Purpose:Esophageal atresia is known to be associated with a variety of additi onal congenital anomalies in multiple organ systems.Emphasis on cardiovascular anomalies has been focused on aortic arch and intrinsic cardiac malformations.P ersistent left superior vena cava(PLSVC)is the most common venous thoracic ano maly in the general population and creates a problem when central venous access is required.This review was undertaken to define the incidence of PLSVC in infa nts with esophageal atresia and to determine if any subgroup of associated anoma lies poses additional risk.Methods:A retrospective,institutional review board-approved review of all children treated for esophageal atresia from 1993 to 20 02 at Riley Hospital for Children was undertaken.Of 118 children,89 had suffic ient data for inclusion.Charts were reviewed for gestational age,weight,type of atresia,echocardiogram,and associated anomalies.Statistical analysis was p erformed using the Fisher’s Exact test.Results:Of 89 children,8(9.9%;conf idence interval,4%-17%)had PLSVC compared with the reported incidence of 0.3%in the general population.Presence of additional organ system anomalies did not significantly increase relative risk for PLSVC.Conclusion:The incidence of PLSVC is significantly increased in children with esophageal atresia when compa red with the general population.This increased incidence of PLSVC is not influe nced by the presence of cardiac or other associated anomalies.This finding shou ld be kept in mind when central venous access is required in this patient popula tion. Purpose: Esophageal atresia is known to be associated with a variety of additi onal congenital anomalies in multiple organ systems. Emphasis on cardiovascular anomalies has been focused on aortic arch and intrinsic cardiac malformations. Persistent left superior vena cava (PLSVC) is the most common venous thoracic ano maly in the general population and creates a problem when central venous access is required. This review was undertaken to define the incidence of PLSVC in infants with esophageal atresia and determine if any subgroup of associated anoma lies poses additional risk. Methods : A retrospective, institutional review board-approved review of all children treated for esophageal atresia from 1993 to 20 02 at Riley Hospital for Children was undertaken. Of 118 children, 89 had sufficient data for inclusion. Chars were reviewed for gestational age, weight , type of atresia, echocardiogram, and associated anomalies. Statistical analysis was p erformed using the Fisher’s Exact test. Results: Of 89 children, 8 (9.9%; conf idence interval, 4% -17%) had PLSVC compared with the reported incidence of 0.3% in the general population. Presence of additional organ system anomalies did not significantly increase relative risk for PLSVC.Conclusion: The incidence of PLSVC is significantly increased in children with esophageal atresia when compa red with the general population.This increased incidence of PLSVC is not influeced by the presence of cardiac or other associated anomalies. This finding shou ld be kept in mind when central venous access is required in this patient popula tion.