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Semin Thromb Hemost 2021; 47(02): 217-228
DOI: 10.1055/s-0041-1723797
Review Article

Current Challenges in the Peripartum Management of Women with von Willebrand Disease

Bridgette Byrne
1   Department of Obstetrics & Gynaecology, Coombe Women and Infants University Hospital, Dublin, Ireland
2   Department of Obstetrics & Gynaecology, Royal College of Surgeons in Ireland, Dublin, Ireland
,
Kevin Ryan
1   Department of Obstetrics & Gynaecology, Coombe Women and Infants University Hospital, Dublin, Ireland
3   National Coagulation Centre, St. James' Hospital, Dublin, Ireland
,
Michelle Lavin
3   National Coagulation Centre, St. James' Hospital, Dublin, Ireland
4   Irish Centre for Vascular Biology, School of Pharmacy & Biomolecular Sciences, Royal College of Surgeons in Ireland, Dublin, Ireland
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Abstract

For many women, pregnancy and childbirth represent their first major hemostatic challenges. Despite advancements in obstetric care, up to 2 to 5% of all deliveries are complicated by postpartum hemorrhage (PPH). To mitigate bleeding risk, physiological changes occur in pregnancy, including increases in plasma von Willebrand factor (VWF) and factor VIII levels. For women with von Willebrand disease (VWD), these physiological alterations are blunted or absent. As a result, women with VWD have a heightened risk of PPH, both primary (in the first 24 hours) and secondary (>24 hours to 6 to 12 weeks postpartum). Pregnancy and delivery management for women with VWD should therefore be carefully coordinated as part of a multidisciplinary team approach. In the absence of large-scale clinical trials, the management of women with VWD during pregnancy is guided by expert consensus guidelines. Clinical practices internationally are not uniform, and areas of considerable clinical uncertainty exist. Traditional peripartum plasma VWF thresholds for hemostatic cover and therapeutic targets are currently under scrutiny, as PPH is not eliminated in women with VWD who receive replacement therapy. The benefit and optimal duration of postpartum tranexamic acid have yet to be defined, and standardized methods of quantification of blood loss at the time of delivery are currently lacking. In this article, we review the evidence base to date and explore the current clinical challenges in the management of pregnant women with VWD.

Keywords

von Willebrand disease - von Willebrand factor - epidural anesthesia - postpartum hemorrhage - obstetrics

Authors' Contributions

M.L., K.R., and B.B. collected and analyzed the data. All authors (B.B., K.R., M.L.) were involved in writing and reviewing the paper.




Publication History

Article published online:
26 February 2021

© 2021. Thieme. All rights reserved.

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