Andrea Lobo, PhD, science writer —

Women with Ehlers-Danlos syndrome (EDS) are at a higher risk for complications during pregnancy than other women, including poorer fetal growth, preterm delivery, and postpartum bleeding, a study using a large U.S. database reports. Likewise, these patients were more likely to deliver via cesarean section. But a number of other pregnancy-related complications, including diabetes and hypertension, were common between these two groups, while pregnancy in patients tended to be at more advanced ages. “Knowledge about obstetrical and fetal complications in patients with EDS is important at improving their overall care before and during each pregnancy,” the researchers wrote. Pregnancy complications reported to be higher than average with EDS. The study, “Pregnancy and Fetal Outcomes in Patients with Ehlers-Danlos Syndrome: A Nationally Representative Analysis,” was published in the journal Current Problems in Cardiology. EDS is a group of genetic disorders affecting the connective tissues that provide structure to joints, skin, blood vessels, and other tissues and organs. Many EDS-causing mutations affect the production and function of collagen, a main component of connective tissue. The 13 types of EDS have a broad range of symptoms, and cardiovascular complications can be frequent. Vascular EDS, generally considered the disease's most severe form, is associated with aneurysms — a weakened area in the blood vessel wall, making it particularly prone to rupture — which can be life-threatening. While some studies indicate that pregnancy in EDS patients carries a higher risk of complications, results to date are not consistent and these studies tend to involve small groups. Researchers conducted a retrospective study using the Health Care Utilization Project-Nationwide Inpatient Sample — the largest publicly available inpatient care database in the U.S. — to understand pregnancy and fetal outcomes in EDS patients. The study covered 1,016 births to women with EDS, out of a total of 5.89 million births recorded between January 2016 and December 2019. Compared to the general population, pregnant women with EDS were significantly more likely to be older than 25 (58.9% vs. 28.2%), and older than 35, classified as at advanced maternal age (16.7% vs. 9%), to be white (90% vs. 52%), and to have multiple gestations (like twins or triplets; 1.57% vs. 0.87%). They were also more likely to belong to higher income groups. The proportion of patients of advanced maternal age “may be related to the more extensive health-related issues in the EDS group and the higher infertility rates leading to a generally delayed age of conception,” the researchers wrote. “This is further supported as the EDS group was more likely to have multiple gestations which could relate to the use of infertility therapies.” Moreover, they were significantly more likely to smoke (14.76% vs. 5.08%), and have hypertension or chronic high blood pressure (5.31% vs. 1.9%), as well as mitral valve prolapse — a type of heart disease affecting the valve between the left upper and lower chambers, 1.18% vs 0.04%. When the analysis was adjusted for age and race, these patients also were almost twice as likely (1.77 times) to show restricted fetal growth, meaning of a child of lower weight and size at birth, and 2.59 times more likely than other women to deliver their baby preterm. Likewise, they were 1.35 times more likely to deliver via cesarean section and more than twice as likely to have postpartum bleeding (2.13 times). "Elective c-section at 32 weeks has been recommended in previous studies for patients with [vascular] EDS to avoid fatal vascular complications of pregnancy, and patients in our study were more likely to undergo a c-section," the researchers wrote. Stillbirths and some common complications not found to at higher rates. Hospital stays for delivery also were also longer among women with EDS, by either three or seven days, possibly for continued surveillance or management of complications. Other pregnancy complications, however, were not more likely in EDS patients than in the general population. Such complications noted in the study were gestational diabetes and hypertension; placenta previa, when the placenta completely or partly covers the opening of the uterus; premature membrane or amniotic sac rupture; sepsis, a potentially life-threatening result of infection; and stillbirth. No cases of an aneurysm rupture in the aorta — the main artery that carries blood away from the heart — or in-hospital mortality among EDS patients were reported. “Further studies regarding each type of EDS and its obstetric complications may aid in pre-pregnancy counseling, [pregnancy] care, and formulating a multidisciplinary obstetric approach for this patient population,” the researchers concluded.

Andrea Lobo, PhD, science writer —

Articles by Andrea Lobo

Preterm delivery, bleeding among pregnancy risks with EDS

Rare Disease Day 2023

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