Perioperative Factor VIII Replacement in Severe Hemophilia A During Neonatal Supracardiac Total Anomalous Pulmonary Venous Connection Repair.
Is'haq Al Aamri, Madan Mohan Maddali, Hajer Nasser Al Nasseri et al.
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Case in brief
A neonate with genetically confirmed severe hemophilia A underwent surgical repair of supracardiac total anomalous pulmonary venous connection. The perioperative management utilized a structured, assay-guided intermittent-bolus recombinant factor VIII strategy, achieving supraphysiologic factor VIII levels. The surgery proceeded without intraoperative hemorrhagic instability, and the postoperative recovery was uneventful, with minimal drain output and no thrombotic complications.
What made this case unusual
This case highlights the effective use of intermittent bolus recombinant factor VIII in a high-risk neonatal cardiac surgery, contrasting with the typical reliance on continuous infusion.
Diagnostic / clinical pearl
Clinicians should consider intermittent bolus recombinant factor VIII with frequent monitoring as a viable strategy for managing hemostasis in neonates with severe hemophilia A undergoing complex surgeries.
Why it matters
This case underscores the importance of tailored coagulation management in high-risk surgical settings, potentially influencing practices in similar cases of congenital coagulopathy.