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Developing a risk prediction model for sudden cardiac death in children with hypertrophic cardiomyopathy

Zhen Zhen1, Yeqiong Xu1, Xi Chen1

  • 1Department of Cardiology, Beijing Children's Hospital, Capital Medical University, National Centre for Children's Health, Beijing, China.

Frontiers in Pediatrics
|September 2, 2025
PubMed

Insights

A new predictive model helps identify children with hypertrophic cardiomyopathy (HCM) at risk for sudden cardiac death (SCD). This tool, based on key risk factors, shows strong accuracy in predicting SCD events in pediatric patients.

Area of Science:

  • Pediatric Cardiology
  • Cardiovascular Research
  • Medical Informatics

Background:

  • Hypertrophic cardiomyopathy (HCM) is a significant cause of sudden cardiac death (SCD) in children.
  • Accurate risk stratification is crucial for managing pediatric HCM patients and preventing SCD.

Purpose of the Study:

  • To develop and validate a predictive model for SCD in pediatric patients diagnosed with HCM.
  • To identify key clinical and electrocardiographic risk factors associated with SCD in this population.

Main Methods:

  • Retrospective analysis of 184 children with HCM from Beijing Children's Hospital (2006-2022).
  • Cox regression analysis to identify SCD risk factors.
  • Nomogram construction based on multivariate analysis for a predictive model.

Main Results:

  • Key predictors of SCD included age <1 year, female sex, family history of HCM, pathological Q-waves, fragmented QRS, combined arrhythmias, and increased ventricular wall thickness.
  • The nomogram demonstrated strong predictive ability with AUCs ranging from 0.839 to 0.887 across 1-10 years.
  • The model showed good calibration, with predicted probabilities aligning with observed outcomes.

Conclusions:

  • A reliable nomogram-based predictive model for SCD in pediatric HCM patients has been developed.
  • The model exhibits strong discriminatory power and good calibration, supporting its potential clinical utility.
  • External validation is recommended prior to widespread clinical implementation.
Abstract

Keywords:
childrenhypertrophic cardiomyopathypredictive modelprognosissudden cardiac death

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