Aortic valve replacement in a patient with factor VII deficiency

Abstract

A 69-year-old man who had never experienced episodes of bleeding was admitted to our hospital with the complaints of dyspnea, palpitation and orthopnea. Electrocardiography, echocardiography and laboratory tests revealed atrial fibrillation, severe aortic stenosis and elevated INR level (2.18). Coagulation tests due to isolated INR elevation showed that the activity of factor VII (FVII) decreased to 9% and the patient was diagnosed with isolated FVII deficiency. As FVII activity<10% is associated with high risk of bleeding during surgical procedures, recombinant FVII (rFVIIa) 40 mcg/kg was administered intravenous bolus infusion two hours before surgery. Aortic valve replacement was performed using a-23 mm bioprosthesis. The activity level of FVII during anesthesia induction and aortic cross declamping were 29% and 22%, respectively. Hemostasis was easy to secure and no extra dose of rFVIIa was given. The patient did not experience any postoperative problem or severe bleeding. Warfarin sodium was not administered to the patient, as he had atrial fibrillation, as well as bioprosthesis implantation and an INR level of 2.5. At the end of a-two-year follow-up period, the patient had a good exercise tolerance without any bleeding or thromboembolic complication. Factor VII deficiency is an extremely rare inherited bleeding disorder. Replacement therapy with rFVIIa with close monitoring of FVII activity is a reliable way to manage patients with FVII deficiency who are scheduled to undergo valvular cardiac surgery.