Programmheft

Sitzung

261
Freie Mitteilungen 3
4. November 2022, 11:00 - 12:00, Gartensaal 2

Abstract

Non-dissecting distal aortic and peripheral arterial aneurysms in patients with Marfan syndrome
Q. Pellenc1, 2, A. Boitet2, Y. Castier2, O. Milleron2, G. Jondeau2, Presenter: Q. Pellenc1, 2 (1Morges, 2Paris)

Objective
In Marfan syndrome (MFS), aortic or peripheral arterial dilatation is usually the consequence of aortic dissection. Non-dissecting distal aortic and peripheral aneurysms (DAPA) are barely described. We sought to determine the incidence and prognostic impact of non-dissecting DAPA requiring surgical repair in a large population of patients with MFS
Methods
Patients referred to the French MFS Referral Center were included in a prospective database, and patients treated for a non-dissecting DAPA between 2013 and 2020 were retrospectively reviewed. First line therapy was open surgery. Patients unfit for open repair or experiencing life-threatening complication underwent endovascular repair
Results
Among 1575 patients with MFS, 19 (1.2%) were operated for 25 non-dissecting DAPA. The mean age was 42.4±11.5 years. Non-dissecting DAPA involved the subclavian or axillar artery (n=12), the descending or thoracoabdominal aorta (n=6), the abdominal aorta and iliac arteries (n=6), and the popliteal artery (n=1). Open and endovascular repairs were performed in 22 and 3 cases, respectively. After a median follow-up of 54.2 months, no local recurrence was noticed and no secondary procedure was performed. Eight patients presented a new aortic event including 2 aortic dissections and 7 new aortic surgeries. Compared to the overall MFS population, the non-dissecting DAPA group presented a significantly higher risk of aortic event (100% vs 28%, p<0.0001), a higher risk of aortic dissection (53% vs 8%, p<0.0001) (Fig.1), and a higher rate of pejorative genetic mutations (68% vs 40%, p=0.011).
Conclusion
Among patients with MFS, the diagnosis of non-dissecting DAPA reguiring surgery is infrequent but associated with a significantly adverse outcome, thus advocating for a specific follow-up
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