Maternal and neonatal bleeding complications in relation to peripartum management in women with Von Willebrand disease: A systematic review

Punt, Marieke C; Waning, M. L.; Mauser‐Bunschoten, E. P.; Kruip, Marieke J.H.A.; Eikenboom, Jeroen; Nieuwenhuizen, Laurens; Mäkelburg, Anja B. U.; Driessens, M. H. E.; Duvekot, Johannes J.; Peters, Marjolein; Middeldorp, Johanna M.; Bloemenkamp, Kitty W. M.; Schutgens, Roger E. G.; Lely, A. Titia; Galen, Karin P M van

doi:10.1016/j.blre.2019.100633

Cited by 30 publications

(36 citation statements)

References 100 publications

(168 reference statements)

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“…Women with bleeding disorders are at increased risk for postpartum haemorrhage (PPH), which occurs for example 2‐3 times more often compared to the normal population in woman with von Willebrand disease (VWD) and carriers of haemophilia 4‐6 . Optimal management strategies to prevent PPH and secure safe delivery for the possibly affected child are currently undefined.…”

Section: Introductionmentioning

confidence: 99%

Clinical management of woman with bleeding disorders: A survey among European haemophilia treatment centres

et al. 2020

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Introduction The impact of bleeding for women with bleeding disorders (WBD) is of increasing focus and importance. Despite this, optimal management strategies are unclear and knowledge gaps persist. Aim To examine practices and define research priorities on diagnosis and management of WBD in Europe. Methods An electronic survey on clinical management of WBD was sent to 136 European haemophilia treatment centres (HTCs), including open questions on knowledge gaps and research priorities. Results Fifty‐nine HTCs from 12 Western (WE) and 13 Central/Eastern European (CEE) countries completed the survey. Less than half runs a joint clinic (24 HTCs, 42%). Most centres without a joint clinic have a named obstetrician (81%) and/or gynaecologist (75%) available for collaboration. Overall 18/54 (33%) European HTCs do not offer preimplantation genetic diagnosis. Third trimester amniocentesis to guide obstetric management is available 28/54 HTCs (52%), less frequent in CEE compared to WE countries (5/17 vs 23/37, P = .03). 53% of HTCs (28/53) reported that only 0%‐25% of WBD seek medical advice for heavy menstrual bleeding (HMB). An algorithm managing acute HMB in WBD is lacking in 22/53 (42%) HTCs. The main reported knowledge and research gaps are lack of awareness & education on WBD among patients and caregivers, optimal diagnostic strategies and effective multidisciplinary management of pregnancy & HMB. Conclusion Joint clinics, prenatal diagnostics and algorithms for managing acute HMB are lacking in many European HTCs. HMB may be an underestimated issue. This survey highlights the need to prioritize improvement of knowledge and patient care for WBD across Europe.

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Section: Introductionmentioning

confidence: 99%

Clinical management of woman with bleeding disorders: A survey among European haemophilia treatment centres

et al. 2020

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“…Recent studies have challenged the traditional approach to treating plasma VWF:Act of 100 IU/dL peripartum. 8,36,52 Despite supplementation with VWF concentrates, women who require treatment remain at an increased risk for PPH in comparison to those who do not require replacement therapy (odds ratio: 3.9; 95% confidence interval: 1.2-12.4). 36 In a prospective study of women who received peripartum VWF replacement in comparison to both women with untreated VWD (due to pregnancy-induced increase in plasma VWF) and controls, treated women had lower plasma VWF levels, significantly higher mean estimated blood loss (EBL) (615 vs. 448 mL; p < 0.05), and lower postpartum hematocrit.…”

Section: What Is the Optimal Plasma Vwf Target Peripartum?mentioning

confidence: 99%

“…PPH incidence in women with type 1 VWD is still higher than the general population and women should be counselled accordingly. 52 Second, the PPH rates described in women with VWD who require replacement therapy suggest that current prophylactic regimens may be inadequate. 52 The minimum plasma VWF threshold required at the time of delivery and in the postpartum period to minimize bleeding risk remains, as yet, unclear.…”

