History and Objectives: Considering the significance of congenital heart disorders and lack of information on its prevalence among neonates in Iran and for determination of the effect of environmental and genetic factors in its etiology, this study was carried out during the years 1996-2000.

Materials and Methods: The study was conducted on existing data and the related data were collected in pre-designed questionnaires and all neonates with a definitive diagnosis of congenital heart disease were evaluated. In this respect, gender, age, type of disorder, family history, history of drug consumption during pregnancy, familial marriage and accompanied anomalies were considered.

Results: The results showed that out 2840 hospitalized neonates, 46 cases (1.6%) had congenital heart disease. The most common from was VSD observed in 22 cases (47.8%). The prevalence was 28.2%, 19.5%, 10.8%, 4.3% and 13% for ASD, TGA, PDA, TOF and other congenital heart disorders respectively. In addition, out of 46 neonates, 20 cases (43.5%) were female and 26 cases (56.5%) were male. Furthermore, in 41.3% of them, there was a familial marriage.

Conclusion: It is concluded that prevalence of CHD is higher than references, especially for TGA. Meanwhile, familial marriage can increase the incidence of CHD. Therefore, it is recommended to perform more studies in this field in Iran.