Pregnancy-related deaths and complications in women with vascular Ehlers–Danlos syndrome

Purpose: 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%). Conclusion: 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. Genet Med 16 12, 874–880.