Congenital bleeding disorders and pregnancy

Congenital bleeding disorders are not so infrequent and they can increase frequency and severity of bleeding complications during and after pregnancy. Bleeding history, such as presence of menorrhagia, methrorrhagia, peritoneal haemorrhage at ovulation, bleeding after miscarriage or abortion or following childbirth, can identify patients with congenital bleeding disorders and anticipate possible complications during pregnancy: the most common congenital deficiencies of clotting factors are von Willebrand disease and haemophilia A and B carrier status. Complications are also reported for other rarer coagulopathies, including platelet disorders. The management and the prophylaxis of bleeding in pregnant women depend on the type of hemostatic defects and it is based on replacement therapy with the deficient clotting factor concentrate. Desmopressin, platelet transfusion and tranexamic acid are other useful weapons in particular occasions and diseases. Other general measures are to avoid forceps and vacuum extraction, to perform a Caesarean section for obstetrical indications only, to allow epidural and spinal anaesthesia only when haemostatic clotting factor levels can be warranted, and to consider the risk of primary and secondary postpartum haemorrhages. In conclusion, pregnancy in women with inherited bleeding disorders is at risk for complications and it requires a multidisciplinary approach involving consultants in obstetrics, anaesthesiology, haematology, and paediatrics.