Aorta Surgery Has Low Risk of Mortality for EDS Patients: Study

Emergency hospital admissions were linked to a greater mortality risk

Lindsey Shapiro, PhD avatar

by Lindsey Shapiro, PhD |

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Mortality rates following surgery for certain complications of the aorta — the main artery that carries blood away from the heart — are low among people with Ehlers-Danlos syndrome (EDS), a study has found.

But non-elective or emergency hospital admissions were linked to a greater mortality risk, data show. The finding emphasizes that “importance should be placed on increased surveillance to reduce nonelective admissions,” researchers noted.

The study, “National trends in thoracic aortic aneurysms and dissections in patients with Marfans and Ehlers Danlos syndrome,” was published in the Journal of Cardiac Surgery

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Problems relating to the aorta — the largest artery in the body that carries oxygenated blood to the rest of the organs — are associated with a high risk of complications in the general population. Aortic aneurysm describes a weakened area in the aorta’s wall, making it particularly prone to rupture or dissection. An acute aortic dissection refers to a tear in the inner layer of the aorta, which can cause rupture and reduce blood flow to the rest of the body.

Because people with connective tissue disorders like EDS and Marfan syndrome have defects in proteins that provide structure and support to the body’s tissues, their aortic walls may be less able to resist these types of injury. Consequently, data have shown that these patients are predisposed to developing aortic complications earlier in life than the general population.

But because the diseases are rare, little is known about risk factors and outcomes related to aneurysm or dissection among EDS and Marfan patients.

In the study, the researchers made use of the National Inpatient Sample database, which contains data from more than seven million hospital stays in the U.S. each year, to learn more about the relationship between aortic complications and these connective tissue disorders.

The researchers found 52,753 records of EDS and 44,666 of Marfan between 2010 and 2017 in the database. These numbers equated to an estimated incidence of EDS in the U.S. of 22.4 per 100,000 people, a number that has been slightly increasing in recent years, the team noted. The incidence of Marfan was 18 per 100,000, which has remained steady.

Of the patients diagnosed with EDS, 180 also had a dissection or aneurysm that was surgically treated during a hospital stay. The same was true of 2,553 Marfan patients.

The relatively higher number of admissions among Marfan patients might imply “a heightened risk for aortic pathology,” the researchers wrote. “Alternatively, this may be a consequence of historic diagnostic capabilities and awareness of [Marfan syndrome] over EDS.”

EDS patients were significantly older and more likely female than in the Marfan group, and were also more likely to have diabetes, high blood pressure, and altered fat levels in the blood.

About half of the patients in each group also underwent valve surgery to replace or repair one of the heart’s four valves. Some patients with Marfan (249) also underwent coronary artery bypass grafting to restore blood flow to the heart, but no EDS patients underwent that procedure.

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Mortality rates nearly five times higher for dissection than aneurysm

In-hospital mortality rates were low in both groups — 2.8% in EDS and 4.6% for Marfan. Mortality rates were almost five times higher among patients who had a dissection compared with those who had an aneurysm.

These mortality rates compare favorably with recent studies among the general population, the researchers noted. “Operative management of aortic dissections and aneurysms have been improving over the last decade, in particular for patients with connective tissue diseases,” they wrote.

The most frequently observed complications among EDS patients were coagulation (blood clotting) disorders (50%), irregular heartbeat (30.6%), and heart attack (22.2%).

In both groups, the median duration of hospital stay was eight days.

In a statistical analysis, having a dissection was a significant predictor of mortality. In contrast, an elective, or planned, hospital admission was protective for survival relative to non-elective, or unplanned (i.e. emergency) hospitalizations.

The team performed a subgroup analysis of the 38.9% of EDS patients and 38.2% of Marfan patients who had non-electively been admitted. Among this group, mortality rates were higher than those seen in the overall population — 7.1% for EDS and 7.8% for Marfan patients.

“The difference in mortality between elective and nonelective admissions emphasizes the importance of medical management and close follow‐up for better risk stratification and earlier intervention,” the researchers wrote.

The database did not record EDS subtypes. Since patients with some EDS types may be particularly susceptible to heart complications, this marks a study limitation, the team noted.