Elisabetta Mariucci1, Lisa Bonori2, Luigi Lovato3, Claudio Graziano4, Cristina Ciuca5, Davide Pacini6, Luca Di Marco6, Emanuela Angeli7, Lucio Careddu7, Gaetano Gargiulo7, Andrea Donti8. 1. Marfan and Heritable Thoracic Aortic Disease Clinic, IRCCS Azienda Ospedaliero-Universitaria di Bologna, Bologna, Italy; Pediatric Cardiology and Adult Congenital Heart Disease Program, IRCCS Azienda Ospedaliero-Universitaria di Bologna, Bologna, Italy. Electronic address: elisabettamariucci@gmail.com. 2. Marfan and Heritable Thoracic Aortic Disease Clinic, IRCCS Azienda Ospedaliero-Universitaria di Bologna, Bologna, Italy. 3. Marfan and Heritable Thoracic Aortic Disease Clinic, IRCCS Azienda Ospedaliero-Universitaria di Bologna, Bologna, Italy; Cardiovascular Radiology Unit, S. IRCCS Azienda Ospedaliero-Universitaria di Bologna, Bologna, Italy. 4. Department of Medical Genetics, IRCCS Azienda Ospedaliero-Universitaria di Bologna, Bologna, Italy. 5. Marfan and Heritable Thoracic Aortic Disease Clinic, IRCCS Azienda Ospedaliero-Universitaria di Bologna, Bologna, Italy; Pediatric Cardiology and Adult Congenital Heart Disease Program, IRCCS Azienda Ospedaliero-Universitaria di Bologna, Bologna, Italy. 6. Cardiac Surgery Unit, IRCCS Azienda Ospedaliero-Universitaria di Bologna, Bologna, Italy. 7. Pediatric and Adult Congenital Heart Cardiac Surgery, IRCCS Azienda Ospedaliero-Universitaria di Bologna, Bologna, Italy. 8. Pediatric Cardiology and Adult Congenital Heart Disease Program, IRCCS Azienda Ospedaliero-Universitaria di Bologna, Bologna, Italy.
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.
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.
Authors: Seyd Shnayien; Petra Gehle; Nick Lasse Beetz; Tobias Daniel Trippel; Karla Philipp; Christoph Maier; Thula Walter-Rittel Journal: Sci Rep Date: 2022-09-12 Impact factor: 4.996