Am J Perinatol 2021; 38(05): 436-448
DOI: 10.1055/s-0039-1700541
Review Article

Type 3 von Willebrand Disease in Pregnancy: A Systematic Literature Review

1   Division of Maternal-Fetal Medicine, Department of Obstetrics and Gynecology, Sidney Kimmel Medical College, Thomas Jefferson University, Philadelphia, Pennsylvania
,
Stephanie L. Kass
2   Sidney Kimmel Medical College, Thomas Jefferson University, Philadelphia, Pennsylvania
,
Melissa L. Russo
3   Division of Maternal-Fetal Medicine, Department of Obstetrics and Gynecology, Women and Infants' Hospital of Rhode Island, The Warren Alpert Medical School of Brown University, Providence, Rhode Island
,
Homa Ahmadzia
4   Division of Maternal-Fetal Medicine, Department of Obstetrics and Gynecology, George Washington University School of Medicine & Health Sciences, Washington, District of Columbia
,
Huda B. Al-Kouatly
1   Division of Maternal-Fetal Medicine, Department of Obstetrics and Gynecology, Sidney Kimmel Medical College, Thomas Jefferson University, Philadelphia, Pennsylvania
› Author Affiliations
Funding None.

Abstract

Objective von Willebrand disease (VWD) is a hereditary bleeding disorder. Type 3 VWD is the most severe and rare phenotype that presents many challenges for management of pregnant women. The aim of this study was to review the maternal characteristics and complications in pregnant women with Type 3 VWD.

Study Design A systematic literature search was performed to include all publications that address Type 3 VWD in pregnancy.

Results Thirteen studies met the inclusion criteria. There were 28 pregnancies with Type 3 VWD in 17 women. All were diagnosed with Type 3 VWD prior to pregnancy. Concentrate treatment was administered before delivery for 19 pregnancies and postpartum for 26 pregnancies. Eight pregnancies required blood products postpartum. Primary postpartum hemorrhage (PPH) was reported in 48% (10/21) and secondary PPH was reported in 56% (5/9). Secondary PPH occurred between 7 and 22 days. No study reported hysterectomies, intensive care unit admissions, or maternal mortality. All 28 pregnancies resulted in 28 live births at term.

Conclusion Our review highlights the maternal outcomes in patients with Type 3 VWD and the different approaches in management during pregnancy and delivery. Despite prior knowledge of this bleeding disorder, PPH was still a significant complication.

Authors' Contributions

Study conception and design: M.M.M. and H.B.A-K. Acquisition of data: M.M.M. and S.L.K. Analysis and interpretation of data and drafting of the article: M.M.M., S.L.K., M.L.R., H.A., and H.B.A-K. All authors agree to be accountable for all aspects of the study and approve the final version to be published.


Note

Search strategy of this work was assessed and reviewed by medical librarian Gary Kaplan.


Supplementary Material



Publication History

Received: 21 June 2019

Accepted: 07 September 2019

Article published online:
22 November 2019

© 2019. Thieme. All rights reserved.

Thieme Medical Publishers, Inc.
333 Seventh Avenue, 18th Floor, New York, NY 10001, USA