A 2-year-and-7-month-old boy underwent an emergent reconstruction surgery of the right ventricle-pulmonary artery (RV-PA) conduit. Although he was successfully weaned from cardiopulmonary bypass (CPB) after reconstruction of the RV-PA conduit, the bleeding continued despite the massive transfusion of red blood cell (RBC), fresh frozen plasma (FFP), and platelets. Because of persistent bleeding and abnormal coagulation laboratory results, we administered the recombinant activated factor VII (rFVIIa), which was not approved for use in the treatment of post-CPB coagulopathy. After administration of rFVIIa, his coagulation data dramatically improved, the bleeding decreased, and the operation was able to be finished.
Keywords: Acquired hemophilia A; Pediatric cardiac surgery; Refractory bleeding; rFVIIa.