Type 3 von Willebrand Disease in Pregnancy: A Systematic Literature Review

Abstract

Objective von Willebrand disease (VWD) is a hereditary bleeding disorder. Type 3 VWD is the most severe and rare phenotype that presents many challenges for management of pregnant women. The aim of this study was to review the maternal characteristics and complications in pregnant women with Type 3 VWD. Study Design A systematic literature search was performed to include all publications that address Type 3 VWD in pregnancy. Results Thirteen studies met the inclusion criteria. There were 28 pregnancies with Type 3 VWD in 17 women. All were diagnosed with Type 3 VWD prior to pregnancy. Concentrate treatment was administered before delivery for 19 pregnancies and postpartum for 26 pregnancies. Eight pregnancies required blood products postpartum. Primary postpartum hemorrhage (PPH) was reported in 48% (10/21) and secondary PPH was reported in 56% (5/9). Secondary PPH occurred between 7 and 22 days. No study reported hysterectomies, intensive care unit admissions, or maternal mortality. All 28 pregnancies resulted in 28 live births at term. Conclusion Our review highlights the maternal outcomes in patients with Type 3 VWD and the different approaches in management during pregnancy and delivery. Despite prior knowledge of this bleeding disorder, PPH was still a significant complication.

Type 2B von Willebrand Disease in Pregnancy: A Systematic Literature Review

“…30 This is similar to the rates of primary and secondary PPH in type 3 VWD, the most severe type, which were 48% and 56%, respectively. 31 Based on our review, there was still a high rate of PPH in type 2B VWD despite factor concentrate treatment. Current guidelines from the National Heart, Lung, and Blood Institute recommend that VWF:RCo and FVIII levels be >50 IU/dL prior to delivery and to maintain VWF:RCo and FVIII above this level for 3 to 5 days post-delivery to prevent PPH.…”

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

“…The clinical impact of these issues is clear, with elevated rates of primary PPH in women with VWD despite prophylactic therapy at the time of delivery. 9,11,12 Large clinical trials to optimize care for pregnant women with VWD are lacking and management is largely based on expert-based consensus guidelines. 13,14 To assess current clinical practice in the management of pregnant women with VWD, we developed a clinician survey Thrombosis and Haemostasis (ISTH) conducted an international survey of health-care providers (HCP).…”

“…This introduces uncertainty regarding plasma VWF therapeutic targets peripartum and duration of treatment. The clinical impact of these issues is clear, with elevated rates of primary PPH in women with VWD despite prophylactic therapy at the time of delivery 9,11,12 . Large clinical trials to optimize care for pregnant women with VWD are lacking and management is largely based on expert‐based consensus guidelines 13,14 …”

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

“…53 In women with type 3 VWD, PPH rates are still significantly elevated even after prophylactic treatment. 54 These data raise important issues in the management of pregnant women with VWD. For some women with type 1 VWD or Low VWF levels, plasma VWF levels may increase during pregnancy into the laboratory normal reference range; however, as VWF levels remain below their pregnant peers, it is unfair to characterize this increase as "normalization."…”