Advertisement
JOGC

Postpartum Hemorrhage in Women with von Willebrand Disease: Consider Other Etiologies

      Abstract

      Objective

      Higher rates of postpartum hemorrhage (PPH) have been reported for women with von Willebrand disease (VWD). Comprehensive multidisciplinary care reduces these rates; thus PPH may not be secondary to VWD.

      Methods

      We conducted a retrospective review for the period of 2009–2018, including all VWD pregnancies at 2 tertiary care academic hospitals to determine rates, etiology, and timing of PPH.

      Results

      A total of 63 women with 80 pregnancies were included. Three women had twin pregnancies. Sixty-six pregnancies (82.5%) involved type 1 VWD; 4 (5.0%), type 2 (unclear subtype); 3 (3.8%) type 2A; 3 (3.8%) type 2B; and 2 (2.5%), type 2M. Median age of patients was 32.9 years (range 19–43 y). Most patients were blood type O (65%), and 33 of 80 pregnancies (41.3%) were nulliparous. The mean bleeding assessment score was 8 (range 0–16). Thirty-seven pregnancies (46.3%) received prophylactic hemostatic treatment prior to delivery. Seventy-four percent of pregnancies were delivered vaginally, and 88% received epidural anaesthesia. The majority of pregnancies (78.8%) had von Willebrand factor (VWF) levels assessed during the third trimester, with most (71.3%) achieving VWF levels above 1.00 IU/mL. Four pregnancies (5.2%) were complicated by primary PPH; uterine atony in 2 and placenta previa in 1. Delayed postpartum bleeding occurred in 5 pregnancies (6.3%).

      Conclusion

      Multidisciplinary care of pregnancies with VWD improves outcomes. Rates of primary and delayed PPH in this study are lower than previously described and are similar to those of women without VWD. In women with VWD, uterine etiologies for primary PPH need to be considered, in a manner similar to the assessment of women without VWD, to ensure hemostasis is achieved.

      Résumé

      Objectif

      On rapporte une augmentation du taux d’hémorragie post-partum (HPP) chez les femmes atteintes de la maladie de von Willebrand (MVW). Comme les soins multidisciplinaires intégrés réduisent le taux d’incidence, l’HPP pourrait ne pas être secondaire à la MVW.

      Méthodologie

      Nous avons effectué un examen rétrospectif de toutes les grossesses avec MVW pour la période de 2009 à 2018 dans deux hôpitaux universitaires de soins tertiaires afin de déterminer le taux d’incidence, l’étiologie et le moment de l’HPP.

      Résultats

      L’étude inclut les données de 63 femmes, pour un total de 80 grossesses. Trois femmes ont eu une grossesse gémellaire. La distribution des grossesses selon le type de MVW va comme suit : 66 (82,5 %) de type 1; 4 (5,0 %) de type 2 (sous-type incertain); 3 (3,8 %) de type 2A; 3 (3,8 %) de type 2B; et 2 (2,5 %) de type 2M. L’âge médian des patientes était de 32,9 ans (intervalle : 19–43 ans). La plupart des patientes étaient du groupe sanguin O (65 %), et 33 des 80 grossesses (41,3 %) étaient chez des patientes nullipares. Le score moyen de l’évaluation des saignements était de 8 (plage : 0–16). Un traitement hémostatique a été donné en prophylaxie avant l’accouchement dans 37 grossesses (46,3 %). L’accouchement s’est fait par voie vaginale dans 74 % des grossesses, et 88 % ont impliqué une anesthésie péridurale. La majorité des grossesses (78,8 %) ont eu un dosage du facteur de von Willebrand (FVW) au troisième trimestre; la plupart (71,3 %) ont eu une concentration du FVW supérieure à 1,00 UI/ml. L’HPP primaire a été en cause dans 4 grossesses (5,2 %); l’atonie utérine, dans 2; et le placenta prævia, dans 1. Des saignements post-partum retardés se sont manifestés dans 5 grossesses (6,3 %).

      Conclusion

      La prise en charge multidisciplinaire des grossesses associées à la MVW améliore les issues. Les taux d’HPP primaire et retardée dans cette étude sont inférieurs à ceux décrits précédemment et sont comparables à ceux observés chez les femmes non atteintes de la MVW. Chez les femmes atteintes de la MVW, il faut envisager les étiologies utérines pour l’HPP primaire, de façon similaire à l’évaluation chez les femmes non atteintes, afin d’obtenir l’hémostase.

