Surgical management of patients with von Willebrand disease: summary of 2 systematic reviews of the literature

Romina Brignardello-Petersen, Abdallah El Alayli, Nedaa Husainat, Mohamad Kalot, Shaneela Shahid, Yazan Aljabirii, Alec Britt, Hani Alturkmani, Hussein El-Khechen, Shahrzad Motaghi, John Roller, Ahmad Dimassi, Omar Abughanimeh, Bader Madoukh, Alice Arapshian, Jean M. Grow, Peter Kouides, Michael Laffan, Frank W.G. Leebeek, Sarah H. O’BrienAlberto Tosetto, Paula D. James, Nathan T. Connell, Veronica Flood, Reem A. Mustafa*

*Corresponding author for this work

Research output: Contribution to journalReview articleAcademicpeer-review

6 Citations (Scopus)
26 Downloads (Pure)


von Willebrand disease (VWD) is the most common inherited bleeding disorder. The management of patients with VWD who are undergoing surgeries is crucial to prevent bleeding complications. We systematically summarized the evidence on the management of patients with VWD who are undergoing major and minor surgeries to support the development of practice guidelines. We searched Medline and EMBASE from inception through October 2019 for randomized clinical trials (RCTs), comparative observational studies, and case series that compared maintaining factor VIII (FVIII) levels or von Willebrand factor (VWF) levels at .0.50 IU/mL for at least 3 days in patients undergoing major surgery, and those with options for perioperative management of patients undergoing minor surgery. Two authors screened and abstracted data and assessed the risk of bias. We conducted meta-analyses when possible. We evaluated the certainty of the evidence using the Grading of Recommendations, Assessment, Development and Evaluations (GRADE) approach. We included 7 case series for major surgeries and 2 RCTs and 12 case series for minor surgeries. Very-low-certainty evidence showed that maintaining FVIII levels or VWF levels of .0.50 IU/mL for at least 3 consecutive days showed excellent hemostatic efficacy (as labeled by the researchers) after 74% to 100% of major surgeries. Low- to very-low-certainty evidence showed that prescribing tranexamic acid and increasing VWF levels to 0.50 IU/mL resulted in fewer bleeding complications after minor procedures compared with increasing VWF levels to 0.50 IU/mL alone. Given the low-quality evidence for guiding management decisions, a shared-decision model leading to individualized therapy plans will be important in patients with VWD who are undergoing surgical and invasive procedures.

Original languageEnglish
Pages (from-to)121-128
Number of pages8
JournalBlood advances
Issue number1
Publication statusPublished - 11 Jan 2022

Bibliographical note

Funding Information:
The authors thank Angela Weyand, Rezan Abdul-Kadir, Susie Cooper, Peter Kouides, Michelle Lavin, Margareth Castro Ozelo, and J. Evan Sadler for their invaluable assistance and ASH, ISTH, NHF, and WFH for their support of the guideline process, with specific thanks to Jenny Castano, Cary Clark, Rob Kunkle, Ellen Riker, Fiona Robinson, and Mark Skinner.

Publisher Copyright:
© 2022 by The American Society of Hematology. Licensed under Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International (CC BY-NC-ND 4.0), permitting only noncommercial, nonderivative use with attribution. All other rights reserved.


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