vEDS not linked to increased risk of life-threatening events in pregnancy
Guidelines to discourage pregnancy in vEDS patients ‘might be too strict’: Study
Vascular Ehlers-Danlos syndrome (vEDS) does not appear to increase the risk of life-threatening complications during pregnancy and delivery, according to a study in the Netherlands that looked at data from 121 pregnancies.
However, about 1 in 5 of those pregnancies ended in miscarriage, a rate about twice as high as in the general Dutch population. Miscarriage was even more frequent in women with a specific disease-causing genetic mutation, and occurred in about 4 out of 5 pregnancies.
Most women had a vaginal birth, with some experiencing complications like heavy bleeding after delivery. No pregnancy-related deaths or life-threatening events, such as arterial dissections (tears in the inner layer of arteries) or uterine ruptures, were reported.
The study’s findings suggest doctors and patients should discuss risks together to make the best decision for each pregnancy.
‘Current advice to discourage pregnancy in all women with vEDS’
“The current advice to discourage pregnancy in all women with vEDS might be too strict,” researchers wrote. However, “given the high risks for pregnancy-related complications in women with vEDS, it is important to create an international consensus statement.”
The study, “Pregnancy and Delivery Outcomes in Vascular Ehlers–Danlos Syndrome: A Retrospective Multicentre Cohort Study,” was published in BJOG: An International Journal of Obstetrics & Gynaecology. It was conducted by a team of researchers in the Netherlands.
EDS is a group of genetic diseases that affect the connective tissues providing structure to the body. While EDS most often causes overly flexible joints and thin skin that tears easily, the various types of EDS manifest in different ways, and symptoms vary greatly in severity.
In addition to overly flexible joints and thin skin, people with vEDS have fragile muscles, internal organs, and blood vessels that are prone to rupturing or dissecting. The disease is often caused by mutations in COL3A1, a gene that codes for part of a protein that holds tissues together.
While women with EDS are at a higher risk of complications during pregnancy and delivery than other women, there is limited information about vEDS, with some being contradictory in terms of adverse outcomes. As a result, recommendations for pregnancy vary greatly, from ending a pregnancy early to scheduling a C-section to making a shared decision on the best approach.
“Because of the scant information about pregnancy-related complications in women with vEDS, reliable evidence-based guidelines for pregnancy and delivery management in vEDS are lacking,” the researchers wrote.
Study looked at medical records of 43 women with vEDS
To better understand the risks associated with pregnancy and delivery in vEDS, the researchers looked at the medical records of 43 women with the condition who had a total of 121 pregnancies (median of three per women).
Seven women had a diagnosis of vEDS before getting pregnant, six before conception, and one in the first eight weeks of pregnancy. Genetic testing identified COL3A1 mutations that were known or likely to cause the disease in all the women.
About 19% of the pregnancies ended in miscarriage, which was significantly more common in women with a specific genetic mutation known as a glycine substitution in the COL3A1 gene (82.6%). Also, three pregnancies (2.5%) ended in stillbirth and 19.1% of the babies were born prematurely (before 37 weeks of gestation).
Most women (79.1%) had a vaginal birth, and complications during delivery, including severe perineal tearing or heavy bleeding, were reported in 14.9% of pregnancies.
“Miscarriage, spontaneous preterm births, [preterm prelabor rupture of membranes] and postpartum [bleeding] occurred twice as often compared to the general population,” the researchers wrote, adding that “severe perineal tears occurred 10 times more often.”
However, “no uterine ruptures, vascular complications or pregnancy-related deaths were reported,” they added.
“In our opinion, women with vEDS and the desire to have a child should be invited for an individualised shared decision-making process about the reproductive possibilities in dedicated centres,” the researchers concluded.
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