The Effect of Oral Sildenafil Therapy on Elevated Pulmonary Vascular Resistance in patients with Pulmonary Hypertension Secondary to Congenital Heart Disease

Abstract

This was a prospective study conducted on 22 patients, who were referred for elective hemodynamic study due to PAH secondary to CHD in Ain Shams University Hospitals over a period of 30 months, between March 2012 and July 2014. Sildenafil affected functional class and there was improvement in 9 patients. PVR decreased in ten patients and successfully performed total repair while on sildenafil therapy in one patient. PVR increased in 6 patients and no change occurred in one patient with DILV&VSD (Taussing Bing anomaly). While four patients died on sildenafil therapy.There were statistically significant increase in PA oxygen saturation 92.9 % compared to 80.3 % in cases with increased PVR after oral sildenafil therapy (p value 0.002), aortic oxygen saturation increased significantly 99.2% compared to 82.4% in 2nd group (p value 0.004), pulmonary blood flow Qp 9.5 (L/min/m2) compared to 3.4(L/min/m2) (p value 0.002). Sildenafil was well tolerated, and no patients withdrew. There was no change in creatinine,urea,liver function tests,or platelet count. There were no significant change in systemic blood pressure, pulmonary blood pressure, PVR, SVR, left and right atrial pressures, superior vena caval oxygen saturation and hemoglobin in cases before and after treatment with oral sildenafil. In this study,sildenafil affected functional class and not hemodynamics . Whether or not longer term use of sildenafil therapy will lead to actual improvement in the hemodynamic parameters and reduction in PVR remains a question that needs longer term follow up in larger patient groups.