Section: What Is the Optimal Plasma Vwf Target Peripartum?mentioning

confidence: 99%

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

Byrne

Ryan

Lavin

2021

Semin Thromb Hemost

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For many women, pregnancy and childbirth represent their first major hemostatic challenges. Despite advancements in obstetric care, up to 2 to 5% of all deliveries are complicated by postpartum hemorrhage (PPH). To mitigate bleeding risk, physiological changes occur in pregnancy, including increases in plasma von Willebrand factor (VWF) and factor VIII levels. For women with von Willebrand disease (VWD), these physiological alterations are blunted or absent. As a result, women with VWD have a heightened risk of PPH, both primary (in the first 24 hours) and secondary (>24 hours to 6 to 12 weeks postpartum). Pregnancy and delivery management for women with VWD should therefore be carefully coordinated as part of a multidisciplinary team approach. In the absence of large-scale clinical trials, the management of women with VWD during pregnancy is guided by expert consensus guidelines. Clinical practices internationally are not uniform, and areas of considerable clinical uncertainty exist. Traditional peripartum plasma VWF thresholds for hemostatic cover and therapeutic targets are currently under scrutiny, as PPH is not eliminated in women with VWD who receive replacement therapy. The benefit and optimal duration of postpartum tranexamic acid have yet to be defined, and standardized methods of quantification of blood loss at the time of delivery are currently lacking. In this article, we review the evidence base to date and explore the current clinical challenges in the management of pregnant women with VWD.

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“…Several studies showed that in women with type 1 VWD, PPH still occurs frequently despite the increase and normalization of endogenous VWF and FVIII. 60 Several other groups have also reported high rates of PPH despite the use of concentrate to normalize FVIII and VWF levels. 36,61,62 In these latter studies, VWD women who were administered predelivery FVIII/VWF factor concentrates, based on low levels of VWF/FVIII in the third trimester, had more blood loss compared with women without VWD or untreated type 1 VWD women.…”

Section: Managing Delivery In Women With Vwdmentioning

confidence: 99%

“…36 These findings suggest that higher FVIII and VWF levels are needed to prevent bleeding in VWD women. 60 In The Netherlands, we therefore recently changed our guideline: we now advise treating VWD women at delivery with desmopressin or factor concentrate if VWF activity (VWF:RCo) levels are ,80 IU/dL in the third trimester and increasing the target peak level of VWF activity to at least 150 IU/dL. This management will be evaluated prospectively in the same study in hemophilia carriers (Prides study).…”

Section: Managing Delivery In Women With Vwdmentioning

confidence: 99%

How I manage pregnancy in carriers of hemophilia and patients with von Willebrand disease

Leebeek¹,

Duvekot

Kruip³

2020

Blood

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Women with inherited bleeding disorders, including carriers of hemophilia A and B, or with von Willebrand disease, have an increased risk of bleeding during pregnancy and delivery. Also the unborn child may be affected by the bleeding disorder for which specific measures have to be considered. This requires a multi-disciplinary approach, including a hematologist, pediatric hematologist, clinical geneticist, obstetrician-perinatologist, and anesthesiologist. An optimal approach includes pre-pregnancy genetic counselling, prenatal diagnostic procedures, and making a treatment plan for delivery for both the mother and child. Recent retrospective studies show that even if strict guidelines are followed, these women are still at risk of postpartum bleeding. This occurs even in case of normalization of coagulation factor levels, either due to the pregnancy-induced rise of factor levels or by infusion of coagulation factor concentrates at the time of delivery. In this article we describe our current diagnostic and clinical management of pregnancy and delivery in women with inherited bleeding disorders. We also briefly discuss possible interventions to improve the outcome of current strategies by increasing target factor levels during and after delivery.

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Maternal and neonatal bleeding complications in relation to peripartum management in women with Von Willebrand disease: A systematic review

Cited by 30 publications

References 100 publications

Clinical management of woman with bleeding disorders: A survey among European haemophilia treatment centres

Clinical management of woman with bleeding disorders: A survey among European haemophilia treatment centres

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

How I manage pregnancy in carriers of hemophilia and patients with von Willebrand disease

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