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

摘要

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.