Abstract
Pregnant persons with bleeding disorders (pwBD) have an increased risk of primary and secondary postpartum hemorrhage (PPH). Patients with von Willebrand disease, a bleeding disorder of unknown cause, or qualitative platelet defects (QPDs) and hemophilia carriers (HC) may or may not naturally achieve an adequately hemostatic state due to the hypercoagulable changes of pregnancy. PwBD greatly benefit from receiving care in a multidisciplinary setting including hematologists, obstetricians, anesthesiologists, and clinical geneticists. Factor levels should be obtained, at minimum, prior to conception as baseline as well as at the 34- to 36-week mark for delivery planning. However, target factor levels for delivery remain controversial given that many bleeding phenotypes do not predictably correlate with levels. Hemostatic therapies include antifibrinolytic agents, desmopressin, factor concentrates, and blood components such as cryoprecipitate, plasma, and platelets. Antifibrinolytics such as tranexamic acid have the most robust evidence for PPH management, though factor concentrates are now routinely utilized in certain circumstances. Blood products are an option for pwBD who have QPDs or when factor concentrates are not available. In patients with certain bleeding disorders, such as HCs, mode-of-delivery discussions must include consideration of the risks both to the affected neonate and to the mother. We favor selecting the mode of delivery based upon maternal indications whenever possible. Post partum, therapies may be continued for days or sometimes weeks after delivery, as pwBD are at high risk for delayed PPH.
