Pregnancy outcomes in women with Von Willebrand disease: a statewide cohort study

Background: Multiple studies conducted between 1990s and 2010s reported increased rates of postpartum hemorrhage (PPH) among women with von Willebrand disease (VWD), even with specialized peripartum care. Objective: To generate contemporary data on pregnancy outcomes among women with VWD using a statewide database, the Utah Population Database. Methods: We included women with a first live singleton birth at Intermountain Health or University of Utah facilities from January 1, 2008, to December 31, 2020. VWD cases were identified using a validated algorithm incorporating diagnosis codes, laboratory, and medication data. Each case was matched (∼1:20) to controls by maternal birth year and age at delivery. Pregnancy outcomes were obtained from Utah birth certificates; PPH became reportable in 2017. Mixed effects logistic regression was used to compare pregnancy outcomes between women with and without VWD for the full cohort (2008-2020) and a limited cohort (2017-2020). Results: We identified 120 women with VWD matched to 2356 controls for the full cohort. Compared with controls, women with VWD had higher odds of blood transfusion (adjusted odds ratio [aOR] 12.8, 95% CI, 4.11-40.1) and preterm birth (aOR 1.82; 95% CI, 1.04-3.20), after adjusting for ethnicity. In the limited cohort, VWD was not significantly associated with PPH (aOR 1.59, 95% CI, 0.36-6.95). Conclusion: Despite increased awareness, women with VWD continue to face a higher risk of adverse pregnancy outcomes compared to the general population.