Perioperative Factor VIII Replacement in Severe Hemophilia A During Neonatal Supracardiac Total Anomalous Pulmonary Venous Connection Repair.
Source: PubMed, NCBI / U.S. National Library of Medicine
Neonatal cardiac surgery in severe hemophilia A remains rarely reported, with limited data guiding perioperative coagulation management during cardiopulmonary bypass. This report describes neonatal repair of supracardiac total anomalous pulmonary venous connection in genetically confirmed severe hemophilia A using a structured, assay-guided intermittent-bolus recombinant factor VIII strategy rather than continuous infusion. Perioperative management targeted supraphysiologic factor VIII activity, with serial monitoring of factor levels, activated clotting time, and rotational thromboelastometry to integrate anticoagulation, antifibrinolysis, and hemostatic replacement. Cardiopulmonary bypass was conducted with standard heparinization, and surgical repair was completed without intraoperative hemorrhagic instability. Postoperative recovery was characterized by minimal drain output, absence of reexploration, and no thrombotic complications, despite factor VIII levels exceeding 200% to 300% in the early perioperative period. This case provides high-resolution perioperative coagulation profiling in a neonate with severe hemophilia A undergoing complex congenital cardiac surgery. It demonstrates that intermittent bolus recombinant factor VIII, when combined with frequent laboratory surveillance and viscoelastic guidance, can provide stable hemostasis during and after cardiopulmonary bypass. This approach challenges the prevailing preference for continuous infusion and offers a pragm
Abstract
Neonatal cardiac surgery in severe hemophilia A remains rarely reported, with limited data guiding perioperative coagulation management during cardiopulmonary bypass. This report describes neonatal repair of supracardiac total anomalous pulmonary venous connection in genetically confirmed severe hemophilia A using a structured, assay-guided intermittent-bolus recombinant factor VIII strategy rather than continuous infusion. Perioperative management targeted supraphysiologic factor VIII activity, with serial monitoring of factor levels, activated clotting time, and rotational thromboelastometry to integrate anticoagulation, antifibrinolysis, and hemostatic replacement. Cardiopulmonary bypass was conducted with standard heparinization, and surgical repair was completed without intraoperative hemorrhagic instability. Postoperative recovery was characterized by minimal drain output, absence of reexploration, and no thrombotic complications, despite factor VIII levels exceeding 200% to 300% in the early perioperative period. This case provides high-resolution perioperative coagulation profiling in a neonate with severe hemophilia A undergoing complex congenital cardiac surgery. It demonstrates that intermittent bolus recombinant factor VIII, when combined with frequent laboratory surveillance and viscoelastic guidance, can provide stable hemostasis during and after cardiopulmonary bypass. This approach challenges the prevailing preference for continuous infusion and offers a pragmatic, resource-adaptable strategy for managing congenital coagulopathy in high-risk neonatal cardiac surgery.