      Keywords

      To read this article in full you will need to make a payment

      Purchase one-time access:

      Academic & Personal: 24 hour online accessCorporate R&D Professionals: 24 hour online access
      One-time access price info
      • For academic or personal research use, select 'Academic and Personal'
      • For corporate R&D use, select 'Corporate R&D Professionals'

      Subscribe:

      Subscribe to Journal of Obstetrics and Gynaecology Canada
      Already a print subscriber? Claim online access
      Already an online subscriber? Sign in
      Institutional Access: Sign in to ScienceDirect

      References

        • Leebeek F.W.G.
        • Eikenboom J.C.J.
        Von Willebrand’s disease.
        N Eng J Med. 2016; 75: 2067-2080
        • Sadler J.E.
        • Budde U.
        • Eikenboom J.C.J.
        • et al.
        Update on the pathophysiology and classification of von Willebrand disease: a report of the subcommittee on von Willebrand factor.
        J Thromb Haemost. 2006; 10: 2103-2114
        • Nichols W.L.
        • Hultin M.B.
        • James A.H.
        • et al.
        von Willebrand disease (VWD): evidence-based diagnosis and management guidelines, the National Heart, Lung, and Blood Institute (NHLBI) expert panel report (USA).
        Haemophilia. 2008; 14: 171-232
        • Reynen E.
        • James P.
        von Willebrand disease and pregnancy: a review of evidence and expert opinion.
        Semin Thromb Hemost. 2016; 42: 717-723
        • Punt M.C.
        • Waning M.L.
        • Mauser-Bunschoten E.P.
        • et al.
        Maternal and neonatal bleeding complications in relation to peripartum management in women with von Willebrand: a systematic review.
        Blood Rev. 2019; 39: 100633
        • Castaman G.
        • James P.D.
        Pregnancy and delivery in women with von Willebrand disease.
        Eur J Haematol. 2019; 103: 73-79
      1. RCOG green top guideline no. 71 (joint with UKHCD). 2017 Management of inherited bleeding disorders in pregnancy. BJOG 124:e193–263.

        • James A.H.
        • Konkle B.A.
        • Kouides P.
        • et al.
        Postpartum von Willebrand factor levels in women with and without von Willebrand disease and implications for prophylaxis.
        Haemophilia. 2015; 1: 81-87
        • Bowman M.
        • Mundell G.
        • Grabell J.
        • et al.
        Generation and validation of the condensed MCMDM-1VWD bleeding questionnaire for von Willebrand disease.
        J Thromb Haemost. 2008; 12: 2062-2066
        • The American College of Obstetrics and Gynecology
        reVITALize: obstetrics data definitions.
        (Available at:)
        • Mehrabadi A.
        • Hutcheon J.A.
        • Lee L.
        • et al.
        Epidemiological investigation of a temporal increase in atonic postpartum haemorrhage: a population-based retrospective cohort study.
        BJOG. 2013; 120: 853-862
        • Stoof S.C.M.
        • van Steenbergen H.W.
        • Zwagemaker A.
        • et al.
        Primary postpartum haemorrhage in women with von Willebrand disease or carriership of haemophilia despite specialised care: a retrospective survey.
        Haemophilia. 2015; 21: 505-512
        • James A.H.
        • Konkle B.A.
        • Kouides P.
        • et al.
        Postpartum von Willebrand factor levels in women with and without von Willebrand disease and implications for prophylaxis.
        Haemophilia. 2015; 21: 81-87
        • Hawke L.
        • Grabell J.
        • Sim W.
        • et al.
        Obstetric bleeding among women with inherited bleeding disorders: a retrospective study.
        Haemophilia. 2016; 22: 906-911
        • Ducloy-Bouthors A.S.
        • Jude B.
        • Duhamel A.
        • et al.
        High-dose tranexamic acid reduces blood loss in postpartum haemorrhage.
        Crit Care. 2011; 15: R117
        • Desborough M.J.R.
        • Oakland K.A.
        • Landoni G.
        • et al.
        Desmopressin for treatment of platelet dysfunction and reversal of antiplatelet agents: a systematic review and meta-analysis of randomized controlled trials.
        J Thromb Haemost. 2017; 15: 263-272
        • Girolami A.
        • Tezza F.
        • Scapin M.
        • et al.
        Arterial and venous thrombosis in patients with von Willebrand’s disease: a critical review of the literature.
        J Thromb Thrombolysis. 2006; 21: 175-178
        • Karanth L.
        • Barua A.
        • Kanagasabai S.
        • et al.
        Desmopressin acetate (DDAVP) for preventing and treating acute bleeds during pregnancy in women with congenital bleeding disorders.
        Cochrane Database Syst Rev. 2019; (:CD009824.pub4)
        • O’Brien S.H.
        • Stanek J.R.
        • Kaur D.
        • et al.
        Laboratory monitoring during pregnancy and post-partum hemorrhage in women with von Willebrand disease.
        J Thromb Haemost. 2020; 3: 604-608