Objective：To investigate the clinical efficacy and safety of sidenafil and bosentan in the therapy of children with pulmonary artery hypertension(PAH) and congenital heart disease(CHD). Methods：Fifty cases of PAH combined with CHD were selected and randomly divided into the A group and B group. A group was given sildenafil therapy，and on this basis the B group was added with bosentan tablets. The two groups were followed up periodically to analyze the clinical outcome，adverse reaction and complication.Results：There were 17 cases males and 13 cases females in the A group，and 8 cases males and 12 cases females in the B.①World Health Organization functional class was improved significantly in both after therapy，and the B group was superior to the A；②The PAH after treatment in both groups was improved，moreover the effect in the B group；③There was no significant changes in the assay，Guardianship residence time and the duration of ventilatory support. No severe side effect and complication were observed. Conclusion：The results revealed the efficacy of bosentan in children with PAH and CHD，and bosentan is safety and validity.