Congenital Factor VII Deficiency and Surgery In a Single Center Experience.

Abstract 3658

Congenital factor VII (FVII) deficiency is rare hemorrhagic disorder, and, in affected patients, surgery is likely to cause excessive bleeding. Recombinant form of activated factor VII (Novoseven^® from Novo Nordisk, Bagsvaerd, Denmark), that was developed as a second-generation bypassing agent, is used in management of bleeds in patients with congenital FVII deficiency recently. Doses lower than those recommended for patients with hemophilia are usually effective in patients with FVII deficiency.

We review the results of 6 surgical procedures in 4 patients with congenital FVII deficiency in East-West Neo Medical Center. Between January 2008 and February 2010, 6 surgeries for patients with congenital FVII deficiency were performed. We administerated rFVIIa pre- and postoperatively. Plasma FVII:C levels were measured in baseline, before and after the intravenous administration of routine dose FVIIa.

Between January 2008 and February 2010, 6 surgeries for 4 patients with congenital FVII deficiency were performed successfully without complications in our center. The median level of FVII:C was 2% (range 2–7%). The median age of patients was 29.5 years (range, 7–37). Types of surgery were 4 orthopedic surgeries, 1 tonsillectomy, and 1 dental extraction. The median duration of hospitalization was 14 days (range 6–15). rFVIIa was used at all surgery except dental extraction. FVIIa was administrated every 4 hours with the first dose being administered 1 hour preoperatively at least 48 h with dose 15–30 μμ¤/kg. After 48 h, the frequency was reduced according to the patient's clinical status. Plasma FVII:C levels were measured before testing and then again after the intravenous administration of routine dose FVIIa. FVII:C levels increased to about 200 %. Dental extraction was performed with only fibrinolytic agents without any replacement. There was no bleeding and thrombogenic complication.

rFVIIa was well-tolerated, maintained effective haemostasis with good clinical outcome. Patients with congenital FVII deficiency requiring surgery can be treated by the use of rFVIIa and antifibrinolytic agents efficiently and safely.