目的 探讨波生坦联合伊洛前列素治疗先天性心脏病（CHD）合并肺动脉高压（PAH）患儿的临床疗效及其对半乳糖凝集素-3（Gal-3）、生长分化因子-15（GDF-15）及氨基末端脑利钠肽前体（NT-proBNP）水平的影响。方法 前瞻性选择邯郸市妇幼保健院2019年1月-2021年1月期间收治的84例CHD合并PAH患儿为研究对象，遵照随机数字表法将患儿分为对照组（n=42）与试验组（n=42），对照组给予基础治疗+口腔雾化吸入伊洛前列素溶液，吸入用伊洛前列素溶液10 μg与生理盐水2 mL混合后雾化吸入，每次15 min，每天6次。试验组在对照组基础上加用波生坦分散片口服治疗，起始剂量2 mg·kg^-1，每天2次，持续4周，随后增加至4 mg·kg^-1，每天2次，持续4周。两组均持续治疗8周。比较两组患儿治疗前后心功能分级、肺血流动力学、肺功能、Borg呼吸困难指数（BDI）评分，同时检测患儿血清Gal-3、GDF-15、NTproBNP水平，观察治疗期间两组患儿不良反应发生情况。结果 治疗后对照组、试验组心功能分级均较治疗前均改善（P<0.05），且试验组心功能分级优于对照组（P<0.05）。治疗后两组肺动脉收缩压（PASP）、肺动脉舒张压（PADP）、平均肺动脉压（MPAP）均低于治疗前（P<0.05），用力肺活量（FVC）、第1秒用力呼气容积（FEV1）、第1秒用力呼气容积占用力肺活量比值（FEV1/FVC）均高于治疗前（P<0.05），且试验组PASP、PADP、MPAP均低于对照组（P<0.05），FVC、FEV1、FEV1/FVC均高于对照组（P<0.05）。治疗后两组BDI评分均低于治疗前（P<0.05），且试验组BDI评分低于对照组（P<0.05）。治疗后两组血清Gal-3、GDF-15、NT-proBNP水平均低于治疗前（P<0.05），且试验组血清Gal-3、GDF-15、NT-proBNP水平低于对照组（P<0.05）。试验组药物相关不良反应发生率（11.90%）与对照组（9.52%）比较，差异不具有统计学意义（P> 0.05）。结论 波生坦联合伊洛前列素治疗CHD合并PAH患儿，可以改善心功能、肺血流动力学、肺功能，降低BDI评分，调节血清Gal-3、GDF-15、NT-proBNP水平，且具有较高安全性。

Objective To investigate the clinical efficacy of bosentan combined with iloprost in treatment of congenital heart disease (CHD) complicated pulmonary artery hypertension (PAH) and its effects on the levels of galectin-3 (Gal-3) and growth differentiation factor-15 (GDF-15) and N-terminal pro-brain natriuretic peptide (NT-proBNP). Methods According to the random number table method, 84 children with CHD complicated PAH admitted from January 2019 to January 2021 from Handan Maternal and Child Health Hospital were divided into a control group (42 cases) and a experiment group (42 cases). The control group was given basic treatment + oral aerosol inhalation of iloprost solution, and the experiment group was given bosentan orally on the basis of the control group. The cardiac function classification, pulmonary hemodynamics, lung function, Borg dyspnea index (BDI) scores, and adverse reactions were compared between the two groups, at the same time, the levels of serum Gal-3 and GDF-15, NTproBNP were detected. Results After treatment, the cardiac function classification of the control group and the treatment group improved compared with before treatment (P < 0.05), and the cardiac function classification of the treatment group was better than that of the control group (P < 0.05). After treatment, the pulmonary artery systolic pressure (PASP), pulmonary artery diastolic pressure (PADP), and mean pulmonary artery pressure (MPAP) were all lower than before treatment (P < 0.05), and forced vital capacity (FVC), forced expiratory volume in the first second (FEV1), ratio of forced expiratory volume in the first second to forced vital capacity (FEV1/FVC) were all higher than before treatment (P < 0.05), and PASP, PADP, MPAP of the treatment group were lower than those of the control group (P < 0.05), and FVC, FEV1, FEV1/FVC were all higher than those of the control group (P < 0.05). After treatment, the BDI scores of the two groups were lower than those before treatment (P < 0.05), and the BDI score of the treatment group was lower than that of the control group (P < 0.05). After treatment, the levels of serum Gal-3, GDF-15, and NT-proBNP in the two groups were lower than those before treatment (P < 0.05), and the levels of serum Gal-3, GDF-15, and NT-proBNP in the treatment group were lower than those in the control group (P < 0.05). The incidence of drug-related adverse reactions in the treatment group (11.90%) was not statistically significant (P > 0.05) compared with the control group (9.52%). Conclusion The treatment of boshengtan combined with iloprost in children with CHD and PAH can improve heart function, pulmonary hemodynamics, lung function, reduce BDI score, and adjust the serum Gal-3, GDF-15, NT-proBNP levels, and has high safety.

R972

河北省自然科学基金资助项目（H2020402004）；邯郸市科学技术研究与发展计划项目（19422083040ZC）