Bleeding Events and Other Complications during Pregnancy and Childbirth in Women with von Willebrand Disease.
Authors: James Andra H. , Jamison Margaret G. 
Published: April 2020

OBJECTIVE: Case reports and case series suggest that women with von Willebrand disease (VWD) are at an increased risk of bleeding and other complications during pregnancy, delivery and the postpartum period. The purpose of this study was to estimate the incidence of bleeding events and other complications in women with VWD during pregnancy and childbirth using a large national database.

METHODS: The Nationwide Inpatient Sample (NIS) from the Healthcare Cost and Utilization Project of the Agency for Healthcare Research and Quality for the years 2000–2003 was queried for all pregnancy-related discharges. Records with a diagnosis of VWD (ICD-9 code 286.4) were compared to records without a diagnosis of VWD. Logistic regression analyses using stratified sample weights were used to compute odds ratios with 95% confidence intervals.

RESULTS: There were 4067 deliveries to women with VWD which was 0.024% of all women who delivered. Black women (odds ratio [OR] 0.2, 95% confidence interval [CI] 0.1, 0.3) and Hispanic women (OR 0.3, 95% CI 0.2, 0.4) were significantly less likely to be diagnosed with VWD than white women. During pregnancy, women with VWD were more likely to be diagnosed with hypertension, cardiomyopathy, anemia and thrombocytopenia, but they were no more likely to develop preeclampsia, eclampsia or placental abruption and were less likely to develop gestational diabetes. Although they were more likely to experience antepartum bleeding, they were no more likely to labor prematurely, have a growth restricted fetus or suffer an intrauterine fetal demise. Although their risk of pulmonary embolism was no different than that of other women, none of the women with VWD experienced a deep vein thrombosis. Women with VWD were more likely to experience a postpartum hemorrhage and had a 5-fold increased risk of being transfused. 5 of the 4067 women with VWD died. Their maternal morality rate of 123 per 100,000 deliveries was 10 times higher than the rate for all other women which was 12.7 per 100,000 deliveries.

CONCLUSION: Although women with VWD do not appear to be at an increased risk of poor fetal outcomes, they are at an increased of bleeding events and death during pregnancy and childbirth.

Odds Ratios (OR) with 95% Confidence Intervals (CI) for Co-Existing Medical Conditions, Pregnancy-Related Complications and Events during Pregnancy in Women with VWD Count OR with 95% CI p value Co-Existing Medical Conditions: Hypertension 54 2.2 (1.1, 3.8) .03 Cardiomyopathy 9 6.8 (1.7, 27.5) .01 Diabetes 34 1.0 (0.4, 2.5) NS Anemia 551 2.1 (1.7, 2.6) &lt; .01 Thrombocytopenia 63 2.5 (1.4, 4.7) &lt; .01 Obesity 21 0.6 (0.2, 1.6) NS Smoking 220 1.9 (1.4, 2.7) &lt; .01 Pregnancy-Related Complications: Gestational diabetes 112 0.6 (0.4, 0.9) .02 Preeclampsia 327 0.9 (0.7, 1.20) NS Eclampsia 13 2.7 (0.7, 10.9) NS Placental abruption 53 1.0 (0.5, 1.8) NS Antepartum bleeding 280 10.2 (7.1, 14.6) &lt; .01 Preterm labor 417 1.1 (0.8, 1.4) NS Fetal growth restriction 49 0.7 (0.3, 1.5) NS Intrauterine fetal death 24 1.6 (0.7, 3.9) NS Thromboembolism, Hemorrhage & Infection: Deep vein thrombosis 0 - - Pulmonary embolus 5 2.0 (0.3, 15.1) NS Postpartum hemorrhage 261 1.5 (1.1, 2.0) &lt; .01 Transfusion 152 4.7 (3.2, 7.0) &lt; .01 Postpartum infection 54 1.0 (0.5, 1.9) NS