Background: Nearly half of the population of Northwest China live in Shaanxi province, but population-based data on the epidemiologic characteristics of congenital heart defects (CHD) in this population is limited. The study aimed to investigate the prevalence and epidemiologic characteristics of the CHD among infants born between 2010 and 2013 in Shaanxi province. Methods: Infants born between 2010 and 2013 in Shaanxi province were surveyed using a stratified multi-stage sampling method. Participant characteristics were recorded by questionnaire, medical records were reviewed and CHD was diagnosed using a specialized neonatal echocardiography. A Poisson regression model was applied to assess the association between any CHD and possible risk factors. Results: A total of 29098 live infants were surveyed with an overall prevalence of 76.0 (95% CI: 66.3, 86.7) per 10000 live infants. The prevalence of major and minor CHD were 26.1 and 49.8 per 10000 live infants, respectively, in surveyed areas. Poisson regression analysis indicated that, compared with singleton infants, the prevalence rate ratio of CHD was higher in twin and multi-fetal infants (PRR: 3.1, 95% CI: 1.6, 6.1). Using southern Shaanxi as a reference, the PRR of CHD were lower in northern (PRR: 0.4, 95% CI: 0.3, 0.6) and central Shaanxi province (PRR: 0.5, 95% CI: 0.4, 0.7). PRR was higher in mothers over 30 years of age than in those under 25 years (PRR: 1.6, 95% CI: 1.0, 2.5), and in mothers with >= 3 parity than that in mothers with only one parity (PRR: 2.2, 95% CI: 1.2, 4.2). The risk for CHD among live infants was positively associated with family history of CHD (PRR: 9.8, 95% CI: 5.3, 18.1). Additionally, CHD was less common in the floating population than the permanent population (PRR: 0.6, 95% CI: 0.4, 0.9). Conclusion: The CHD among live infants seemed to be a serious health problem in Shaanxi province as well as in Northwestern China. Our research have important policy implications for recommendations on CHD intervention in Northwest China.