Background: In Brazil, since 2001, the congenital abnormalities have represented the second cause of death in children younger than one year, with cardiovascular defects being responsible for 39.4% of these deaths.Objectives: To establish the prevalence and the characteristics of the congenital cardiac malformations in pediatric necropsies performed in Hospital Regional da Asa Sul, Brasília, DF, Brazil, from January 1996 to December 2007.Methods: This is a descriptive, cross-sectional study that reviewed 1,591 necropsies performed from January 1996 to December 2007 and found 189 (11.9%) with congenital cardiac malformations, included in this study.Results: The cardiac abnormalities were observed mainly in the group of live births (117/61.9%), followed by the stillbirths (35/18.5%), the infant group (30/15.9%) and the preschoolers' group (7/3.7%), with no cases identified among schoolaged children. The main alterations detected were: interatrial communication in 96 patients (27%), interventricular communication in 66 patients (18.5%) and patent ductus arteriosus in 51 (14.3%), with no predominance of either sex. In 133 patients (70.4%), the cardiopathies were multiple and in 96 (50.8%) they were associated with anomalies in other organs and systems; among these, 45 (23.8%) presented cardiopathies as syndrome components, especially trisomies, at all age ranges. Conclusions:The results of the present study show a high prevalence of congenital cardiac anomalies in our country and distribution and association that were similar to the ones observed in developed countries. The high mortality associated to such anomalies highlights the need for more comprehensive research in order to identify the risk factors and seek the primary prevention of some of these defects. ( awareness of the most prevalent anomalies and the possible associated risk factors can allow an early intervention directed at primary prevention, with a positive impact on the quality of life of the child and its family.The aims of the present study are to estimate the prevalence and study the morphologic characteristics of congenital cardiac malformations identified in pediatric necropsies performed at Hospital Regional da Asa Sul, Brasília, DF, Brazil, from January 1996 to December 2007. MethodsA descriptive, cross-sectional study was carried out, which consisted in the review of patients' files from the Cytopathologic and Pathologic Anatomy Center of Hospital Regional da Asa Sul (HRAS), of all deaths that occurred in this hospital between January 1996 and December 2007. The HRAS is a public hospital and a reference one for high-risk pregnancies in the Federal District (DF).Among the necropsies performed at the HRAS, all that presented cardiac malformations were reviewed by the author and a pathologist. The following variables were analyzed: maternal and child's age, necropsy diagnosis, sex, cardiac malformations and associated ones. Cardiorespiratory IntroductionCongenital malformations are associated with high mortality and it has been estima...