Abstract: Congenital heart disease is easy to cause birth defects, which can seriously affect the health of newborns. At present, the specific etiology and pathogenesis of congenital heart disease is still unclear. Plateau area has its unique environmental characteristics, and the most important factors leading to physiological response at the high altitude are atmospheric depression and the corresponding hypoxia. The incidence rate of congenital heart disease in atmospheric depression and hypoxia environments of plateau area is significantly higher than that in plain area. This study reviewed the research progress of the relationship between hypoxia and congenitalheart disease in plateau area.