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ORIGINAL RESEARCH article

Front. Pediatr.

Sec. Pediatric Cardiology

Volume 13 - 2025 | doi: 10.3389/fped.2025.1628585

Developing a Risk Prediction Model for Sudden Cardiac Death in Children with Hypertrophic Cardiomyopathy

Provisionally accepted
Zhen  ZhenZhen ZhenYeqiong  XuYeqiong XuXi  ChenXi ChenJia  NaJia NaXiao  Yan YanXiao Yan YanYue  YuanYue Yuan*
  • Department of Cardiology, Beijing Children’s Hospital, Capital Medical University, National Centre for Children’s Health, Beijing, China

The final, formatted version of the article will be published soon.

Objective: This study aimed to develop a predictive model for sudden cardiac death (SCD) in children with hypertrophic cardiomyopathy (HCM). Methods: The retrospective study included children diagnosed with HCM who visited Beijing Children’s Hospital, Capital Medical University between January 2006 and August 2022. Cox regression analysis was used to identify risk factors for SCD. A nomogram was constructed based on risk factors identified through multivariate analysis. Results: A total of 184 children (115 boys and 69 girls) were included in the study. The median (IQR) age at the initial diagnosis was 4.54 (0.50–10.25) years. Of these, 141 children were diagnosed with primary HCM, while 43 had secondary HCM. The multivariate analysis showed that age <1 year (hazard ratio [HR], 95% confidence interval [CI]: 6.232 [2.858–13.591]), female sex (HR 2.547 [1.460–4.444]), a family history of HCM (HR 2.622 [1.468–4.683]), pathological Q-waves (HR 2.290 [1.285–4.082]), fragmented QRS waves (HR 3.526 [1.786–6.963]), combined arrhythmias (HR 2.218 [1.136–4.333]), increased interventricular septal thickness (HR 1.055 [1.008–1.105]), and increased left ventricular posterior wall thickness (HR 1.060 [1.026–1.096]) were significantly associated with SCD. The nomogram-based SCD prediction model demonstrated strong discriminatory ability, with areas under the curve (AUC) of 0.887 (95% CI: 0.829–0.945) at 1 year, 0.839 (95% CI: 0.777–0.902) at 2 years, 0.847 (95% CI: 0.782–0.912) at 3 years, 0.855 (95% CI: 0.791–0.919) at 4 years, 0.850 (95% CI: 0.789–0.911) at 5 years, and 0.845 (95% CI: 0.763–0.926) at 10 years. Predicted probabilities closely aligned with observed probabilities, indicating good calibration of the model. Conclusion: A predictive model for SCD in children with HCM was developed, demonstrating strong internal consistency and reliability. External validation is recommended before clinical implementation.

Keywords: Children, Hypertrophic Cardiomyopathy, prognosis, Sudden cardiac death, predictive model

Received: 14 May 2025; Accepted: 31 Jul 2025.

Copyright: © 2025 Zhen, Xu, Chen, Na, Yan and Yuan. This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) or licensor are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.

* Correspondence: Yue Yuan, Department of Cardiology, Beijing Children’s Hospital, Capital Medical University, National Centre for Children’s Health, Beijing, China

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