PB0224 - Effective management of surgical procedures of inherited FVII deficient patients – 15 years experience at University Hospital in Ostrava

Background: Inherited factor VII (FVII) deficiency is the most common of the rare inherited coagulation disorders. The clinical symptoms range from asymptomatic to severe life threatening disorders. The International Society on Thrombosis and Haemostasis Scientific Standardization Committee classified FVII deficiency as follows: severe FVII:C < 10%; moderate FVII:C 10-20%; mild FVII:C 20-50%.

Aims: Efficient haemostasis during surgery can be achieved with FVII:C 10-20%, depending on the type of surgery.

Methods: There are 45 patients with FVII deficiency registered at Haemophilia Comprehensive Care Centre in Ostrava (Czech Republic). A total of 25 patients have a severe form, 8 of them have FVII:C < 1%; three patients have a moderate and 16 patients have a mild form. Twenty-four of our patients with FVII:C < 20% underwent 36 surgical procedures between the years 2008 and 2022. Twenty-five operations were managed by substitution plasma-derived FVII (pdFVII, Factor VII Baxalta/ previously Baxter), and 10 patients were substituted by activated recombinant FVII (rFVIIa, NovoSeven®). In one case, both drugs were used sequentially.

Results: rVIIa was used at a dose of 15-30 ug/kg and pdFVII was used at a dose of 8-20 IU/kg every 4 hours during the first two days, day 3-6 every 6 hours, from day 7 every 8 hours. The time of the treatment was in the range of 1-10 days depending on the type of surgery. All patients were also given tranexamic acid as a concomitant therapy for 5-10 days post-surgery except for patients undergoing cataract surgery and renal tumour surgery. Thromboprophylaxis by low-molecular-weight heparin (LMWH) was used during 24 operations.

Conclusion(s): No excessive blood loss during operations was registered in any group of substituted patients. The postoperative course was without complications except for one pulmonary embolism on day 49 after nephrectomy for renal carcinoma in a man with FVII:C 1.4% and multiple risk factors for thromboembolism despite LMWH prophylaxis.