西地那非在儿童先天性心脏病相关性肺动脉高压中的应用

The effects of Sildenafil in children with pulmonary arterial hypertension due to congenital heart disease

目的:观察西地那非治疗先天性心脏病(CHD)相关性肺动脉高压(PAH)儿童患者的安全性和有效性。方法:选择13例年龄<18岁的CHD合并PAH的患者,口服西地那非每次0.25～1mg/kg,3次/d进行治疗。对比患者用药前后的6 min步行试验距离(6 MWT)、平均肺动脉压力(mPAP)、肺血管阻力指数(PVRI)、肺循环与体循环平均压比率(Pp/Ps)及肺循环与体循环阻力比率(Rp/Rs)的变化。结果:13例服药患者平均随访(9.5±6.2)个月,6 MWT平均增加(47.36±15.7)m,P<0.05。其中11例分别行用药前后的心导管检查,检查结果示mPAP从(87.1±8.4)mmHg(1 mmHg=0.133 kPa)降至(82.2±3.7)mmHg,P=0.1;PVRI从(24.5±7.4)Wood units m2降至(20.3±5.4)Wood units m2,P<0.05;Pp/Ps从(0.99±0.09)降至(0.89±0.05),P<0.05;Rp/Rs从(0.91±0.25)降至(0.86±0.17),P=0.5。所有患者服药期间无明显不良反应及肝肾功能异常。结论:西地那非在CHD相关性PAH儿童患者中应用是安全的,能显著改善患者的活动耐量,降低肺血管阻力。

Objective：To study the safety and effectiveness of Sildenafil in children with pulmonary ar-terial hypertension（PAH） due to congenital heart disease（CHD）.Methods：13 patients yonger than 18 years old had received sildenafil therapy.The dose of sildenafil was 1-4 mg·kg^-1·d^-1,3 times daily.The 6 minute walking distance test and the hemodynamics by cardiac catheterization〔including mean pulmonary artery pres-sure（mPAP）;pulmoanry vascular resistance index（PVRI）;the ratio of mean pulmonary artery pressure and systemic artery pressure（Pp/Ps） and the ratio of pulmonary vascular resistance and systemic vascular resist-ance（Rp/Rs）〕were compared before and after the therapy.Results：During sildenafil therapy,the 6-minute walking distance increased from（433 ± 79） m to（481 ± 78） m,P = 0.004.Mean PAP decreased from（87.1 ± 8.4） mmHg to（82.2 ± 3.7） mmHg,P = 0.1;PVRI significantly decreased from（24.5 ± 7.4） Wood units m2 to（20.3 ± 5.4） Wood units m2,P〈0.05.Pp/Ps decreased from（0.99 ± 0.09） to（0.89 ± 0.05）,P〈0.05.Rp/Rs decreased from（0.91 ± 0.25） to（0.86 ± 0.17）,P = 0.5.Liver and kidney reactive had no significant changes.No adverse events and systemic hypotension were attributed to sildenafil thearapy.Conclusion： Sildenafil improved exercise capacity and hemodynamics in children with PAH associated with CHD