Data on the prevalence and impact of anemia and packed red blood cell (PRBC) transfusions in children with congenital heart disease are limited. Our objectives were to determine the prevalence of anemia and its impact and the impact of PRBC transfusion in the initial 5 days after surgical repair on postoperative outcomes in infants with ventricular septal defect (VSD) and atrioventricular (AV) canal. Retrospective chart review of infants (1 year old) (n = 195) with AV canal or VSD who underwent surgical repair at Children's Hospital of Michigan during a 10-year period. Statistical analyses (SPSS 17.0) included Chi square and Student t test as well as regression analysis with significance set at p = 0.05. Preoperative anemia was diagnosed in 45 of 195 (23%) children. Anemic infants had VSD more frequently (80%), significantly shorter bypass and cross-clamp durations, and higher red cell distribution widths. Postoperative outcomes and PRBC transfusions were similar in the groups. On regression analysis, AV canal was associated with a significantly lower (odds ratio 0.21; 95% confidence interval 0.07-0.68, p = 0.009) risk of anemia. Infants who received a PRBC transfusion (n = 42) had significantly lower birth weights as well as weights at surgery and longer postoperative durations of pressor use, ventilation, oxygen supplementation, and length of stay than those who did not (n = 153) receive transfusions. PRBC transfusion was independently associated with longer postoperative length of stay, oxygen, pressor use, and ventilator duration. Approximately 23% of infants with AV canal or VSD are anemic. PRBC transfusions in the initial 5 postoperative days, but not anemia, are independently associated with adverse postoperative outcomes. Further studies to evaluate conservative transfusion strategies in this population are needed.