Objective To investigate the expression and significance of purine receptor P2X ligand gated ion channel 7(P2X7R)in children with congenital heart disease(CHD)and pulmonary hypertension(PAH).Methods 80 children with CHD admitted to our hospital from June 2020 to December 2022 were seleceted,including 18 cases of simple CHD,6 cases of CHD combined with mild PAH,20 cases of moderate PAH,and 16 cases of severe PAH.20 healthy children were selected as controls.The expression level of serum P2X7R using enzyme-linked immunosorbent assay was detected.The effect of hypoxia on the expression of P2X7R protein in human pulmonary vascular smooth muscle cells,the effect of silencing the P2X7R gene on the proliferation and migration of hypoxic human pulmonary vascular smooth muscle cells were observed.Results The serum P2X7R level in children with CHD combined with PAH was higher than those in healthy children,and as the condition of PAH worsened,the expression level of serum P2X7R was higher(P<0.05).Hypoxia reduced the expression level of P2X7R protein in human pulmonary vascular smooth muscle cells(P<0.05).Silencing the P2X7R gene reduced the proliferation and migration of hypoxic human pulmonary vascular smooth muscle cells(P<0.05).Conclusion The level of P2X7R in serum of children with CHD and PAH increased,and P2X7R may participate in the development of PAH by affecting the proliferation and migration of pulmonary vascular smooth muscle cells.