Management of Hereditary Hypofibrinogenemia During Pregnancy: A Scoping Review Towards Personalized Obstetric Care

Background: Hereditary fibrinogen disorders comprise a rare and heterogeneous group of conditions characterized by highly variable clinical phenotypes, ranging from entirely asymptomatic to severe hemorrhage or paradoxical thrombosis. Within this spectrum, hereditary hypofibrinogenemia (HH) poses a significant obstetrical challenge due to the lack of evidence-based management guidelines during pregnancy. Methods: A scoping review of the literature was conducted to identify reported cases of pregnancies with HH reaching the third trimester. PubMed, Scopus, and Cochrane Library were searched through April 2026 for eligible studies reporting maternal and neonatal outcomes, fibrinogen replacement therapy during pregnancy, and peri- and postpartum management. A complementary LeapSpace search was also performed. Data were extracted using a structured form and owing to the heterogeneity and descriptive nature of the available evidence, results were synthesized narratively. Results: Out of 202 unique records identified, a total of 13 studies, comprising 33 pregnancies, were included. All evidence arose from case reports and small case series, with substantial variability in patient characteristics and clinical management. Successful outcomes are associated with early diagnosis, careful assessment of medical and obstetrical history, and close multidisciplinary surveillance. Maintaining fibrinogen levels above 50–100 mg/dL during pregnancy and ≥150 mg/dL peripartum appeared beneficial. The use of global coagulation assessment tools such as rotational thromboelastometry (ROTEM®), particularly the FIBTEM® assay, may support individualized management beyond fibrinogen levels alone; however, up to date it has been incorporated in the management of a single pregnancy. Conclusions: Management of pregnancy in women with HH should be individualized and multidisciplinary, with tailored fibrinogen supplementation strategies to optimize maternal and neonatal outcomes. Small sample sizes and the heterogeneity of the reported results limit the certainty of these findings, requiring further research to establish subtype-specific recommendations and to define additional coagulation parameters that may improve perinatal care.