1038 Type 1 von willebrand disease in pregnancy

Abstract

Type 1 von Willebrand Disease (VWD) is the most common subtype of VWD compromising 75% of VWD patients. Our objective was to evaluate the rate of postpartum hemorrhage (PPH) in women with Type 1 VWD. This is a systematic review of Type 1 VWD in pregnancy. The primary outcome was rate of PPH, defined as a cumulative blood loss greater than or equal to 1000 ml, or blood loss accompanied by signs and symptoms of hypovolemia within 24 hours postpartum or requiring blood products. Secondary PPH was defined as significant bleeding 24 hours to 12 weeks postpartum. Electronic databases were searched for eligible articles. The reference lists of all identified articles were examined to identify additional studies. Seven articles (n=144 pregnancies) met inclusion criteria. The rate of primary PPH was 4/144 (2.8%). The secondary PPH rate was reported in four studies and occurred in 7/48 pregnancies (14.6%), ranging from 2 to 19 days postpartum. Five studies reported on delivery route, with frequencies of spontaneous vaginal, operative vaginal and cesarean delivery being 54/62 (87%), 3/62 (5%) and 5/62 (8%), respectively. Desmopressin was used in 17/123 reported cases (13.8%). Tranexamic acid was only used in one of 43 deliveries (2.3%). Data regarding blood product transfusion was available for 31 pregnancies. Blood products were given in 5 pregnancies (3 received cryoprecipitate and 2 received red blood cells). According to this systematic review, the frequency of primary PPH in pregnancies affected by Type 1 VWD is 2.8%. This is similar to the overall PPH rates of 3% reported in the literature. Although the sample size was small, secondary PPH occurred in almost 15% of pregnancies, while in the overall obstetrical population this occurs in approximately 1% of pregnancies. Women with type 1 VWD may not be at increased risk of primary PPH; however, with the return of clotting factors to baseline in the postpartum period, these women appear to be at increased risk of secondary PPH.View Large Image Figure ViewerDownload Hi-res image Download (PPT)