Marfan Syndrome Research Today is a free monthly online journal that collates and summarizes the latest research about Marfan Syndrome, including details on genetics, causes, symptoms, treatment.

Variation in the histopathological features of patients with ascending aortic aneurysms: a study of 111 surgically excised cases.

Collins MJ, Dev V, Strauss BH, Fedak PW, Butany J

Department of Pathology, Toronto General Hospital, Toronto, Ontario, Canada.

BACKGROUND: Ascending aortic aneurysms (AA) are a common, though poorly understood medical condition. AIMS: To document the histological changes in a large series of human ascending AA, and to correlate these changes with clinical variables. METHODS: 111 ascending AA were excised at surgery over a 3 year period. Each aneurysm was received as a continuous ring of tissue. Sections were taken from the anterior, posterior, greater and lesser curvature of the aorta and graded in a semi-quantitative fashion for the degree of elastin fragmentation, elastin loss, smooth muscle cell (SMC) loss, intimal changes and inflammation. RESULTS: Mean patient age at surgery was 58.7 (15.6) years; there were 70 men and 41 women. 12 patients had Marfan syndrome, 34 (30.6%) had a bicuspid aortic valve (BAV), while 71 (64.0%) had a tricuspid aortic valve (TAV). Inflammatory cells were present in 28 cases (25.2%) and were confined to the adventitia. No particular region of the aortic circumference was more severely affected, however a BAV was associated with significantly less intimal change, and less fragmentation and loss of elastic tissue compared with patients with a TAV. Advanced age (>65 years), female gender and Marfan syndrome were all associated with more severe elastin degeneration and smooth muscle cell loss (p<0.05 for all). CONCLUSION: Results indicate a wide variation in the histological appearance in ascending AA, depending on patient characteristics. They suggest that the underlying aneurysm pathogenesis may also be highly variable; this warrants further investigation.

Published 31 March 2008 in J Clin Pathol, 61(4): 519-23.
Full-text of this article is available online (may require subscription).

© 2005-2008 Marfan Syndrome Research Today. All Rights Reserved.