Hereditary factor VII deficiency in pregnancy: peripartum treatment with factor VII concentrate.

This report describes pregnant sisters with severe factor VII deficiency and peripartum management with factor VII concentrate. In this family, two affected members had previously developed severe postpartum hemorrhage when managed with fresh frozen plasma. Therapy-related complications owing to plasma infusion included viral disease transmission and pulmonary edema. Therefore, to lessen the risks of complications, specific factor therapy was initiated shortly before parturition in both patients. Factor VII concentrate was administered prior to delivery and every 6 hr for the next 72 hr to keep trough levels at approximately 10%. Based on peak and trough levels, the mean factor VII half-lives were determined to be 3.6 and 5.7 hr. Use of the concentrate was associated with uncomplicated delivery and minimal postpartum bleeding.