7  Management during pregnancy and delivery

1 Prophylactic treatment during pregnancy

Due to a weak correlation between the FVII activity level and the bleeding tendency in FVII deficiency, prophylaxis with rFVIIa is only recommended in high-risk patients (as defined above).

2 Hemostatic treatment during delivery

From published case reports it seems like FVII levels usually increase during pregnancy in patients with non-severe FVII deficiency [13] and therefore, testing during third trimester is recommended before recommendation on hemostatic treatment at time of delivery. In a systematic review published 2022 by Kadir and Gomez [4] 114 women from 62 publications of whom 46 women had severe FVII-deficiency defined as FVII:C < 5. Many of the included publications are small and none of the included publications included patients from the Nordic countries. Vaginal bleeding in pregnant women occurred in four women (7%). Fortyfive deliveries were included in the systematic review. Caesarean section was performed in 50% of the women. Prophylactic treatment was administered in 33 deliveries (31 women) of whom 16 women received rVIIa but also pdFVII, FFP, platelet transfusion and tranexamic acid were used. Post-partum hemorrhage occurred in 27% (12/45) of the women in whom 42% needed blood transfusion. Bleeding occurred in some cases despite the use of hemostatic prophylaxis. There is also a risk of thrombosis in women with other risk factors for thrombosis and was reported in six women in this review (seven thrombotic events).

2.1 Recommendations for pregnancy and delivery

  • All patients with FVII deficiency should receive tranexamic acid at start of delivery or prior to caesarean section.

  • Irrespective of mode of delivery, rFVIIa or other hemostatic agents should be available in the case of hemorrhages.

  • Prophylactic treatment with rFVIIa during vaginal delivery and caesarean section should only be considered in high-risk patients.

  • rFVIIa is given 15-30 μg/kg prior to and post-partum every 4-6 hours for at least 3 days.