Clinical outcomes of peripartum fibrinogen replacement plus tranexamic acid in congenital hypofibrinogenemia: a single-centre case series of eight pregnancies

Pregnancy in congenital hypofibrinogenemia is rare and hemorrhage-prone, with no reported use of tranexamic acid (TXA). We retrospectively reviewed eight pregnancies in six women (2015-2025). One intrauterine fetal death occurred. Seven live births were complicated by preterm membrane rupture, placental abruption, and postpartum hemorrhage (PPH; 4/8). All received peripartum fibrinogen replacement; seven also received intravenous TXA (1-3 g). Despite predelivery fibrinogen <0.8-1.89 g/l, no major maternal bleeding occurred. All neonates had low fibrinogen but remained asymptomatic and healthy at discharge. Despite prior asymptomatic status, pregnancies are frequently complicated, yet neonatal outcomes remain favorable. In this small single-center observational cohort, combined peripartum fibrinogen substitution plus intravenous TXA (1-3 g) was well tolerated without maternal venous thromboembolism, even among patients with predelivery fibrinogen levels <1.0 g/l. This descriptive case series provides preliminary safety data for this rare obstetric population in TXA.