Von Willebrand Disease and Pregnancy: A Review of Evidence and Expert Opinion

Abstract

von Willebrand disease (VWD) is a common, inherited bleeding disorder. There are three main types of VWD, which result in a quantitative or qualitative deficiency in von Willebrand factor (VWF) and in severe cases, also Factor VIII (FVIII). The severity of bleeding depends on the underlying pathophysiology. Type 1 VWD is usually mild, while types 2 or 3 VWD can be associated with moderate or significant bleeding. Managing pregnant women with VWD requires a multidisciplinary approach. Such patients are at increased risk of postpartum hemorrhage. Whether women with VWD are at increased risk of spontaneous abortion remains unclear. Because of increased risk of bleeding, there are special considerations for delivery and obstetrical analgesia. There is a lack of high-quality evidence supporting monitoring and treatment of VWD in pregnancy. Most experts recommend that FVIII and VWF levels be monitored prior to delivery and treatment initiated when levels remain below 0.50 IU/mL. Some experts consider desmopressin (DDAVP) to be the preferred initial treatment in type 1 and most type 2 VWD. DDAVP is relatively contraindicated in type 2B disease. Plasma-derived FVIII and VWF replacements are the treatment of choice in type 2B and 3 VWD and in type 1 or 2 VWD when patients do not respond to DDAVP.

A single‐centre study of management of pregnant women with von Willebrand disease

“…8,9 The optimal route of delivery and the type of anaesthesia used in women with VWD remain controversial. 10 Generally, if the VWF levels are higher than 50 IU dL −1 , experts consider vaginal delivery and caesarean section to be equally safe, 7 and epidural analgesia is considered a reasonable option in women with type 1 VWD but not in women with type 2 and type 3 VWD. 10 One-third (28%) of all deliveries in this series were by caesarean section and this is higher than observed in the healthy population (21%); in type 2 VWD patients, Herein, the overall incidence of primary PPH and severe primary PPH was 6.9% and 3.4%, respectively, and the incidence of secondary PPH was also low (2.3%).…”

“…10 Generally, if the VWF levels are higher than 50 IU dL −1 , experts consider vaginal delivery and caesarean section to be equally safe, 7 and epidural analgesia is considered a reasonable option in women with type 1 VWD but not in women with type 2 and type 3 VWD. 10 One-third (28%) of all deliveries in this series were by caesarean section and this is higher than observed in the healthy population (21%); in type 2 VWD patients, Herein, the overall incidence of primary PPH and severe primary PPH was 6.9% and 3.4%, respectively, and the incidence of secondary PPH was also low (2.3%). These results are likely to be a result of individual prophylactic care according to the type of VWD and preterm factor levels and are not greatly different to those reported in the healthy population during the same period in France: 5% for primary PPH and 2% for severe PPH.…”

How I treat type 2B von Willebrand disease

“…42 In women with type 2B VWD, VWF:Ag and VWF:RCo tend to increase (although not to normal pregnancy levels), and thrombocytopenia, if not evident prepregnancy, often becomes apparent and progressively worsens during pregnancy. 7,44,45 The patient returned at 20 weeks' gestation. An ultrasound revealed complete placenta previa, for which her obstetrician recommends cesarean section (C-section) at 34 weeks' gestation.…”

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