Perioperative management in a patient with congenital factor VII deficiency following mechanical valve replacement: a case report

Congenital factor VII deficiency (CFVIID) is a rare hemorrhagic disorder which elevates perioperative bleeding risks. Mechanical prosthesis mandates lifelong anticoagulation, while international normalized ratio (INR) of CFVIID deviates significantly from the normal range which could not accurately reflect the real anticoagulation. Perioperative management for CFVIID patients undergoing mechanical valve replacement are highly challenging, with no clinical guidelines currently available. We reported a 59-year-old CFVIID patient undergoing mechanical valve replacement. Perioperative recombinant activated factor VII (rFVIIa) replacement secured hemostasis. After postoperative cerebral thrombosis, anticoagulation of warfarin was initiated and guided by factor Ⅱ/Ⅹ activity (30–45%) rather than INR. No bleeding or recurrent thrombosis was observed. Perioperative rFVIIa replacement guarantees hemostatic safety for cardiac surgery. Lifelong warfarin anticoagulation is essential for CFVIID patient with mechanical prosthesis. Monitoring factor Ⅱ/Ⅹ activity instead of INR can balance bleeding and thrombosis risks for such cases.