Single-Center Results of Endovascular Repair in Patients With Marfan Syndrome

Marfan syndrome (MFS) was the order of business on Thursday afternoon, with Ahmed Eleshra, MD, a vascular surgeon from Mansoura, Egypt, relaying single-center results of endovascular repair for this condition. We spoke with Dr. Eleshra, who gave us a glimpse into the data he presented.

Is it correct that for Marfan syndrome, aortic dilatation, rupture, and dissection have previously made endovascular repair questionable? What else should be highlighted?

Marfan syndrome (MFS) aortopathy is a complex clinical entity. There is a lack of high-quality evidence to support any particular approach to aortic repair in patients with connective tissue disorders, including MFS and a dearth of comparative data between open repair and endovascular repair. However, open repair is currently accepted as the standard approach to the treatment of aortic disease in patients with MFS as endovascular repair in these patients has been reported to have poor durability and high mortality, conversion rate, and reintervention rate.

Guidelines of involved societies confine endovascular therapy in MFS to patients with prohibitive open surgical risk, emergency cases, and as a bridge to definitive open repair. As patients with MFS nowadays frequently reach older ages and have a history of multiple previous aortic surgeries, the endovascular repair is used with an increasing frequency in specialized centers for those patients.

The current study reports our single-center experience over a 10-year period regarding endovascular treatment of aortic pathologies in patients with MFS.

Given that Marfan patients are typically younger, is durability of repair also an issue?

Open surgical repair of aortic root aneurysm is well established and has excellent long-term results. A subset of patients with MFS presents with a thoracoabdominal aortic aneurysm (TAAA) either as a long-term sequelae of aortic dissection (AD) or as a degenerative aneurysm.

Freedom from reintervention was acceptable in the current study with 77% at 12 months. The continued degeneration of the aorta at the level of the aortic landings zones remains a concern and potential cause for reinterventions (freedom from reintervention related to disease progression in 84% at 12 months).

The study had improved outcome compared to the previous report. This could probably be explained by the relatively younger age of cohort; lower number of acute dissection cases with its impact on high retrograde dissection, endoleak, and reintervention; and the use of dedicated device with lower radial force in the distal graft end and probably by different amount in oversizing or greater percentage of landing within synthetic grafts.

Can you introduce the aims and design of the single-center work you are presenting?

The objectives were to study the outcome of endovascular treatment of aortic pathologies in patients with MFS at a single institution. To do this, consecutive MFS patients who underwent endovascular repair of hybrid procedures for aortic pathologies from January 2010 to May 2020 were identified. Several endovascular and hybrid strategies have been used. Technical success, short- and mid-term survival, complications, and reinterventions were retrospectively analyzed.

What were the key results?

During the study period, 24 patients with MFS (median age, 48 [13-78] years; 58% males) were treated. Indications for intervention were chronic aortic dissection with aneurysm degeneration in 16 patients (67%), acute type B aortic dissection in 4 patients (17%), aortic aneurysm without any dissection in 3 patients (13%), and aortic intramural hematoma in 1 patient (4%). Most patients were asymptomatic (83%), 3 (13%) were symptomatic and 1 (4%) had a contained rupture. The median aneurysm diameter was 56 (35-86) mm. Hybrid procedures were performed in 7 (29%) patients. Thoracic endovascular repair was performed in 12 (50%) patients, a fenestrated or branched endovascular aortic repair in 4 (17%) patients, and placement of an iliac artery stent-graft in 1 (4%) patient. Procedures were staged in 12 (50%) patients. Technical success was achieved in all patients. The median intensive care unit stay was 6 (range, 1-30) days, and the median hospital stay was 23 (range, 3-112) days. Early mortality was reported in 1 (4%) patient. Wound infection was seen in 7 b(29%) patients and gastrointestinal complications in 3 (13%) patients. The median follow-up was 42 (range, 1-127) months. The cumulative survival rate was 87% at 24 months. The cumulative freedom from reintervention was 77% at 12 months.

What are the implications of these new data? 

This highly selected cohort supports the use of endovascular and hybrid aortic repair for aortic pathologies in high-risk patients with MFS. Endovascular and hybrid repair for aortic pathologies in patients with MFS appears feasible with acceptable early and late outcomes.