Optimising Aortic Endovascular Repair in Patients with Marfan Syndrome

Abstract

WHAT THIS PAPER ADDSThe described protocol of endovascular aortic repair in patients with Marfan syndrome seems promising because it leads to significant aortic remodelling while the rate of landing zone complication remains low. The article emphasizes the benefit of integrating endovascular aortic repair in a multistage aortic repair strategy, which may lead to total aortic replacement eventually.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 AE 17 years), the index procedures were initial endovascular aortic repair (n ¼ 10), elephant trunk completion (n ¼ 6), and anastomotic pseudo-aneurysm after thoracic open repair (n ¼ 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 (e 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 ¼ 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 ¼ .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.

One-Year Single-Center Results of the Multilayer Flow Modulator Stents for the Treatment of Type B Aortic Dissection

“…The latest advances in off-the-shelf branched stent-grafts are promising; however, a large variety of costly off-the-shelf stent-grafts is necessary to treat aortic dissections in up to 30% of distinctive anatomical variations. 57,58 In patients with Marfan syndrome, endovascular intervention is increasingly advocated 59,60 and can be discussed with TBAD patients who need emergent repair. Future studies will show the exact role of TEVAR in TBAD treatment.…”

Endovascular stent grafting and open surgical replacement for chronic thoracic aortic aneurysms: a systematic review and prospective cohort study

“…Experts also recorded consensus for ESG for large aneurysms in the DTA in CTD patients at high operative risk, despite conventional 'wisdom' that implanting stents in the intrinsically weak aortic tissue of CTD patients should be avoided. [51][52][53] For the oldest patients, CTD played an influential role in decisions. Clinicians tended to prefer intervention for smaller aneurysms in patients with CTDs, possibly because of concerns that complications are seen with aneurysms of smaller diameters in this population.…”

Long-term outcomes after aortic root replacement for patients with Marfan syndrome

“…However, in one study of Marfan syndrome patients and AD, FET appeared to be an efficient therapeutic alternative, and positive aorta remodeling was observed through a delicate approach. Aortic stent grafting may become part of multistage aortic repair, allowing complete treatment of the dissected aorta (15). Both TAR and FET were found to be feasible and efficacious for type A AD following previous ARR in patients with Marfan syndrome (16), while true lumen expansion across the aorta, false lumen, and distal aorta stabilization has been shown to be obtainable through TEVAR (17,18).…”