OBJECTIVE： To observe clinical effects of alprostadil combined with nitric oxide in the treatment of congenital heart disease complicated with pulmonary hypertension and its effects on immune function. METHODS： In retrospective study， 40 cases of congenital heart disease complicated with pulmonary hypertension in thoracic and cardiovascular surgery department of our hospital during Aug. 2009 to Feb. 2015 were selected and divided into nitric oxide group （single group） and alprostadil+nitric oxide group （combination group） according to therapy method， with 20 cases in each group. Both groups received conventional surgery. Single group was given extrinsic inhalation of nitric oxide （volume fraction of 20×10－6） after surgery； combination group was additionally given intravenous pump of alprostadil 5 ng/（kg·min） for consecutive 72 h， on the basis of combination group. The heart rate， left ventricular ejection fraction （LVEF）， mean arterial pressure， pulmonary arterial pressure， T lymphocyte and immunoglobulin （Ig） of children before and after treatment， and the occurrence of ADR were compared between 2 groups. RESULTS： Before treatment， there was no statistical significance in above indexes between 2 groups （P＞0.05）. After treatment， heart rate， LVEF， mean arterial pressure， the levels of CD3+， IgA， IgM and IgG in 2 groups had no significant change， but CD4+， CD4+/CD8+， Treg/CD4+ were increased significantly； pulmonary arterial pressure， the levels of CD8+， Th1， Th2， Th1/Th2 and Th17/CD4+， supplement C3 and supplement C4 were decreased significantly， compared to before treatment. Above indexes in the combination group was significantly better than the single group， with statistical significance （P＜0.05）. No obvious ADR was found in 2 groups. CONCLUSIONS： Alprostadil combined with nitric oxide could significantly reduce the pulmonary hypertension in children with congenital heart disease complicated with pulmonary hypertension， improve the level of T lymphocytes， and decrease the levels of supplement C3 and supplement C4 with good safety.