Primary postpartum haemorrhage in women with von Willebrand disease or carriership of haemophilia despite specialised care: a retrospective survey

Abstract

Pregnant women with bleeding disorders require specialised peripartum care to prevent postpartum haemorrhage (PPH). If third trimester coagulation factor levels are <0.50 IU mL(-1) , prophylactic treatment is indicated and administered according to international guidelines. However, optimal dose and duration are unknown and bleeding may still occur. The aim of this study was to investigate the outcome in women with von Willebrand disease (VWD) or haemophilia carriership treated according to current practice guidelines. From the period 2002-2011, 185 deliveries in 154 VWD women or haemophilia carriers were retrospectively included. Data on blood loss, bleeding disorder characteristics and obstetric risk factors were obtained. The outcome was primary PPH, defined as blood loss ≥500 mL within 24 h postpartum and severe PPH as blood loss ≥1000 mL. Primary PPH was observed in 62 deliveries (34%), 14 (8%) of which resulted in severe PPH. In 26 deliveries prophylactic treatment was administered due to factor levels below the 0.50 IU mL(-1) cut-off in the third trimester, 14 of which (54%) were complicated by PPH. We found an increased PPH risk in deliveries given prophylactic treatment compared with deliveries without (OR 2.7, 95% CI 1.2-6.3). In conclusion, PPH incidence was highest in deliveries with the lowest factor levels in the third trimester. Currently, delivery outcome in women with bleeding disorders is unsatisfactory, given the high PPH incidence despite specialised care. Future studies are required to optimise management of deliveries in this patient population.

Blood volume–based von Willebrand factor to prevent postpartum hemorrhage in von Willebrand disease

“…7 Postpartum blood loss in VWD At delivery, the mean estimated blood loss in women with VWD is 1.4-fold greater than in controls, 615 vs 448 mL, despite VWF concentrate treatment, and continues over the next 6 weeks postpartum, accompanied by a significantly lower hematocrit, 28.6% 7 ( Figure 1B). These findings, confirmed in retrospective studies, [8][9][10][11] remain poorly understood. Why do women with VWD experience excessive postpartum blood loss despite treatment?…”

“…3,4 Postpartum hemorrhage (PPH), defined as 500 cm 3 or greater blood loss in the 24 hours after vaginal delivery, is more common in women with VWD than healthy controls 5,6 and associated with blood loss anemia, red cell transfusion, and prolonged hospitalization. Yet, despite treatment with VWF concentrate at delivery, women with VWD have lower VWF levels and greater blood loss at delivery than pregnant controls without VWD 7,8 ( Figure 1A-B). Why current treatment fails is unknown, and the optimal dose and duration of VWF concentrate at delivery and in the postpartum period remains elusive.…”

Management of Inherited Bleeding Disorders in Pregnancy

“…However, the risk of secondary PPH may be high even despite replacement therapy 72 and therefore, a careful assessment of blood loss and thrombotic risk should be performed daily, and thromboprophylaxis prescribed if indicated.…”

“…52 Those with low levels in the third trimester can have higher risk even following factor concentrate. 72 Carriers should be identified as being at increased risk of PPH and offered uterotonics for the third stage of labour. 73 Clotting factor levels fall from 2 days postpartum and although the mechanism for cessation of bleeding is largely mechanical (through uterine contraction), borderline levels of factor VIII and IX are associated with increased risk of PPH.…”

Antifibrinolytic therapy for preventing VWD-related postpartum hemorrhage: indications and limitations

“…1 Besides addressing these causes in the VWD patient with PPH, certainly raising the VWF level if it has not yet normalized is in order, although how intensive and frequent dosing should be is still in question. [8][9][10] Typically, additional treatment includes antifibrinolytic therapy given the increased fibrinolysis in general associated with hemorrhage from the mucosal uterine surface. 11 Also, data are accruing in favor of antifibrinolytic therapy, 12 as a blood-conserving agent particularly in surgery 13,14 and in menorrhagia.…”