Is prophylaxis required for delivery in women with factor VII deficiency?

Abstract

Introduction Factor VII (fVII) deficiency is a rare congenital bleeding disorder in which fVII activity level and bleeding tendency do not completely correlate. Pregnancy and delivery present a significant hemostatic challenge to women with fVII deficiency. Treatment with recombinant factor VIIa (rfVIIa) carries a thrombotic risk and the literature is unclear whether prophylaxis is necessary prior to delivery. Aim To define management, hemorrhagic and thrombotic complications of pregnant women with fVII deficiency through a systematic review. Methods Medical databases (PubMed, MEDLINE, CINAHL, Academic Search Premier, Cochrane Library, Web of Science and Scopus) were searched using “factor VII deficiency” and “pregnancy” or “surgery.” Overall 34 articles, 4 abstracts, and 3 institutional cases were reviewed. Results Literature from 1953–2011 reported 94 live births from 62 women with fVII deficiency. The median fVII activity was 5.5%. Hemostatic prophylaxis was used in 32% of deliveries. Without prophylaxis, 40 vaginal deliveries and 16 cesarean sections were completed. The odds of receiving prophylaxis were 2.9 times higher in women undergoing cesarean section compared to vaginal delivery. Post-partum hemorrhage occurred in 10% of deliveries with prophylaxis and 13% of deliveries without prophylaxis. The fVII level did not significantly differ between women who did and did not receive prophylaxis. Conclusion We present the only systematic review of the management of pregnancy in fVII deficient women. No difference in post-partum hemorrhage was seen in deliveries with and without prophylaxis. Therefore we recommend that rfVIIa be available in the case of hemorrhage or surgical intervention, but not as mandatory prophylaxis.

Women with congenital factor VII deficiency: clinical phenotype and treatment options from two international studies

“…However, as we do not have a denominator (number of deliveries), we were unable to calculate the incidence of this bleeding event. Recently, a systematic review on 94 deliveries in women with FVII deficiency [32] reported a 13% incidence of postpartum bleeding in women who did not receive prophylaxis with rFVIIa (higher in caesarean sections than in vaginal deliveries) vs. 10% of those who were given RT; the lack of a significant difference led the authors to conclude that in this clinical setting, prophylaxis should not be considered as mandatory, but an individualized approach should be used based on the response to previous haemostatic challenges and the mode of delivery.…”

Guideline for the diagnosis and management of the rare coagulation disorders

“…PPH occurred in 13% of deliveries without FVII replacement, but also in 10% of deliveries with FVII replacement. Women with no history of bleeding did not experience PPH (Baumann Kreuziger et al , ).…”

How I treat postpartum hemorrhage

“…74 For most rare congenital factor deficiencies, baseline factor level does not correlate with propensity to bleed and for heterozygous women; it is usually sufficient to have factor concentrate available in the case of hemorrhage or surgical intervention, but not as routine prophylaxis. 75 However, women with homozygosity and/or a bleeding phenotype require treatment with specific factor concentrate (for fibrinogen, FVII, FXI, and FXIII deficiencies) or if this is unavailable, prothrombin complex concentrate (FII and FX deficiencies) or solvent detergent-fresh frozen plasma (FV and FXI deficiencies). Women with afibrinogenemia or FXIII deficiency require replacement therapy throughout pregnancy to prevent fetal loss.…”