Factor VII Deficiency in Pregnancy: A Case Series and Overview of the Literature

INTRODUCTION: Factor VII (FVII) deficiency is the most prevalent of the rare inherited coagulation disorders. Although FVII activity rises during pregnancy, the relationship between factor level and bleeding risk is inconsistent. Management of affected women lacks robust guideline support, and data from adequately sized case series are scarce.
METHODS: We retrospectively reviewed the clinical and laboratory records of 10 consecutive women with confirmed FVII deficiency who delivered at a tertiary referral centre between 2010 and 2023. Demographic data, bleeding and family history, FVII activity, INR, PT, mode of delivery, recombinant activated FVII (rFVIIa) dosing, estimated blood loss, incidence of postpartum haemorrhage (PPH), and transfusion requirements were recorded.
RESULTS: Mean FVII activity was 19.6 ± 15.4%; four patients (40%) had levels below 10%. Bleeding history was present in six (60%), most commonly menorrhagia. Mean gestational age at delivery was 38.2 ± 1.4 weeks. Six women (60%) delivered by caesarean section. rFVIIa prophylaxis was used in nine cases (90%), with a mean dose of 4.4 ± 2.3 mg. Mean estimated blood loss was 995 ± 486 mL. PPH occurred in four women (40%), three of whom had FVII levels above 10%, and all four had received prophylactic rFVIIa.
DISCUSSION AND CONCLUSION: FVII activity alone does not reliably predict bleeding risk during pregnancy. PPH can occur despite prophylactic rFVIIa, and management must be individualised according to factor level, bleeding phenotype, mode of delivery, and overall obstetric risk. Delivery should be planned in tertiary centres with multidisciplinary support and pre-agreed protocols for repeat rFVIIa dosing.

Keywords: Factor VII deficiency, Postpartum haemorrhage, Pregnancy, Recombinant activated factor VIIa