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