Relation of aortic root dilatation and age in Marfan's syndrome

Abstract

Aortic root dilatation develops early in MFS and was present in 35% by the age of 5 years and 68% by 19 years. Even though new aortic root dilation is relatively rare, it is not possible to safely discharge patients with MFS as about one-third of the patients in our series who developed new pathological aortic root dilation did so after the age of 19 years.

The Aortic Root: Natural History After Root-Sparing Ascending Replacement in Nonsyndromic Aneurysmal Patients

“…The most important impact on root dilation is exerted by the Marfan syndrome. Based on a mutation in the FBN1 gene encoding fibrillin-1 [27], Marfan patients had progressive dilation of the root (approximately 60% to 84% of all patients [28]) with the risk of dissection, rupture, and valve regurgitation. The growth rate in this connective tissue disorder was estimated to be approximately 1.5 mm per year by Lazarevic and colleagues [29] in 2006, and therefore was 3.5-fold higher than in the present nonsyndomic cohort.…”

Aortic root ectasia as a phenotypic feature of a mitochondrial disorder

“…Aortic root ectasia may not only be due to a mitochondrial defect but also due to arterial hypertension, Marfan syndrome, osteogenesis imperfecta, senescence, or congenital heart defects (anulo‐aortic ectasia) . Additionally, ARE has been recently detected as a major determinant of aortic dilation .…”

Anomalous Coronary Artery Origin in a Young Patient with Marfan Syndrome

“…Dilation of the aorta root and its descending portion (60–100%), with secondary aortic insufficiency, increases risk of acute aortic dissection and death. Aburawi and O'Sullivan described that aortic root dilatation develops early in MFS and was present in 35% by the age of 5 years, 68% by 19 years, and at least 80% by 40 years [ 11 ]. Other cardiac complications are less common in Marfan syndrome [ 12 – 20 ].…”