Endovascular Aortic Repair Aids Some With Marfan


Major Finding: In all, 7 of 16 patients with Marfan syndrome had successful endovascular treatment of their aortas.

Data Source: Review of endovascular repairs at the University of Florida, Gainesville, from 2000 to 2010.

Disclosures: Dr. Waterman and Dr. Endean said they had no relevant disclosures.

NAPLES, FLA. — Some patients with Marfan syndrome benefit from endovascular aortic repair, according to a review of 16 patients who were treated at the University of Florida, Gainesville, between January 2000 and June 2010.

Most patients had a history of multiple interventions, which points to the complexity of Marfan syndrome and a need to follow patients closely over the long term, Dr. Alyson L. Waterman said at the meeting.

Dr. Waterman and her associates reviewed their experience with endovascular abdominal aorta repair (EVAR) and thoracic endovascular aorta repair (TEVAR) for Marfan syndrome patients at the University of Florida.

After a median of 8.3 months, seven patients had clinical and radiographic evidence of successful treatment. Another six patients were primary treatment failures (for example, they had a type I endovascular leak or had persistent false lumen flow), and two patients had a secondary treatment failure, that is, initial success followed by proximal or distal aortic failure unrelated to the original intervention site. The remaining patient was lost to follow-up.

“Results are sobering but support [the concept that] some Marfan syndrome patients benefit from endovascular therapy,” said Dr. Waterman of the department of surgery at the University of Florida, Gainesville.

The main causes of death in Marfan syndrome are aortic dissection and rupture, Dr. Waterman said. She pointed out that open surgery is still required for repair of the ascending aorta, but endovascular intervention is an option for descending thoracic and abdominal aorta repair for those with this connective-tissue disorder.

The 16 Marfan syndrome patients in the series underwent a total of 19 relevant procedures (1 EVAR, 15 TEVAR, and 3 combined procedures). The median patient age was 52 years. Three patients had a secondary TEVAR within 28 months of their index intervention.

In all, 15 of the patients had previous surgery (from 17 years to 1 week prior) of the ascending aorta or arch.

Chronic dissection and/or aneurysmal dilation of the descending aorta were the indications for elective intervention in 13 patients. Two acute dissection/malperfusion cases and one anastomotic disruption early after an open surgery led to emergency intervention in three patients.

Four of the TEVAR procedures required adjunctive endovascular procedures. They comprised one subclavian artery embolization, one vertebral artery stent, one renal artery stent, and one celiac and superior mesenteric artery stent.

All four EVAR procedures required complex adjunctive endovascular procedures involving visceral arteries.

Five patients died during follow-up. Of them, two died perioperatively: the patient who underwent emergent TEVAR for anastomotic disruption, and a patient who required multivisceral revascularization in conjunction with a second TEVAR. Two patients died following discharge: one because of a respiratory failure at 3 months, and the other because of a cardiac arrest after 4 months.

The remaining patient died more than 6 years after EVAR from advanced age (84 years).

“This is clearly a group for whom treatment options are difficult at best,” said study discussant Dr. Eric D. Endean of the University of Kentucky, Lexington.

All were deemed poor candidates for open surgery, and they had a history of an average of almost two previous aortic operations. Six (38%) were classified as primary treatment failures, all required open repair, and half died, Dr. Endean commented. “Results are indeed sobering.”

“As you point out, diagnosis of Marfan syndrome was based on clinical diagnosis,” Dr. Endean said. He asked how confident Dr. Waterman is about the diagnoses in her series.

“That is one of the difficulties with Marfan syndrome; a lot is based on clinical diagnosis alone.” Not all patients undergo genetic screening for the fibrillin-1 mutation, she said.

A small population that makes the study “essentially a case series” is a limitation, Dr. Waterman said.

“Open surgery still has a big role in replacement of these aortas because these aortas are not genetically normal,” Dr. Waterman said.

However, there is still a place for endovascular therapy and a need to identify which patients are likely to benefit, she explained.

Dr. Waterman added that the cardiologists at University of Florida also treat Marfan syndrome patients, and they have yet to look at these patient populations at the two institutions combined.

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