Cautious Hope for Endovascular Aortic Tx in Connective Tissue Disease

Endovascular aortic repair earned more confidence for patients with dissections and aneurysms associated with hereditary connective tissue disease (CTD), as selected patients showed acceptable short-term and midterm survival in the real world.

Retrospective reports from major aortic centers showed a 30-day mortality rate of 2.9% among consecutive patients with CTD who had undergone endovascular aortic repair in 2005-2020, according to a group led by Karl Wilhelm Olsson, MD, PhD, of Uppsala University in Sweden.

Their 171-person EVICTUS cohort showed that survival at 1 year and 5 years differed by specific CTD:

• Marfan syndrome (n=142): 96.2% and 80.6%, respectively

• Loeys-Dietz syndrome (n=17): 93.8% and 85.2%

• Vascular Ehlers-Danlos syndrome (vEDS; n=12): 75.0% and 43.8%

"The perioperative mortality and morbidity were acceptable for patients with Marfan syndrome or Loeys-Dietz syndrome, especially given that compared with the reports of open surgery, this cohort featured patients with an older median age and more patients emergently treated for complicated dissections, as well as symptomatic or ruptured aneurysms," Olsson's group reported in JAMA Surgery.

On the other hand, the researchers acknowledged, the results for vEDS reaffirm "that patients with vEDS have a dismal prognosis and any surgical intervention (open or endovascular) is especially hazardous."

Traditionally, any hereditary CTD is a contraindication to endovascular aortic interventions. Individuals with these relatively rare genetic disorders have diverse defects in the proteins that support organs and organ systems, including the body's major vessels. As such, their aortic tissues are expected to be fragile and unlikely to hold a stent in place.

In the present report, patients largely had endovascular aortic repair as a redo or secondary procedure, with a minority undergoing it as an emergent index procedure or because they were not candidates for open surgery, according to Olsson and colleagues.

The investigators reported that 53.2% of the CTD cohort had undergone secondary procedures over a median 4.7 years -- a relatively high percentage. However, just 8.2% of those were conversions to open repair. "These observations imply that neither open nor endovascular procedures in patients with CTD should be regarded as a single curative treatment, but rather as part of a continuous strategy of surveillance and repeated treatments as indicated," the team suggested.

"EVICTUS has provided us a glimpse of this future, challenged guideline recommendations, and provided the initial, larger complex endovascular experience to enlighten our debates. EVICTUS breaks our professional rules, informing our art," wrote W. Darrin Clouse, MD, of the University of Virginia in Charlottesville, in an accompanying editorial.

"It will be fascinating to observe CTD approaches as newer devices are created and surgeons trained," Clouse said. "Will technology overcome the device-aortic tissue interface? How comfortable will surgeons be, and where will open surgery be performed?"

EVICTUS was a collaboration between 18 aortic centers across several continents. The 171 participants with CTD had a median age of 49.9 years, and 62.6% were men.

Endovascular aortic repair was performed in 40.9% for complicated aortic dissections, 48.0% for uncomplicated dissections, and 11.1% for degenerative aneurysms. Nearly 80% of the group had already undergone open aortic surgery before endovascular repair.

Olsson's group noted that primary technical success was achieved in 98.2%, defined as the successful introduction and deployment of the device in the absence of surgical conversion to open repair, death in 24 hours or less, type 1 or 3 endoleaks as evidenced by procedural angiography, or graft obstruction.

"It is noteworthy that the cohort represented only 16% of CTD aortic reconstructions at these centers. Selection bias is obvious and not uniform among centers," Clouse warned.

Yet any rigorous comparison between endovascular repair and surgery would require a randomized trial that is "difficult to accomplish" given the rarity and heterogeneity of CTDs with aortic involvement, the study authors said.

For now, long-term follow-up of the EVICTUS group is being planned, the researchers said.

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