Maternal Complications of Ehlers-Danlos Syndrome in Pregnancy

Critical Distinction: Vascular EDS (Type IV) vs. Other Subtypes

• The American Heart Association suggests that pregnancy complications in EDS vary dramatically by subtype, with vascular EDS (Type IV) carrying catastrophic maternal mortality risk from arterial and uterine rupture, while hypermobile and classical EDS are generally well-tolerated with primarily musculoskeletal and wound-healing complications 1
• Genetic testing should be obtained immediately if not already done, as 26.4% of clinically diagnosed EDS cases have alternative genetic conditions requiring different management, according to the American College of Medical Genetics 2, 3

Vascular EDS (Type IV) - High Maternal Mortality Risk

• The outcome of pregnancy in women with vascular EDS is poor, with maternal death occurring from rupture of the gravid uterus and vessel rupture at delivery or in the postpartum period, with a high strength of evidence 1
• Specific life-threatening complications include arterial rupture and dissection, with median survival of only 48 years in vascular EDS patients generally, and uterine rupture during pregnancy or delivery 1, 2, 3
• Vessel rupture during delivery or postpartum period is also a significant risk 1, 2

Management Implications by Subtype

For Confirmed or Suspected Vascular EDS:

• Baseline vascular imaging from head to pelvis should be performed to identify pre-existing aneurysms or dissections that could rupture peripartum, as recommended by the Society for Vascular Surgery 2
• Planned cesarean delivery at 34-38 weeks is recommended to avoid spontaneous labor and minimize uterine rupture risk, though cesarean itself carries surgical risks, according to the American College of Obstetricians and Gynecologists 2
• Invasive hemodynamic monitoring must be avoided due to risk of fatal vascular complications, with a high strength of evidence 1, 2, 3

For Hypermobile and Classical EDS:

• Active management of third stage labor with uterotonics should be implemented immediately after delivery to prevent postpartum hemorrhage, as recommended by the Royal College of Obstetricians and Gynaecologists 2
• Episiotomy should be avoided unless absolutely necessary due to excessive bleeding risk from tissue fragility, with a moderate strength of evidence 2
• Meticulous surgical technique with careful tissue handling is essential, using pledgeted sutures for anastomoses, according to the American College of Surgeons 1
• Patients should be monitored closely for delayed postpartum hemorrhage due to tissue fragility and delayed wound healing, with a moderate strength of evidence 2

Common Pitfalls to Avoid

• Never perform invasive vascular procedures or monitoring in confirmed vascular EDS, as fatal complications have been associated with invasive imaging and catheterization in these patients, with a high strength of evidence 1, 2, 3
• Do not assume clinical diagnosis is accurate - genetic testing is essential as clinical overlap exists between EDS subtypes and other connective tissue disorders, according to the American College of Medical Genetics 2, 3