Mid-Term Follow-up of School-Aged Children With Borderline Long QT Interval

Aya Miyazaki1, Heima Sakaguchi1, Yu Matsumura1

  • 1Department of Pediatric Cardiology, National Cerebral and Cardiovascular Center.

Insights

Children with borderline long QT syndrome (b-LQT) require ongoing monitoring, as their LQTS score and corrected QT interval (QTc) can change. A low LQTS score suggests a lower risk, but continued follow-up is recommended for b-LQT evaluation.

Area of Science:

  • Pediatric Cardiology
  • Clinical Electrophysiology
  • Genetics

Background:

  • Definitive diagnostic criteria and follow-up strategies for borderline long QT syndrome (b-LQT) in children are lacking.
  • b-LQT is defined as a corrected QT interval (QTc) between 400-500 ms.

Purpose of the Study:

  • To retrospectively evaluate the clinical course, genetic testing, and QTc changes in school-aged children with b-LQT.
  • To assess the utility of the LQTS score in managing pediatric b-LQT patients.

Main Methods:

  • Retrospective analysis of 59 school-aged children (5-18 years) with b-LQT.
  • Evaluation of clinical events, genetic testing, QTc measurements, and LQTS scores during a mean follow-up of 6 years.

Main Results:

  • Two patients experienced syncope; no aborted cardiac arrests or sudden cardiac deaths occurred.
  • Positive genetic testing rates varied by LQTS probability (92% high, 57% intermediate, 67% low).
  • Maximum and mean QTc values differed significantly across LQTS probability categories; QTc at rest and recovery changed, altering LQTS probability in 50% of patients.

Conclusions:

  • The LQTS score is a useful tool for evaluating pediatric b-LQT, with low scores indicating low cardiac event risk.
  • LQTS scores and QTc can change during follow-up, necessitating continued monitoring for b-LQT.
  • Clear guidelines for b-LQT follow-up are needed.
Abstract

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