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

Autor:	Pellenc Q; Department of Vascular and Thoracic Surgery, Bichat Hospital, Assistance Publique-Hopitaux de Paris (AP-HP), Paris, France.; Centre de Référence pour le Syndrome de Marfan et apparentés, Bichat Hospital, Assistance Publique-Hopitaux de Paris (AP-HP), Paris, France.; INSERM U 1148, LVTS, Bichat Hospital, Paris, France.; Université de Paris, Paris, France., Boitet A; Department of Vascular and Thoracic Surgery, Bichat Hospital, Assistance Publique-Hopitaux de Paris (AP-HP), Paris, France., Roussel A; Department of Vascular and Thoracic Surgery, Bichat Hospital, Assistance Publique-Hopitaux de Paris (AP-HP), Paris, France.; Université de Paris, Paris, France., Milleron O; Centre de Référence pour le Syndrome de Marfan et apparentés, Bichat Hospital, Assistance Publique-Hopitaux de Paris (AP-HP), Paris, France.; INSERM U 1148, LVTS, Bichat Hospital, Paris, France.; Department of Cardiology, Bichat Hospital, Assistance Publique-Hopitaux de Paris (AP-HP), Paris, France., Mordant P; Department of Vascular and Thoracic Surgery, Bichat Hospital, Assistance Publique-Hopitaux de Paris (AP-HP), Paris, France.; Université de Paris, Paris, France., Senemaud J; Department of Vascular and Thoracic Surgery, Bichat Hospital, Assistance Publique-Hopitaux de Paris (AP-HP), Paris, France.; INSERM U 1148, LVTS, Bichat Hospital, Paris, France.; Université de Paris, Paris, France., Cerceau P; Department of Vascular and Thoracic Surgery, Bichat Hospital, Assistance Publique-Hopitaux de Paris (AP-HP), Paris, France., Jondeau G; Centre de Référence pour le Syndrome de Marfan et apparentés, Bichat Hospital, Assistance Publique-Hopitaux de Paris (AP-HP), Paris, France.; INSERM U 1148, LVTS, Bichat Hospital, Paris, France.; Université de Paris, Paris, France.; Department of Cardiology, Bichat Hospital, Assistance Publique-Hopitaux de Paris (AP-HP), Paris, France., Castier Y; Department of Vascular and Thoracic Surgery, Bichat Hospital, Assistance Publique-Hopitaux de Paris (AP-HP), Paris, France.; INSERM U 1148, LVTS, Bichat Hospital, Paris, France.; Université de Paris, Paris, France.
Jazyk:	angličtina
Zdroj:	Frontiers in cardiovascular medicine [Front Cardiovasc Med] 2022 Mar 11; Vol. 9, pp. 827357. Date of Electronic Publication: 2022 Mar 11 (Print Publication: 2022).
DOI:	10.3389/fcvm.2022.827357
Abstrakt:	Background: In Marfan syndrome (MFS), an 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 a surgical repair in a large population of patients with MFS. Methods: The patients referred to the French MFS reference center were included in a prospective database, and the patients treated for a non-dissecting DAPA between 2013 and 2020 were retrospectively reviewed. The first-line therapy was open surgery. The patients unfit for open repair or experiencing life-threatening complications underwent endovascular repair. Results: Among 1,575 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 axillary artery ( n = 12), the descending or thoracoabdominal aorta ( n = 6), the abdominal aorta andiliac arteries ( n = 6), and the popliteal artery ( n = 1). Open and endovascular repairs were performed in 22 and three 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 two aortic dissections and seven new aortic surgeries. Compared to the overall MFS population, the non-dissecting DAPA group presented a significantly higher risk of an 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 the patients with MFS, the diagnosis of non-dissecting DAPA is infrequent but is associated with a significant adverse outcome, thus, advocating for a specific follow-up. Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest. (Copyright © 2022 Pellenc, Boitet, Roussel, Milleron, Mordant, Senemaud, Cerceau, Jondeau and Castier.)
Databáze:	MEDLINE
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