Abstract: Objective：To evaluate the efficacy and safety of bosentan combined with phosphodiesterase inhibitor in the treatment of postoperative pulmonary hypertension in children with congenital heart disease. Methods：Computers were searched for PubMed， EMbase， The Cochrane Library， CBM， WanFang Data and CNKI databases to collect randomized controlled trials (RCT) of bosentan combined with phosphodiesterase inhibitors in the treatment of postoperative pulmonary hypertension in children with congenital heart disease. The search time limit is built until March 1， 2019. Two investigators independently screened the literature according to inclusion and exclusion criteria， extracted the data and evaluated the risk of bias in the included studies. Meta-analysis was performed using RevMan 5.3 software. Results：A total of 458 patients were included in the six RCTs. Meta-analysis showed that the total effective rate of bosentan combined with phosphodiesterase inhibitor was higher than that of single-drug phosphodiesterase inhibitor [OR=4.05， 95% CI (1.84， 8.95)， P=0.000 5]， lowering NYHAFC scores in children with pulmonary hypertension after congenital heart disease [MD=-0.21， 95% CI (-0.30， -0.12)， P<0.000 01]， Brog score [MD=-0.30， 95% CI (-0.37)， -0.23)， P<0.000 01]， pulmonary systolic pressure [MD=-9.65， 95% CI (-12.39，-6.90)， P< 0.000 01] and mean pulmonary artery pressure [MD=-7.94， 95% CI (-12.15)， -3.73)， P=0.000 2]； increased oxygen saturation SpO2 in children with pulmonary hypertension after congenital heart disease [MD=4.19， 95% CI (3.24， 5.14， P<0.000 01)， pulmonary circulation blood flow / Systemic blood flow ratio Qp/Qs [MD=0.16， 95% CI (0.06， 0.25， P=0.000 8) and prolonged 6MWD in children with pulmonary hypertension after congenital heart disease [MD=39.16， 95% CI (14.83， 63.49)， P=0.002]. However， in the incidence of adverse reactions [OR=0.60， 95% CI (0.30， 1.16)， P=0.13]， the difference between the two groups was not statistically significant. Conclusion：Compared with single-drug phosphodiesterase inhibitors，bosentan combined with phosphodiesterase inhibitors are more effective， can improve the total effective rate of children with pulmonary hypertension after congenital heart disease， and improve the pulmonary artery after congenital heart disease. Respiratory function， cardiac function and exercise capacity of high-pressure children， while reducing pulmonary artery pressure， but the effect on the incidence of adverse reactions is not clear. Due to the limitations of the number and quality of the included studies， the above conclusions need further research to verify， in the hope Provide evidence-based evidence for clinical medicine.