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January 1, 2020The Anatolian Journal of CardiologyOpen Access

QTc >500 ms is linked to ~5-fold higher major cardiac event risk in pediatric LQTS.

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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

YEYakup Ergül

Discussion

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Overview

QTc >500 ms marks higher event risk in pediatric LQTS; leaves open whether risk-stratified interventions improve outcomes.

Key Points

  • The study aims to report on the clinical manifestations, genetic characteristics, and outcomes of children with long QT syndrome (LQTS).
  • Retrospective review of pediatric patients diagnosed with LQTS from January 2011 to April 2020.
  • 145 patients (76 males) included, average age 9.2 years.
  • Statistical analyses of risk factors for major cardiac events were performed.
  • 38 patients (26.2%) diagnosed through family screening, with 15 (10.3%) having experienced cardiac arrest.
  • 82% had pathogenic mutations identified for LQTS genes, and 3.4% died during follow-up.
  • Significant predictors of cardiac events included QTc >500 ms, T wave alternans, high Schwartz score, and Jervell and Lange-Nielsen syndrome.

Study Design

Type

Observational (n=145)

Multicenter

No

Structured PICO

P
Population
145 pediatric patients diagnosed with Long QT syndrome (LQTS), mean age 9.2±4.5 years, 76 males, at a single tertiary pediatric cardiology center in Turkey.
O
Outcome
Clinical and genetic characteristics and outcomes (major cardiac events, death)hard clinical

Main Result

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.

Limitations

  • The genetic tests of some patients have not been completed
  • Retrospective design
  • The number of participants with major cardiac events was not large enough to allow comparisons or relationships based on demographic features or assignment to genetic subgroups

Cite This Study

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.

synapsesocial.com/papers/6a08623aafa0a1b8dbddf507https://doi.org/10.14744/anatoljcardiol.2020.08791
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Also Consider

Synapse has enriched 5 closely related papers on similar clinical questions. Consider them for comparative context:

  1. 1T-wave alternans in long QT syndrome2018 · 11 citations
  2. 2Long QT Syndrome: a Korean Single Center Study2013 · 15 citations
  3. 3Long QT Syndrome: Genetics and Future Perspective2019 · 150 citations
  4. 4Risk of Aborted Cardiac Arrest or Sudden Cardiac Death During Adolescence in the Long-QT Syndrome2006 · 314 citations
  5. 5Common Genotypes of Long QT Syndrome in China and the Role of ECG Prediction2015 · 20 citations