Optimising Aortic Endovascular Repair in Patients with Marfan Syndrome

Objective: In Marfan syndrome (MFS) patients, endovascular repair carries a risk of aortic wall injury, which may result in retrograde aortic dissection, dilatation, or false aneurysm at the landing zones. It was hypothesised that potentially these complications may be avoided using modified practices. This study aimed to describe experience of a specific protocol for endovascular aortic repair in patients with MFS.

Methods: All MFS patients treated by aortic endovascular repair between February 2015 and August 2018 were included prospectively. The following rules were applied: (i) excluding stent grafts with bare stents and barbs, (ii) proximal landing in a pre-existing graft, or (iii) minimising proximal oversizing when landing in the proximal native aorta (<10%), and (iv) distal undersizing for chronic dissection cases.

Results: In eighteen patients (55% men, mean age: 47 ± 17 years), the index procedures were initial endovascular aortic repair (n = 10), elephant trunk completion (n = 6), and anastomotic pseudo-aneurysm after thoracic open repair (n = 2). The technical success rate was 100%. Proximal landing was in the native aorta in 11 patients (61%), with a mean proximal oversizing of 2.4 mm (8% oversized). Distal landing in the native aorta was performed in 16 cases (89%), with a mean distal undersizing of 8.9 mm (- 23%). No mortality, spinal cord ischaemia, stroke, or retrograde aortic dissection occurred post-operatively. One type 1b endoleak was observed. The mean follow up was 21.4 months. Aortic aneurysm related mortality was 5% (n = 1) and occurred after distal thoraco-abdominal surgery planned from the outset (prior to endovascular repair). Another patient presented a proximal landing zone complication with aortic enlargement. The mean maximum aortic diameter decreased significantly from 59 mm to 45 mm (p = .0005) after treatment.

Conclusion: The specific protocol described in this study seems to optimise the results of endovascular aortic repair in MFS patients with significant aortic remodelling.