Laboratory monitoring during pregnancy and post‐partum hemorrhage in women with von Willebrand disease

Abstract

Background Evidence‐based guidelines recommend that von Willebrand factor (VWF) levels be obtained in the third trimester of pregnancy to facilitate peripartum planning for women with von Willebrand disease (VWD). Objectives To identify the frequency of third trimester monitoring in a nationally representative sample of pregnant women with VWD in the United States, as well as the frequency of reproductive bleeding after pregnancy. Patients/Methods Patient data were obtained from the Truven Health MarketScan Research Databases. International Classification of Diseases‐9 codes were used to identify women with VWD and evidence of infant delivery. We defined third trimester monitoring as a laboratory claim for VWF levels during the 3 months before delivery. Clinical outcomes studied included postpartum hemorrhage (PPH) and heavy menstrual bleeding (HMB). Results We identified 2238 unique pregnant females with VWD. Of these, 32% (n = 714) underwent third‐trimester testing of VWF levels. Diagnostic codes consistent with PPH were recorded for 6.5% of women in the 6 weeks after infant delivery. The frequency of PPH in women who underwent VWF monitoring (4.9%) was significantly lower than in those who did not undergo monitoring (7.3%), (risk difference −2.4%, 95% CI −4.4% to −0.3%, P = .023). Diagnostic codes consistent with HMB were recorded for 4.7% of women in the 3 months after infant delivery. Conclusions Third‐trimester VWF monitoring was associated with a lower risk of PPH, but testing was performed in only one‐third of insured pregnant women with VWD in the United States despite expert recommendations.

Examining international practices in the management of pregnant women with von Willebrand disease

“…While antenatal monitoring of plasma VWF levels is recommended, 13,17,18 the frequency and timing of monitoring may vary among centers 19 . Respondents advised assessment of plasma VWF levels in the third trimester most frequently (94.7%), either alone (28%), in combination with first trimester levels (29.5%), second trimester (4.5%), in all three trimesters (13.6%), or used another schedule.…”

“…Failure to evaluate plasma VWF levels in pregnancy has been associated with higher rates of PPH at delivery. 25 As a result, both Royal (UK) and American Colleges of Obstetricians and Gynecologists (RCOG, ACOG) recommend the antenatal assessment of plasma VWF levels in women with VWD, although their guidance differs in frequency. 13,18 The RCOG advise assessment of plasma VWF levels at booking, third trimester, and prior to any invasive procedures, with the ACOG favoring third trimester assessment to facilitate delivery planning.…”

“…While antenatal monitoring of plasma VWF levels is recommended, 13,17,18 the frequency and timing of monitoring may vary among centers. 19 Respondents advised assessment of plasma VWF levels in the third trimester most frequently (94.7%), either alone (28%), in combination with first trimester levels (29.5%), second F I G U R E 1 Geographical distribution of survey respondents: 10 participants indicated by red dot, 5 by yellow, single participant by blue dot trimester (4.5%), in all three trimesters (13.6%), or used another schedule. Of interest, for women with low VWF levels, 43.8% of respondents checked levels once during pregnancy and, if they had increased to within the normal reference range (>50 IU/dL), no further antenatal monitoring was performed.…”

Current Challenges in the Peripartum Management of Women with von Willebrand Disease

“…19 Despite these guidelines, a large U.S. sample identified that plasma VWF levels were assessed in only 32% of women with VWD in the third trimester. 41 This not only impeded care delivery but also resulted in direct clinical consequences, with a higher incidence of PPH in those whose plasma VWF levels were not reassessed (7.3 vs. 4.9%; p ¼ 0.023). 41 Prevention of PPH in women with VWD focuses on ensuring adequacy of plasma VWF and FVIII levels (as well as platelet count for patients with type 2B VWD) at the time of delivery.…”

“…41 This not only impeded care delivery but also resulted in direct clinical consequences, with a higher incidence of PPH in those whose plasma VWF levels were not reassessed (7.3 vs. 4.9%; p ¼ 0.023). 41 Prevention of PPH in women with VWD focuses on ensuring adequacy of plasma VWF and FVIII levels (as well as platelet count for patients with type 2B VWD) at the time of delivery. Traditionally, women with plasma VWF and/or FVIII levels < 50 IU/dL at the time of delivery will receive hemostatic support, targeting plasma VWF levels of 100 IU/dL at the time of delivery and maintaining >50 IU/dL for 3 to 7 days postpartum, depending on the mode of delivery.…”