2024 年第 9 期 第 0 卷

扩张型心肌病患儿远期预后的危险因素研究

Risk factors for long-term prognosis in children with dilated cardiomyopathy

作者：温海初李扬杜杰

英文作者：Wen Haichu Li Yang Du Jie

单位：首都医科大学附属北京安贞医院北京市心肺血管疾病研究所教育部重塑相关心血管疾病重点实验室心血管重大疾病防治协同创新中心，北京100029

英文单位：Beijing Anzhen Hospital Capital Medical University Beijing Institute of Heart Lung and Blood Vessel Diseases Key Laboratory of Remodeling-related Cardiovascular Diseases Ministry of Education Collaborative Innovation Center for Cardiovascular Disorders Beijing 100029 China

关键词：儿童扩张型心肌病；不良心血管事件；预后评分

英文关键词：Pediatricdilatedcardiomyopathy;Adversecardiovascularevents;Prognosticscore

• 摘要：

• 目的 基于临床危险因素构建儿童扩张型心肌病（PDCM）患者远期预后评分，并验证其危险分层能力。方法 选取2015年9月至2019年12月入住首都医科大学附属北京安贞医院的扩张型心肌病（DCM）患儿临床资料作回顾性分析，将2015年9月至2017年3月纳入的DCM患儿作为发现集（87例），2017年4月至2019年12月纳入的DCM患儿作为验证集（51例）。根据是否发生不良事件，将DCM患儿分为事件组和无事件组。利用单因素Cox回归筛选与不良事件相关的临床危险因素，进一步利用多因素Cox回归系数为临床危险因素赋以权重，构建预后评分。Kaplan-Meier曲线用于评估评分的风险分层能力。结果 发现集中，事件组左心室射血分数（LVEF）和左心室短轴缩短率（LVFS）均低于无事件组；血尿素氮、血尿酸和B型脑钠肽（BNP）水平均高于无事件组（均P<0.05）。验证集中，事件组年龄、血肌酐和BNP大于/高于无事件组（均P<0.05）。单因素Cox回归分析结果表明，年龄、LVEF、LVFS、左心室舒张末期内径z评分、BNP、血尿素氮、血尿酸和C反应蛋白与不良事件显著相关（均P<0.05）。多因素Cox回归分析最终纳入LVEF、BNP和C反应蛋白构建预后评分。在验证集中，利用预后评分进行分层后，低危、高危DCM患儿的无事件生存率分别为81.2%和51.1%（Log-rank P=0.049）。结论 基于临床危险因素构建了PDCM远期不良事件的预后评分，能够较好的进行危险分层。

• Objective To construct a long-term prognosis score in patients with pediatric dilated cardiomyopathy (PDCM) based on clinical risk factors and verify its risk stratification ability. Methods The clinical data of children with dilated cardiomyopathy (DCM) admitted to Beijing Anzhen Hospital, Capital Medical University from September 2015 to December 2019 were retrospectively analyzed. Children with DCM from September 2015 to March 2017 were enrolled as the discovery set (87 cases), and children with DCM from April 2017 to December 2019 were enrolled as the validation set (51 cases). According to the presence or absence of adverse events, the children with DCM were divided into event group and non-event group. Univariate Cox regression was used to screen clinical risk factors related to adverse events, and multivariate Cox regression coefficients was further used to assign weights to clinical risk factors to construct a prognostic score. Kaplan-Meier curves was used to assess the risk stratification ability of the scores. Results In the discovery set, the left ventricular ejection fraction (LVEF) and left ventricular fractional shortening (LVFS) in the event group were lower than those in the non-event group. The levels of blood urea nitrogen, serum uric acid and B-type natriuretic peptide (BNP) in the event group were higher than those in the non-event group (all P<0.05). In the validation set, the age, serum creatinine and BNP in the event group were higher than those in the non-event group (all P<0.05). Univariate Cox regression analysis showed that age, LVEF, LVFS, left ventricular end-diastolic diameter z score, BNP, blood urea nitrogen, serum uric acid and C-reactive protein were significantly correlated with adverse events (all P<0.05). Multivariate Cox regression analysis finally included LVEF, BNP and C-reactive protein to construct the prognostic score. In validation set, after the use of prognostic score for layered, low-risk and high-risk children with DCM non-event survival rates were 81.2% and 51.1% respectively (Log-rank P=0.049). Conclusion The prognostic score of PDCM long-term adverse events based on clinical risk factors is construct, it can be used for risk stratification.