Maternal and neonatal bleeding complications in relation to peripartum management in women with Von Willebrand disease: A systematic review

M.C Punt,M.L Waning,E.P Mauser-Bunschoten,M.J.H.A Kruip,J Eikenboom,L Nieuwenhuizen,A.B.U Makelburg,M.H.E Driessens,J.J Duvekot,M Peters,J.M Middeldorp,K.W.M Bloemenkamp,R.E.G Schutgens,A.T Lely,K.P.M Van Galen

doi:10.1016/j.blre.2019.100633

Abstract

Women with Von Willebrand disease (VWD) have an increased risk of developing postpartum hemorrhage (PPH). Our aim is to evaluate peripartum management strategies in relation to maternal and neonatal bleeding complications in VWD. Electronic databases were searched up to January 2019. Seventy-one case-reports and -series and 16 cohort studies were selected, including 811 deliveries. Cohort studies reported primary PPH in 32% and secondary PPH in 13% of the women. The overall primary PPH incidence in the individual patient data was 34%, similar between women who received prophylactic treatment to prevent PPH and those who didn't. Neonatal bleeding events were reported in 4.6% of deliveries. Overall, the available evidence on peripartum management in women with VWD was of low quality. The ongoing high risk for PPH is evident, despite prophylactic treatment, as well as the need for higher quality evidence from larger prospective cohort studies to improve management strategies.

Highlights

The most common inherited bleeding disorder is Von Willebrand disease (VWD)
Von Willebrand factor (VWF) levels increase during pregnancy in patients with VWD type 1
This review aims to answer the following research questions for women with VWD: 1. Which third trimester and peripartum management strategies have been published?

Summary

Introduction

In VWD, the Von Willebrand factor (VWF)-antigen level can be too low (VWD type 1), absent (VWD type 3) or dysfunctional (VWD type 2), resulting in decreased VWF-activity. Women with VWD, depending on the subtype of their disease and its severity, might need prophylactic treatment to prevent bleeding events. These potentially include prolonged bleeding after trauma, heavy menstruation and potential excessive postpartum blood loss (postpartum hemorrhage, PPH) [1]. Hemostasis is changed into a procoagulant state to prepare for childbirth This procoagulant state is the result of an increase in clotting factor levels and a decrease in anticoagulant clotting factors [2,3]. In VWD type 2, there is an increase in dysfunctional VWF-antigen, without increase of the VWF-activity

Objectives

Methods

Results

Discussion

Conclusion