-QT Syndrome Type 1

Abstract: Background-␤-Blocker efficacy in long-QT syndrome type 1 is good but variably reported, and the causes of cardiac events despite ␤-blocker therapy have not been ascertained. Methods and Results-This was a retrospective study of the details surrounding cardiac events in 216 genotyped long-QT syndrome type 1 patients treated with ␤-blocker and followed up for a median time of 10 years. Before ␤-blocker, cardiac events occurred in 157 patients (73%) at a median age of 9 years, with cardiac arrest (CA) in 26 (12%)… Show more

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Cited by 278 publications
(99 citation statements)
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“…Increasingly, evidence suggests those who are asymptomatic at diagnosis remain so once started on appropriate β‐blockers3, 20. The increasing recognition of LQTS in the medical community and the implementation of familial screening means that many are diagnosed prior to the onset of symptoms, compared with prior eras when more than half of patients were diagnosed following a symptomatic event 3, 18, 19, 21. Secondly, and closely associated with the above, the overall QTc intervals reported here are shorter than prior studies, whose average QTc range up to 520 ms.…”
Section: Discussionmentioning
confidence: 99%
See 1 more Smart Citation
“…Increasingly, evidence suggests those who are asymptomatic at diagnosis remain so once started on appropriate β‐blockers3, 20. The increasing recognition of LQTS in the medical community and the implementation of familial screening means that many are diagnosed prior to the onset of symptoms, compared with prior eras when more than half of patients were diagnosed following a symptomatic event 3, 18, 19, 21. Secondly, and closely associated with the above, the overall QTc intervals reported here are shorter than prior studies, whose average QTc range up to 520 ms.…”
Section: Discussionmentioning
confidence: 99%
“…It has long been recognized, even from Jervell and Lange‐Nielsen's original description, that adrenergic stimulation (precipitated by exercise or emotion) further delays ventricular repolarization, which is associated with a concomitant increased risk for ventricular arrhythmias in a genotype‐specific manner 2. Anti‐adrenergic therapy, either β‐blockers or cardiac sympathectomy, decreases cardiac adrenergic stimulation, which in turn significantly reduces the risk of life‐threatening cardiac episodes 3, 4, 5…”
Section: Introductionmentioning
confidence: 99%
“…Consequently, sports disqualification for patients with LQTS, in accordance with current guidelines, is commonly adopted by physicians (7). More recent literature has demonstrated a significant risk reduction in patients compliant with betablocker therapy, further fueling the debate (13).…”
Section: Discussionmentioning
confidence: 99%
“…Interestingly, there is a so-called "gender risk reversal" effect which takes place as LQT patients age: males are at higher risk of cardiac events before age 15, but after that age females are at higher risk 12 . Females also seem to have a higher risk of untoward events associated with the use of QT prolonging medications 24 . These gender-related differential effects are thought to be hormonal.…”
Section: Discussionmentioning
confidence: 99%