The features of the mode of delivery in patients with congenital deficiency of coagulation factor VII: case reports

Abstract

Congenital deficiency of clotting factor VII (FVII) — refers to rare hemorrhagic diatheses, for which no unified approaches to the management of pregnancy and childbirth have been developed. The analysis of hemostasiological management of four pregnant women with congenital hypoproconvertinemia is presented. Cases reports. All patients had no or minimal history of hemorrhagic syndrome. In two cases, the diagnosis was made during the actual pregnancy. Provision of hemostasis during delivery (two — vaginal delivery, two — cesarean section) was carried out with recombinant activated FVII (rFVIIa): in two cases, once at a dose of 15–30 mcg/kg, in one twice with repeated administration after 12 hours, in another one tranexamic acid was used. There were no hemorrhagic or thrombotic complications. The thromboelastography (TEG) during pregnancy did not correspond to the severity of hypoproconvertinemia: at FVII activity < 5%, the TEG parameters indicated hypercoagulation. Discussion. There is a weak correlation between the level of FVII activity in plasma and the severity of hemorrhagic syndrome. The decision on the mode of delivery in patients with FVII deficiency is made according to obstetric criteria. In most cases, a single administration of rFVIIa is sufficient at a dose of 15–30 mcg/kg at the beginning of labor or before performing a caesarean section.