Outcome for Mothers and Neonates Among Pregnant Women with Von Willebrand Disease: A Prospective Nationwide Multicenter Study

• Published in: Blood 2024-11, Vol.144, p.3703-3703
• Main Authors: Huang, Qiusha, Zhu, Xiaolu, He, Yun, Fu, HaiXia, Chen, Qi, Zhao, Peng, Wu, Jin, Chang, YingJun, Xu, LanPing, Xu, Xue, Zhang, Xiaohong, Wang, Jianliu, Huang, Xiaojun, Zhang, Xiaohui
• Format: Article
• Language: English
• ISSN: 0006-4971
• DOI: 10.1182/blood-2024-205168

Introduction Von Willebrand disease (VWD) is the most common hereditary bleeding disorder characterized by defective platelet adhesion and aggregation. Women with VWD are at a greater risk of bleeding, which may affect the health of mothers and children during pregnancy and the intra- and postpartum periods. Given the wide heterogeneity of phenotypes and of the underlying pathophysiological mechanisms associated with the disorder, pregnancy and delivery in VWD represent a significant clinical challenge. There are only limited data on the outcomes of the mothers and the neonates among women with VWD during pregnancy and childbirth. In this study, we aimed to report the maternal and fetal outcomes of women with VWD and compare them with those of women without VWD. Methods This was a prospective nationwide multicenter observational study. We consecutively enrolled 210 pregnant women (302 pregnancies) with VWD from 18 academic tertiary centers in China from January 2013 to December 2023. Three controls were randomly selected for each VWD patient at the time of hospital admission (i.e., the control and case were hospitalized at approximately the same time). Finally, 302 pregnancies with VWD and 906 pregnancies without VWD were included in this prospective observational study. We compared outcomes for mothers and neonates in both groups. We also studied obstetrical complications in both groups. Logistic regression was used to compute odds ratios with 95% CIs. Results In total, 302 pregnancies of 210 patients were assessed (VWD type 1 n = 143, type 2A n = 18, type 2B n = 6, type 2M n = 5, type 2N n = 7, type 3 n = 1, subtype unidentified n = 30). The median age of patients at childbirth was 28 years. The patients' median age at VWD diagnosis was 20 (16-30) years. 82 (39.0%) women had a history of first trimester miscarriage. The patients' median ISTH bleeding score before pregnancy was 2 (0-11). Median gestational age at delivery was 38 (35-40) weeks. Among 302 pregnancies with 314 fetuses (12 twins), 301 neonates were live births, and 13 were intrauterine deaths thought to be unrelated to VWD. Among 301 neonates, 24 were born preterm, and 277 were born at term. No differences in premature delivery were observed between patients with VWD and those without VWD (P = 0.287). The median Apgar score at 10 min was 10.0. No neonatal deaths were observed in this study. And there were no cases of neonatal intracranial hemorrhage. Among those cases, 56 involved cesarean se