Background: There are few data on the prevalence and clinical consequences of coronary artery aneurysms (CAAs) in adult patients with Marfan syndrome (MFS). Methods: We performed a retrospective cohort study of 109 patients with pathogenic variants in the FBN1 gene. Diameters of the left main coronary artery (LMCA) and right- coronary artery (RCA) were measured by computed tomography angiography. Results: The overall prevalence of CAA was 46%. The prevalence rates of CAA were 18% and 68% in patients with a native aortic root (group 1) and patients with previous aortic-root replacement (group 2), respectively. Previous aortic dissection or aortic intervention, longer time from aortic-root replacement, higher systemic score, significant mitral valve involvement, and diffuse aortic disease were correlated with CAA. During a mean follow-up of 8.5 ± 7.6 years, 4 patients developed pseudoaneurysms of the coronary anastomoses, requiring surgery. Conclusions: CAAs are common in adult patients with MFS and are associated with a more severe aortic phenotype and a longer follow-up after aortic-root replacement. Our study demonstrates that coronary artery size should be regularly followed, mostly after aortic-root replacement and in patients with severe aortic phenotypes. Large multicentre studies are warranted to elucidate the most appropriate surveillance plan.

Coronary Artery Aneurysms in Patients With Marfan Syndrome: Frequent, Progressive, and Relevant / Mariucci E.; Bonori L.; Lovato L.; Graziano C.; Ciuca C.; Pacini D.; Di Marco L.; Angeli E.; Careddu L.; Gargiulo G.; Donti A.. - In: CANADIAN JOURNAL OF CARDIOLOGY. - ISSN 0828-282X. - STAMPA. - 37:8(2021), pp. 1225-1231. [10.1016/j.cjca.2021.03.002]

Coronary Artery Aneurysms in Patients With Marfan Syndrome: Frequent, Progressive, and Relevant

Mariucci E.
Primo
;
Bonori L.;Ciuca C.;Pacini D.;Angeli E.;Careddu L.;Gargiulo G.
Ultimo
;
2021

Abstract

Background: There are few data on the prevalence and clinical consequences of coronary artery aneurysms (CAAs) in adult patients with Marfan syndrome (MFS). Methods: We performed a retrospective cohort study of 109 patients with pathogenic variants in the FBN1 gene. Diameters of the left main coronary artery (LMCA) and right- coronary artery (RCA) were measured by computed tomography angiography. Results: The overall prevalence of CAA was 46%. The prevalence rates of CAA were 18% and 68% in patients with a native aortic root (group 1) and patients with previous aortic-root replacement (group 2), respectively. Previous aortic dissection or aortic intervention, longer time from aortic-root replacement, higher systemic score, significant mitral valve involvement, and diffuse aortic disease were correlated with CAA. During a mean follow-up of 8.5 ± 7.6 years, 4 patients developed pseudoaneurysms of the coronary anastomoses, requiring surgery. Conclusions: CAAs are common in adult patients with MFS and are associated with a more severe aortic phenotype and a longer follow-up after aortic-root replacement. Our study demonstrates that coronary artery size should be regularly followed, mostly after aortic-root replacement and in patients with severe aortic phenotypes. Large multicentre studies are warranted to elucidate the most appropriate surveillance plan.
2021
Coronary Artery Aneurysms in Patients With Marfan Syndrome: Frequent, Progressive, and Relevant / Mariucci E.; Bonori L.; Lovato L.; Graziano C.; Ciuca C.; Pacini D.; Di Marco L.; Angeli E.; Careddu L.; Gargiulo G.; Donti A.. - In: CANADIAN JOURNAL OF CARDIOLOGY. - ISSN 0828-282X. - STAMPA. - 37:8(2021), pp. 1225-1231. [10.1016/j.cjca.2021.03.002]
Mariucci E.; Bonori L.; Lovato L.; Graziano C.; Ciuca C.; Pacini D.; Di Marco L.; Angeli E.; Careddu L.; Gargiulo G.; Donti A.
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/11585/849801
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