title: Pregnancy-related deaths and complications in women with vascular Ehlers-Danlos syndrome.
abstract: OBJECTIVE
  The purpose of this study was to characterize the nature and magnitude of pregnancy risks in women with vascular Ehlers-Danlos syndrome.
  
  
  METHODS
  Pregnancy-related death rate was determined by a review of pedigrees of families with vascular Ehlers-Danlos syndrome. Maternal morbidity was characterized through semistructured interviews with women with vascular Ehlers-Danlos syndrome or their next of kin.
  
  
  RESULTS
  Pregnancy-related deaths occurred in 30 of 565 deliveries (5.3%). There was no difference in Kaplan-Meier survival curves between parous versus nulliparous women with vascular Ehlers-Danlos syndrome. Interviews with 39 women indicated that 46% of deliveries were uncomplicated. The most common pregnancy-related complications were third-/fourth-degree lacerations (20%) and preterm delivery (19%). Life-threatening complications occurred in 14.5% of deliveries and included arterial dissection/rupture (9.2%), uterine rupture (2.6%), and surgical complications (2.6%). There were 5 maternal deaths in 76 deliveries (6.5%).
  
  
  CONCLUSIONS
  The risk of pregnancy-related complications is increased in women with vascular Ehlers-Danlos syndrome compared with the general population; however, survival data indicate that pregnancy does not appear to affect overall mortality compared with nulliparous women with vascular Ehlers-Danlos syndrome. The data were insufficient to determine whether mode or timing of delivery influenced risk of complications. Women with vascular Ehlers-Danlos syndrome should be engaged in a shared decision-making process when contemplating pregnancy and pregnancy management.
authors.affiliation: 1] Department of Pathology, University of Washington, Seattle, Washington, USA [2] Department of Medicine (Medical Genetics), University of Washington, Seattle, Washington, USA.
authors.forename: Mitzi L
authors.initials: ML
authors.lastname: Murray
authors.affiliation: Department of Pathology, University of Washington, Seattle, Washington, USA.
authors.forename: Melanie
authors.initials: M
authors.lastname: Pepin
authors.affiliation: Maternal and Fetal Specialty Center, Swedish Medical Center, Seattle, Washington, USA.
authors.forename: Suzanne
authors.initials: S
authors.lastname: Peterson
authors.affiliation: 1] Department of Pathology, University of Washington, Seattle, Washington, USA [2] Department of Medicine (Medical Genetics), University of Washington, Seattle, Washington, USA.
authors.forename: Peter H
authors.initials: PH
authors.lastname: Byers
mesh_terms: D000293:Adolescent; D000328:Adult; D017677:Age Distribution; D000368:Aged; D000369:Aged, 80 and over; D000784:Aortic Dissection / Q000150:complications; D004535:Ehlers-Danlos Syndrome / Q000150:complications* / Q000235:genetics / Q000401:mortality*; D005260:Female; D006801:Humans; D053208:Kaplan-Meier Estimate; D063130:Maternal Death; D008875:Middle Aged; D009154:Mutation; D010298:Parity; D010375:Pedigree; D011247:Pregnancy; D011248:Pregnancy Complications / Q000401:mortality*; D012189:Retrospective Studies; D012307:Risk Factors; D012422:Rupture, Spontaneous; D016896:Treatment Outcome; D014597:Uterine Rupture; D055815:Young Adult