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Prevalence, risk factors, and outcomes of QT prolongation in primary and RASopathy-associated hypertrophic

Anna Wålinder Österberg1, Sandar Min2, Emmi Helle3

  • 1Crown Princess Victoria Children's Hospital, Linköping University Hospital, Linköping University, Linköping, Sweden; Division of Paediatrics, Department of Biomedical and Clinical Sciences, Medical Faculty, Linköping University, Linköping, Sweden (Present Address); Division of Cardiology, Department of Paediatrics, The Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada.

Heart Rhythm
|June 5, 2025
PubMed

Insights

QT prolongation, measured by JTc interval, is linked to sudden cardiac death (SCD) risk in pediatric hypertrophic cardiomyopathy. Incorporating JTc prolongation improves SCD risk prediction in primary HCM patients.

Area of Science:

  • Cardiology
  • Pediatric Electrophysiology
  • Genetic Cardiology

Background:

  • The clinical significance of QT prolongation in pediatric hypertrophic cardiomyopathy (HCM) remains poorly understood.
  • QT prolongation is a potential indicator of increased risk in cardiovascular conditions.

Purpose of the Study:

  • To determine the prevalence, risk factors, and outcomes associated with QT prolongation in pediatric patients diagnosed with hypertrophic cardiomyopathy.
  • To assess the association between JTc interval prolongation and the risk of sudden cardiac death (SCD) events in this population.

Main Methods:

  • A cohort of pediatric patients with primary hypertrophic cardiomyopathy (P-HCM) and RASopathy-associated HCM were analyzed.
  • The corrected JT (JTc) interval was calculated to measure QT prolongation.
  • Statistical models were used to analyze factors associated with JTc duration and the risk of SCD events.

Main Results:

  • A significant prevalence of prolonged JTc was observed in both P-HCM (24%) and RASopathy HCM (44%) cohorts.
  • JTc prolongation was independently associated with an increased risk of SCD events in P-HCM patients (HR 2.9).
  • Including JTc prolongation in risk prediction models significantly improved the accuracy for 5-year SCD risk in P-HCM.

Conclusions:

  • Prolongation of the JTc interval is an independent risk factor for SCD events in pediatric patients with primary hypertrophic cardiomyopathy.
  • Measuring JTc interval aids in refining SCD risk stratification for P-HCM patients.
  • Enhanced monitoring for SCD events is recommended for P-HCM patients exhibiting JTc prolongation.
Abstract

Keywords:
Hypertrophic cardiomyopathyJTc prolongationQTc prolongationRASopathySudden cardiac death

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