ArticleFrontiers in cardiovascular medicine2025
Long-term fate of long QT syndrome patients diagnosed in childhood and differential effects of beta-blockers.
Article in Frontiers in cardiovascular medicine, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 2 papers.
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Who cites it
2 citing papers in PubMed.
- From Molecules to Machines: An Integrative Framework Linking Molecular Pathogenesis, Multi-Factorial Risk, Risk Stratification, Clinical Management, and Artificial Intelligence in QT Prolongation and Sudden Cardiac Death.Clinical cardiology · 2026Review
- Structural and Echocardiographic Abnormalities in Congenital Long QT Syndrome: A Review of the Literature.Medicina (Kaunas, Lithuania) · 2026Review
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Authors and funding
7 authors.
Funding
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Abstract
Background and aims: Congenital long QT syndrome (LQTS) is associated with the risk of major arrhythmic events (MAEs), which can be modified by beta-blocker (BB) administration. We aimed to evaluate the effects of selective vs. non-selective BBs on the cumulative burden of MAEs by performing a retrospective analysis of a cohort of LQTS patients diagnosed during childhood. Methods: All pediatric patients ( Results: Mean QTc and median Schwartz score were 501.20 ms and 4.0 points, respectively. Pathogenic or likely pathogenic variants were identified in 124 of 143 tested patients (86.7%). BBs were administered to 156 patients (87.2%). Eleven patients died (6.1%), yielding survival probabilities at 5/10/20 years of 97.7%/94.0%/90.2%. Freedom from MAEs was 93.9%/89.4%/79.3% at 5/10/20 years. MAE was independently predicted by early presentation [hazard ratio (HR): 6.92, Conclusions: The 20-year survival probability of patients with LQTS diagnosed during childhood was 90.2% in the BB treatment era. Non-selective BBs were significantly more effective in decreasing the cumulative MAE burden.
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