Perioperative Hemostatic Management of a Newborn With Hereditary Hemophilia A Undergoing Emergent Surgery for Dextro-Transposition of the Great Arteries

Hemophilia A is an inherited bleeding disorder characterized by a lack of plasma clotting factor VIII (FVIII). In prophylaxis or during surgery, FVIII infusions are necessary to prevent bleeding. The authors describe the perioperative challenges and application of a multidisciplinary hemostatic management approach to a Caucasian male newborn, with antenatal diagnoses of moderate hemophilia A (2 IU/dL) and dextro-transposition of the great arteries requiring arterial switch surgery within the first month of life. Because both conditions are rare, only few reports in the literature are available describing perioperative management of hemophilia in neonates and children undergoing cardiac surgery. After baseline FVIII determination and normal standard coagulation studies, iterative intravenous pharmacist-prepared plasma-derived FVIII boluses were calculated (35 IU/kg) and administered intravenously every 6 hours for 24 hours, then switched to a continuous infusion and guided by daily chromogenic clotting FVIII activity assay for targeted values between 80 and 100 IU/dL. Successful cardiac surgery, using cardiopulmonary bypass, was performed with continuous infusion of FVIII at 5 IU/kg/h. Thirteen days after surgery, the FVIII antibody screening remained negative and continuous infusion was switched in favor of a daily intravenous bolus treatment to facilitate reconciliation to the center of origin. The authors' multidisciplinary strategy, established antenatally, allowed for successful care in this highly complex and rare situation.