摘要Background Congenital heart diseases(CHD)are the most common type of birth defects,affecting millions of newborns every year;no prevalence data are available in S?o Paulo State,Brazil's most populous state.The objective is to identify trends in prevalence and risk factors for CHD in S?o Paulo State,Brazil.Methods We developed a population-based study to estimate the CHD trend of prevalence in recent years,stratified by maternal age and geographical clusters,using all cases of CHD identified by the Live Births Information System(SINASC-Sistema de Informa??o Sobre Nascidos Vivos)from January 1,2005,to December 31,2018.We calculated the trends of prevalence,the regression coefficient(β),the annual percent change(APC),and 95％confidence interval using the Prais-Winsten regression model,with the Durbin-Watson test.Results We found 10,594 cases of CHD among 8,536,101 live births(LB),a prevalence of 12.4/10,000 LB.There was no difference in the sex distribution;they are primarily Caucasian(60.2％),75.2％born at term,and 74.4％weight＞2500 g,66.9％of births were by cesarean section.S?o Paulo State presented an increasing CHD trend of prevalence(APC=18.9％).The highest CHD prevalence rate was in mothers aged≥35 years(22.2/10,000 LB).There were 12,271 specific congenital heart defects among 10,594 patients(1.16 CHD/patient).Atrial septal defect has the highest number of cases(3835),with a prevalence of 4.49/10,000 LB,corresponding to 31.3％of all CHD.Conclusion CHD had an increasing prevalence trend in recent years,being highest in S?o Paulo City and≥35-year mothers.