Canadian Journal of Cardiology
Clinical Research| Volume 37, ISSUE 8, P1225-1231, August 2021

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

Published:March 08, 2021DOI:



      There are few data on the prevalence and clinical consequences of coronary artery aneurysms (CAAs) in adult patients with Marfan syndrome (MFS).


      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.


      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.


      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.



      Rares sont les données sur la prévalence et les conséquences cliniques de l’anévrysme de l’artère coronaire (AAC) chez les patients adultes atteints du syndrome de Marfan.


      Nous avons réalisé une étude de cohorte rétrospective sur 109 patients porteurs de variants pathogènes du gène FBN1. Les diamètres de l’artère coronaire principale gauche (ACPG) et de l’artère coronaire droite (ACD) ont été mesurés par angiographie par tomodensitométrie.


      La prévalence globale de l’AAC était de 46 %. Les taux de prévalence de l’AAC étaient de 18 % et de 68 % chez les patients présentant une racine aortique native (groupe 1) et chez ceux ayant subi un remplacement de la racine aortique (groupe 2), respectivement. Une corrélation a été observée entre l’AAC et une dissection ou une intervention aortique antérieure, un temps écoulé plus long depuis le remplacement de la racine aortique, un score systémique plus élevé, une atteinte importante de la valve mitrale et une maladie aortique. Pendant le suivi d’une durée moyenne de 8,5 ± 7,6 ans, de faux anévrysmes anastomotiques des coronaires nécessitant une intervention chirurgicale sont apparus chez quatre patients.


      Les AAC sont courants chez les patients adultes atteints du syndrome de Marfan et sont associés à un phénotype aortique plus sévère et à une période de suivi plus longue après le remplacement de la racine aortique. Notre étude démontre que le diamètre de l’artère coronaire devrait faire l’objet d’un suivi régulier, en particulier après le remplacement de la racine aortique et chez les patients présentant un phénotype aortique sévère. Des études multicentriques de grande envergure permettraient de déterminer les modalités de surveillance les plus appropriées.
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