AUTHOR=Pellenc Quentin , Boitet Auréline , Roussel Arnaud , Milleron Olivier , Mordant Pierre , Senemaud Jean , Cerceau Pierre , Jondeau Guillaume , Castier Yves 

TITLE=Non-Dissecting Distal Aortic and Peripheral Arterial Aneurysms in Patients With Marfan Syndrome

JOURNAL=Frontiers in Cardiovascular Medicine

VOLUME=Volume 9 - 2022

YEAR=2022

URL=https://www.frontiersin.org/journals/cardiovascular-medicine/articles/10.3389/fcvm.2022.827357

DOI=10.3389/fcvm.2022.827357

ISSN=2297-055X

ABSTRACT=Background: 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 reference 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), 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 is infrequent but associated with a significantly adverse outcome, thus advocating for a specific follow-up.