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

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.

To generate contemporary data on pregnancy outcomes among women with VWD using a statewide database, the Utah Population Database.

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).

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).

Despite increased awareness, women with VWD continue to face a higher risk of adverse pregnancy outcomes compared to the general population.