目的 探讨西地那非治疗高海拔地区儿童先天性心脏病并发重度肺动脉高压的临床疗效.方法 选择2012 年1 月-2014 年12 月阿坝藏族羌族自治州人民医院儿科收治的先天性心脏病合并重度肺动脉高压患儿150 例,随机分为对照组和治疗组,每组各75 例.对照组采用常规方法治疗,治疗组在常规治疗基础上口服枸橼酸西地那非片,0.3 mg/kg,3 次/d.两组均连续治疗7 d.观察两组的临床疗效,同时比较两组治疗前后N 末端脑钠肽(NT-proBNP)、C 反应蛋白(CRP)、肺循环阻力指数(PVRI)、每分心输出量(CO)、氧合指数(OI)、肺动脉收缩压(PASP)、平均肺动脉压(mPAP)、血氧分压(PaO₂)、心指数(CI)、呼吸困难评分的变化.结果 治疗后,对照组和治疗组的总有效率分别为69.3%、92.0%,两组总有效率比较差异有统计学意义(P< 0.05).治疗后,两组患者呼吸困难评分、NT-ProBNP、CRP、mPAP、PASP 均显著降低,PaO₂、OI、CI、PVRI、CO 显著升高,同组治疗前后差异有统计学意义(P< 0.05);且治疗组这些观察指标的观察程度优于对照组,两组比较差异有统计学意义(P< 0.05).结论 西地那非治疗高海拔地区儿童先天性心脏病合并重度肺动脉高压临床疗效较好,可降低肺动脉阻力,改善肺循环血流动力学指标,具有较高的安全性和可靠性,值得在临床推广应用.

objective To investigate the efficacy of sildenafil in treatment of high altitude congenital heart disease in children complicated with severe pulmonary hypertension. Methods Patients (150 cases) with congenital heart disease in children complicated with severe pulmonary hypertension in Department of Pediatrics of People's Hospital of Aba Tibetan and Qiang Autonomous Prefecture from January 2012 to December 2014 were randomly divided into control and treatment groups. Each group had 75 cases. Patients in control group were given conventional treatment. Patients in treatment group were po administered with Sildenafil Citrate Tablets on the basis of the control group, 0.3 mg/kg, three times daily. Two groups were treated for 7 d. After treatment, the efficacy was evaluated, and the changes of NT-proBNP, CRP, PVRI, CO, OI, PASP, mPAP, PaO₂, CI, and dyspnea score in two groups before and after treatment were compared. Results After treatment, the efficacies in the control and treatment groups were 69.3% and 92.0%, respectively, and there were differences between two groups (P < 0.05). After treatment, dyspnea score, NT-ProBNP, CRP, mPAP, and PASP in two groups were significantly reduced, while PaO₂, OI, CI, PVRI, and CO in two groups were significantly increased, and the difference was statistically significant in the same group (P < 0.05). And these indicators in treatment group improved better than those in the control group, with significant differences between two groups (P < 0.05). Conclusion Sildenafil has clinical curative effect in treatment of high altitude congenital heart disease in children complicated with severe pulmonary hypertension, can reduce pulmonary artery resistance, and can significantly improve the hemodynamic parameters about pulmonary circulation with high safety and reliability, which is worth popularizing in clinical application.