Postpartum haemorrhage in pregnant carriers of haemophilia and women with von Willebrand disease: a nationwide inpatient database study.

BACKGROUND: Postpartum haemorrhage (PPH) is a major cause of maternal mortality worldwide. Previous studies have presented varying conclusions regarding the PPH risk in pregnant haemophilia carriers or women with von Willebrand disease (VWD). We aimed to evaluate PPH occurrence in this demographic using a nationwide inpatient database in Japan. METHODS: In this retrospective study, we identified women aged 15-49 years who gave birth while hospitalised between July 2010 and March 2021, using the Japanese Diagnosis Procedure Combination database. These pregnant women were categorised into three groups: the haemophilia, VWD, and control cohort groups. The assessed outcomes were PPH and interventions for bleeding. Multivariable logistic regression analyses were performed to assess the association between coagulation disorders and patient outcomes. RESULTS: We identified 113 pregnant women in the haemophilia group, 184 in the VWD group, and 1,459,451 in the control group. The outcomes of multivariable logistic regression analyses demonstrated that PPH occurrence was not higher in the haemophilia group (odds ratio, 0.74; 95% confidence interval, 0.46-1.17) than in the control group. Conversely, the VWD group was significantly associated with PPH (odds ratio, 1.46; 95% confidence interval, 1.05-2.02) and a higher incidence of interventions for bleeding (odds ratio, 2.49; 95% confidence interval, 1.55-4.00). CONCLUSIONS: Despite the absence of a substantial correlation between haemophilia and PPH in pregnant haemophilia carriers, a discernible association emerged between VWD and PPH in pregnant women. Healthcare providers need to be mindful of the high prevalence of undiagnosed VWD and prepare adequately for delivery.