title: Postpartum Hemorrhage in Women with Von Willebrand Disease - A Retrospective Observational Study.
abstract: von Willebrand disease (VWD) is a hereditary bleeding disorder, caused by a deficiency in the levels and/or function of von Willebrand factor (VWF). Women with VWD appear to be at increased risk of experiencing postpartum hemorrhage (PPH), though the levels of VWF increase during pregnancy. There is limited knowledge of how PPH is associated with the subtype of VWD, plasma levels of other coagulations factors than VWF and given hemostatic treatment.
  
  
  
  The aims were to investigate the incidence of PPH in women with VWD and to analyse the correlation between PPH and: (1) type of VWD, (2) laboratory monitoring of VWF and FVIII and (3) hemostatic drug treatment.
  
  
  
  This was a retrospective observational study. The study participants (n = 34) were recruited from the Coagulation Unit, Karolinska University hospital. Fifty-nine deliveries, which occurred in 14 different obstetrics units (years 1995-2012) were included in the study.
  
  
  
  The incidence of primary PPH was 44%, severe primary PPH 20% and secondary PPH 12%. VWD type 3 was associated with a higher risk of experiencing severe primary PPH compared to other subtypes. FVIII:C in pregnancy was inversely correlated to blood loss during delivery. There was a significantly higher incidence of secondary PPH when the VWD diagnosis was unknown at time of delivery.
  
  
  
  The women with VWD are at higher risk of PPH, especially those with type 3 VWD or when diagnosis is unknown prior to delivery. Identification of pregnant women with undiagnosed VWD may be of importance in order to prevent PPH.
authors: Department of Women's and Children's Health, Karolinska Institutet, Karolinska University Hospital, Solna, 171 76, Stockholm, Sweden.
authors: Igor
authors: 0000-0003-1809-0270
authors: I
authors: Govorov
authors: Department of Women's and Children's Health, Karolinska Institutet, Karolinska University Hospital, Solna, 171 76, Stockholm, Sweden.
authors: Signe
authors: S
authors: Löfgren
authors: Department of Molecular Medicine and Surgery, Karolinska Institutet, Solna, 171 76, Stockholm, Sweden.
authors: Roza
authors: R
authors: Chaireti
authors: Department of Medicine, Karolinska University Hospital Solna, 171 76, Stockholm, Sweden.
authors: Margareta
authors: M
authors: Holmström
authors: Department of Women's and Children's Health, Karolinska Institutet, Karolinska University Hospital, Solna, 171 76, Stockholm, Sweden.
authors: Katarina
authors: K
authors: Bremme
authors: Department of Women's and Children's Health, Karolinska Institutet, Karolinska University Hospital, Solna, 171 76, Stockholm, Sweden.
authors: Miriam
authors: M
authors: Mints
mesh_terms: D005169:Factor VIII / Q000032:analysis*; D005260:Female; D006490:Hemostatics / Q000627:therapeutic use; D006801:Humans; D006473:Postpartum Hemorrhage / Q000188:drug therapy / Q000453:epidemiology*; D011247:Pregnancy; D012189:Retrospective Studies; D013548:Sweden; D014842:von Willebrand Diseases / Q000150:complications / Q000378:metabolism*; D014841:von Willebrand Factor / Q000032:analysis*
chemical_list: D006490:Hemostatics; D014841:von Willebrand Factor; D005169:Factor VIII