Journal of cardiothoracic and vascular anesthesia
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J. Cardiothorac. Vasc. Anesth. · Oct 2022
Case ReportsPerioperative 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. ⋯ 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.