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

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。

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

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