Optimizing Aortic Endovascular Repair in Patients with Marfan Syndrome

Abstract

Introduction: The reported experience in endovascular aortic repair for Marfan syndrome (MFS) is limited, and results are heterogeneous. In this population, thoracic stent-graft placement carries a risk of aortic wall aggression, which may result in retrograde aortic dissection, dilatation, or false aneurysms at the landing zone. We hypothesized that these complications may be potentially avoided using modified practices. This study aimed to describe our experience with the use of a specific protocol of endovascular aortic repair in patients with MFS Methods: All MFS patients with FBN-1 gene mutation who were treated with aortic stent-graft placement between February 2015 and August 2018 were included. The following rules were applied: 1) excluding bare stents and barbs in the choice of a stent-graft, 2) proximal landing in a preexisting graft, or 3) minimization of proximal oversizing in the case of proximal landing in the native aorta (< 10%), and 4) distal undersizing Results: Eighteen patients were included (55% men, mean age: 47 ± 17 years). The index procedure was initial endovascular aortic repair in 10 cases, elephant trunk completion in 6, and anastomotic pseudo-aneurysm after thoracic open repair in 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. No mortality, spinal cord ischemia, stroke, and retrograde aortic dissection occurred during the postoperative course. One type 1b endoleak was observed. The mean follow-up period was 21.4 months. The aortic aneurysm-related mortality was 5.5% (n = 1) and occurred after planned open thoracoabdominal surgery. One patient presented a proximal landing zone complication (5.5%) with aortic enlargement requiring arch repair. The maximal aortic diameter significantly decreased from 59 mm to 45 mm (p < 0.0001) after treatment. Conclusion: The specific protocol described in this study seems to optimize the results of endovascular aortic repair in MFS patients with significant aortic remodeling Disclosure: Nothing to disclose