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为肥厚型心肌病患儿心脏性猝死开发风险预测模型。

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

作者信息

Zhen Zhen, Xu Yeqiong, Chen Xi, Na Jia, Xiao Yanyan, Yuan Yue

机构信息

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

出版信息

Front Pediatr. 2025 Aug 14;13:1628585. doi: 10.3389/fped.2025.1628585. eCollection 2025.

Abstract

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.

摘要

目的

本研究旨在建立肥厚型心肌病(HCM)患儿心源性猝死(SCD)的预测模型。

方法

这项回顾性研究纳入了2006年1月至2022年8月期间就诊于首都医科大学附属北京儿童医院且被诊断为HCM的患儿。采用Cox回归分析确定SCD的危险因素。基于多变量分析确定的危险因素构建列线图。

结果

本研究共纳入184例患儿(115例男孩和69例女孩)。初次诊断时的中位(IQR)年龄为4.54(0.50 - 10.25)岁。其中,141例患儿被诊断为原发性HCM,43例为继发性HCM。多变量分析显示,年龄<1岁[风险比(HR),95%置信区间(CI):6.232(2.858 - 13.591)]、女性[HR:2.547(1.460 - 4.444)]、HCM家族史[HR:2.622(1.468 - 4.683)]、病理性Q波[HR:2.290(1.285 - 4.082)]、碎裂QRS波[HR:3.526(1.786 - 6.963)]、合并心律失常(HR:2.218 [1.136 - 4.333])、室间隔厚度增加[HR:1.055(1.008 - 1.105)]以及左心室后壁厚度增加[HR:1.060(1.026 - 1.096)]与SCD显著相关。基于列线图的SCD预测模型显示出较强的区分能力,1年时曲线下面积(AUC)为0.887(95% CI:0.829 - 0.945),2年时为0.839(95% CI:0.777 - 0.902),3年时为0.847(95% CI:0.782 - 0.912),4年时为0.855(95% CI:0.791 - 0.919),5年时为0.850(95% CI:0.789 - 0.911),10年时为0.845(95% CI:0.763 - 0.926)。预测概率与观察概率密切相符,表明模型具有良好的校准度。

结论

建立了HCM患儿SCD的预测模型,显示出较强的内部一致性和可靠性。建议在临床应用前进行外部验证。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/75e2/12391110/6abd8bab3db4/fped-13-1628585-g001.jpg

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