Aortic Branch Aneurysms and Vascular Risk in Patients With Marfan Syndrome

López-Sainz, Ángela; Milà, Laia; Rodríguez-Palomares, José F.; Limeres, J; Granato, Chiara; Mura, L La; A, Sabaté; Guala, Andrea; Gutiérrez, Laura; Galián-Gay, Laura; Sao-Avilés, Augusto; Bellmunt, Sergi; Rodríguez, Rafael; Cuéllar-Calàbria, Hug; Roque, Albert; Ferreira‐González, Ignacio; Evangelista, Artur; Teixidó-Turà, Gisela

doi:10.1016/j.jacc.2021.04.054

Cited by 22 publications

(15 citation statements)

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“…Our results are consistent with previous studies ( 5 , 7 , 24 ), showing an adverse impact of non-dissecting DAPA on the prognosis of patients with MFS, thus, highlighting the need for an adaptation of cardiovascular care with a specific follow-up protocol. These reports corroborate the need for screening distal aortic and peripheral aneurysms in patients with FBN1 -mutated MFS, especially after the first aortic event (aortic surgery or acute aortic dissection).…”

Section: Discussionsupporting

confidence: 93%

“…In a recent study, Lopez–Sainz et al demonstrated that aortic branch aneurysms are related to both age and ascending aorta dilatation. Their presence independently predicts the need for aortic surgery (the hazard ratio: 3.4; 95% confidence interval: 1.1 to 10.3; p = 0.028) ( 5 ). One explanation of the particular severity of this population could come from genetic analysis.…”

Section: Discussionmentioning

confidence: 99%

“…No recommendations exist concerning the peripheral aneurysms screening in patients with MFS (4, 10). This entity is, thus, probably underdiagnosed in a patient population with MFS, as suggested by Lopez-Sainz et al who found the aortic branch aneurysms in almost 27% of the MFS-mutated population (5). In a preliminary report, Yetman et al described that more than 30% (44/140) of their cohort of patients with MFS had distal aortic or peripheral non-dissecting aneurysms after thoracoabdominal CTA or MRI (7).…”

Section: Incidence and Localizationmentioning

confidence: 94%

“…Consequently, the incidence, management, and prognostic impact of distal aortic and peripheral aneurysms (DAPA) that are not related to aortic dissection remain unknown in patients with MFS. A recent study has suggested that aortic branch aneurysms could be present in up to one-quarter of patients with MFS and might independently predict the need for aortic surgery ( 5 ). We sought to determine the incidence, management, and prognostic impact of non-dissecting distal aortic and peripheral aneurysms (DAPA), requiring a surgical repair in patients referred to the French MFS reference center, with a special focus on the prognostic impact of this condition on a subsequent aortic event and overall survival.…”

Section: Introductionmentioning

confidence: 99%

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

Pellenc

Boitet

Roussel

et al. 2022

Front. Cardiovasc. Med.

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BackgroundIn 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.MethodsThe 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.ResultsAmong 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).ConclusionAmong 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.

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Section: Discussionsupporting

confidence: 93%

Section: Discussionmentioning

confidence: 99%

Section: Incidence and Localizationmentioning

confidence: 94%

Section: Introductionmentioning

confidence: 99%

See 2 more Smart Citations

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

Pellenc

Boitet

Roussel

et al. 2022

Front. Cardiovasc. Med.

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“… 10 The branch arteries of the aorta are also dilated in MFS; however, only 4.8% of these patients were treated during follow-up, and no spontaneous ruptures occurred. 11 In contrast, in patients with vEDS, complications in the aorta are not as common as they are in those with MFS. One study of patients with vEDS reported that aortic events occurred in 13% of patients, and events in the branch arteries of the abdominal aorta occurred in 12%.…”

Section: Discussionmentioning

confidence: 98%