Abstract: Objective To describe the situation of perinatal birth defects(BDs) in Henan Province and analyze the epidemiological characteristics of perinatal congenital heart disease(CHD). Methods The information of children with CHD and the total number of live births in Henan Province from 2018 to 2019 were collected through Henan Provincial Maternity and Child Monitoring System and birth medical certificate of delivery institution. Study subjects were the children with CHD born from 2018 to 2019 and pregnant women who delivered children with CHD during the same period. Incidence of CHD and the rank of disease causes,as well as the proportion ratios of CHD of different genders, urban or rural areas, and maternal ages were analyzed. Results There were 2 409 990 live births in Henan Province from 2018 to 2019, among which 23 572 children were BDs, including 14 068 from 75 surveillance hospitals and 9 504 from 461 non-surveillance hospitals. The incidence of BDs in Henan Province increased from 91. 78/10 000 in 2018 to 104. 14/10 000 in 2019, with an annual growth rate of 13. 47% and an overall incidence rate of 97. 81/10 000. The incidence of CHD ranked the first in BDs, and its incidence was higher in 2019 than that in 2018. Among the sub-types of CHD, the top three were atrial septal defect/patent foramen ovale, patent duct us arteries us and ventricular septal defect, with proportion ratios of 58. 82%, 34. 61% and 9. 95%, respectively. Conclusion Maternal health education during pregnancy and prenatal screening/diagnosis should be strengthened to timely detect potential congenital defects and decrease the rate of birth defects. At the same time,birth defect surveillance program should be further improved to strengthen monitoring and reduce the occurrence of under-reporting.