Why the study?
Long QT syndrome is an inherited primary arrhythmia syndrome linked to life-threatening arrhythmias and sudden death, prompting this evaluation of clinical features, genetics, and outcomes in pediatric patients at a Turkish tertiary center.
Population
145 pediatric patients diagnosed with LQTS in Turkey
Design
Single-center retrospective review
Follow-up
Median 35.6±25.8 months
Key result
In children with congenital long QT syndrome, a QTc interval >500 ms was associated with a 5.1-fold increased risk of major cardiac events compared to a shorter QTc interval.
Authors
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QTc >500 ms marks higher event risk in pediatric LQTS; leaves open whether risk-stratified interventions improve outcomes.
Observational (n=145)
No
Effect estimate: OR 5.1 (95% CI 2.3-11)
p-value: p=0.001
In a pediatric cohort with LQTS, QTc >500 ms, T wave alternans, high Schwartz score, and Jervell and Lange-Nielsen syndrome were significant predictors of major cardiac events.
Yakup Ergül (2020) conducted an observational in Congenital long QT syndrome (n=145). Risk factor assessment (QTc >500 ms) vs. QTc ≤500 ms was evaluated on Major cardiac events (syncope, aborted cardiac arrest, appropriate ICD shocks, and sudden cardiac death) (OR 5.1, 95% CI 2.3-11, p=0.001). In children with congenital long QT syndrome, a QTc interval >500 ms was associated with a 5.1-fold increased risk of major cardiac events compared to a shorter QTc interval.
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