Postpartum Hemorrhage In Women With Von Willebrand Disease and Other Bleeding Disorders

Postpartum hemorrhage (PPH) is a cause of potentially serious morbidity and mortality in women of reproductive age. Prior data suggests that women with von Willebrand Disease (VWD) and other inherited bleeding disorders (OBD) experience PPH at a rate greater than the general population. Little published data exist regarding the incidence or predictors of PPH in women with VWD or OBD to guide obstetricians and hematologists in the management of these at-risk women.

In order to evaluate the incidence of PPH, clinical characteristics and obstetric outcomes of women with VWD and other inherited bleeding disorders we analyzed data from the Pennsylvania Health Care Cost Containment Council (PHC4) database between January 1, 2007 and December 31, 2011. The council collects data from over 4.5 million inpatient hospital discharges and ambulatory/outpatient procedure records each year from hospitals and freestanding ambulatory surgery centers in Pennsylvania. Data from all women with VWD and OBD (including factor XI deficiency, hemophilia A carriers, hemophilia B carriers) as well as all women who experienced PPH was extracted from the database. Data elements, in addition to demographic factors, were identified by ICD-9 codes, including medical comorbidities, hospital length of stay, obstetric complications, and birth outcomes. We compared data from women with VWD and OBD, specifically in those with and without PPH. Differences between groups were analyzed using chi-square or Fisher’s exact tests for categorical variables and two-sample t-tests for continuous variables.

Between 2007 and 2011, there were a total of 1,234 women with bleeding disorders, including 506 with VWD and 728 with OBD, who underwent delivery. Of these, 54 (4.4%) experienced PPH, including 28 (5.5%) with VWD and 26 (3.6%) with OBD. Among women with VWD, those with PPH were younger, 25.4 vs. 27.8 years (p=0.034), and nearly 2-fold more likely to have anemia, 27.6% vs. 14.2% (p=0.059), but had no greater frequency of caesarean section delivery (p=0.234) or pregnancy complications (p=0.678), including antepartum bleeding, preeclampsia, placental abruption, gestational diabetes, fetal growth restriction, preterm labor, or intrauterine fetal death. There were no differences in live births or mortality (all p≥0.999), nor in medical comorbidities (p=0.300), including diabetes, hypertension, obesity, and hyperlipidemia. Among women with OBD, those with PPH were over 5-fold more likely to have anemia, 48.1% vs. 8.6% (p<0.001), but had no higher rates of caesarean section delivery (p=0.234) or pregnancy complications, mortality or lower rates of live births (all p≥0.999).

The incidence of postpartum hemorrhage (PPH) in women with VWD is 5.5% and 3.6% in those with OBD. Importantly, anemia appears to be a marker of PPH in women with bleeding disorders as it is present in over one-fourth (27.6%) of women with VWD and PPH and in nearly one-half (48.1%) of those with OBD. These data suggest that screening hemoglobin, along with standard screening of factor levels in late pregnancy, may help identify those with VWD or OBD at risk for PPH. Future prospective studies will be needed to confirm this hypothesis.

No relevant conflicts of interest to declare.