The antifibrinolytic drug, tranexamic acid, decreases blood loss in adult patients undergoing cardiac surgery. However, its efficacy has not been extensively studied in children. Using a prospective, randomized, double-blind study design, we examined 41 children undergoing repeat sternotomy for repair of congenital heart defects. After induction of anesthesia and prior to skin incision, patients received either tranexamic acid (100 mg/kg, followed by 10 mg.kg-1.h-1) or saline placebo. At the onset of cardiopulmonary bypass, a second bolus of tranexamic acid (100 mg/kg) or placebo was administered. Total blood loss and transfusion requirements during the period from protamine administration until 24 h after admission to the intensive care unit were recorded. Children who were treated with tranexamic acid had 24% less total blood loss (26 +/- 7 vs 34 +/- 17 mL/kg) compared with children who received placebo (univariate analysis P = 0.03 and multivariate analysis P < 0.01). Additionally, the total transfusion requirements, total donor unit exposure, and financial cost of blood components were less in the tranexamic acid group. In conclusion, tranexamic acid can reduce perioperative blood loss in children undergoing repeat cardiac surgery.