目的 探討波生坦聯(lián)合伊洛前列素治療先天性心臟?。–HD）合并肺動脈高壓（PAH）患兒的臨床療效及其對半乳糖凝集素-3（Gal-3）、生長分化因子-15（GDF-15）及氨基末端腦利鈉肽前體（NT-proBNP）水平的影響。方法 前瞻性選擇邯鄲市婦幼保健院2019年1月-2021年1月期間收治的84例CHD合并PAH患兒為研究對象，遵照隨機(jī)數(shù)字表法將患兒分為對照組（n=42）與試驗(yàn)組（n=42），對照組給予基礎(chǔ)治療+口腔霧化吸入伊洛前列素溶液，吸入用伊洛前列素溶液10 μg與生理鹽水2 mL混合后霧化吸入，每次15 min，每天6次。試驗(yàn)組在對照組基礎(chǔ)上加用波生坦分散片口服治療，起始劑量2 mg·kg^-1，每天2次，持續(xù)4周，隨后增加至4 mg·kg^-1，每天2次，持續(xù)4周。兩組均持續(xù)治療8周。比較兩組患兒治療前后心功能分級、肺血流動力學(xué)、肺功能、Borg呼吸困難指數(shù)（BDI）評分，同時(shí)檢測患兒血清Gal-3、GDF-15、NTproBNP水平，觀察治療期間兩組患兒不良反應(yīng)發(fā)生情況。結(jié)果 治療后對照組、試驗(yàn)組心功能分級均較治療前均改善（P<0.05），且試驗(yàn)組心功能分級優(yōu)于對照組（P<0.05）。治療后兩組肺動脈收縮壓（PASP）、肺動脈舒張壓（PADP）、平均肺動脈壓（MPAP）均低于治療前（P<0.05），用力肺活量（FVC）、第1秒用力呼氣容積（FEV1）、第1秒用力呼氣容積占用力肺活量比值（FEV1/FVC）均高于治療前（P<0.05），且試驗(yàn)組PASP、PADP、MPAP均低于對照組（P<0.05），F(xiàn)VC、FEV1、FEV1/FVC均高于對照組（P<0.05）。治療后兩組BDI評分均低于治療前（P<0.05），且試驗(yàn)組BDI評分低于對照組（P<0.05）。治療后兩組血清Gal-3、GDF-15、NT-proBNP水平均低于治療前（P<0.05），且試驗(yàn)組血清Gal-3、GDF-15、NT-proBNP水平低于對照組（P<0.05）。試驗(yàn)組藥物相關(guān)不良反應(yīng)發(fā)生率（11.90%）與對照組（9.52%）比較，差異不具有統(tǒng)計(jì)學(xué)意義（P> 0.05）。結(jié)論 波生坦聯(lián)合伊洛前列素治療CHD合并PAH患兒，可以改善心功能、肺血流動力學(xué)、肺功能，降低BDI評分，調(diào)節(jié)血清Gal-3、GDF-15、NT-proBNP水平，且具有較高安全性。

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

河北省自然科學(xué)基金資助項(xiàng)目（H2020402004）；邯鄲市科學(xué)技術(shù)研究與發(fā)展計(jì)劃項(xiàng)目（19422083040ZC）