Risk of Postpartum Hemorrhage in Women With Von Willebrand Disease: A Real-World Data Analysis

Women with von Willebrand disease (VWD) are at increased risk for postpartum hemorrhage (PPH), but contemporary United States data on maternal outcomes and real-world peripartum treatment patterns are limited.

To evaluate maternal outcomes associated with claims-recorded VWD in a national claims cohort and describe exploratory treatment and geozip analyses.

We retrospectively analyzed FAIR Health claims from 2011-2021. Delivery encounters, VWD, and outcomes were identified using ICD-9/10 codes; laboratory confirmation was unavailable. Logistic regression estimated odds ratios (ORs) and 95% confidence intervals (CIs) for PPH, length of stay above the median, 30-day emergency department visit or readmission, and in-hospital mortality.

Among 1,498,768 delivery encounters, 1,514 (0.10%) involved claims-recorded VWD. Overall outcome frequencies were 3.79% for PPH, 24.81% for length of stay above the median, 7.43% for 30-day utilization, and 0.036% for mortality. Claims-recorded VWD was associated with PPH (OR 1.70, 95% CI 1.38-2.10), length of stay above the median (OR 1.58, 95% CI 1.41-1.78), and 30-day utilization (OR 1.20, 95% CI 1.01-1.43), but not mortality (OR 1.79, 95% CI 0.25-12.73). In exploratory analyses, VWF concentrate claims were associated with higher PPH odds versus neither captured desmopressin nor VWF concentrate (OR 3.17, 95% CI 1.28-7.87); desmopressin showed a similar non-significant pattern (OR 3.32, 95% CI 0.93-11.82).

Claims-recorded VWD was associated with PPH and greater peripartum healthcare utilization. Exploratory treatment associations may reflect confounding by indication, diagnostic misclassification, or treatment timing and cannot establish benefit or harm. Prospective studies incorporating laboratory and clinical data are needed.

Von Willebrand disease is the most common inherited bleeding disorder. For many women, it can cause heavy periods and can also increase the risk of heavy bleeding after delivery, called postpartum haemorrhage. Even though this risk is well recognised, there is still limited information about what happens in real-world delivery care across the United States. In this study, we looked at a large national insurance claims database that included almost 1.5 million delivery encounters from 2011 to 2021. Of these, 1,514 were among women with von Willebrand disease. Compared with women without von Willebrand disease, women with von Willebrand disease had higher odds of postpartum haemorrhage, longer hospital stays, and emergency department visits or readmissions within 30 days of delivery. We also looked at outcomes among women with von Willebrand disease based on whether treatment was documented in the claims data. Women who had claims for von Willebrand factor concentrate or desmopressin had higher observed odds of postpartum haemorrhage than women without documented treatment. This does not mean that the treatment caused bleeding. More likely, these women were treated because they were already considered to be at higher risk, had more severe disease, were actively bleeding, or raised greater concern for their clinical team. Overall, this study shows that postpartum bleeding remains an important and ongoing issue for women with von Willebrand disease. Future studies should include laboratory results, bleeding history, details about treatment timing and dose, delivery setting, and access to specialised bleeding disorder care. These details are needed to better understand which patients are at highest risk and how to improve the prevention of postpartum haemorrhage.