Abstract
Introduction . Marfan syndrome is a hereditary disease, which is based on a congenital dysplasia of the connective
tissue with the defeat of a number of organ systems. The most dramatic is the involvement in the
pathological process of the cardiovascular system.
Objective: the study of the pathogenesis and morphogenesis of aortic aneurizm in Marfan syndrome.
Material and methods . There are the results of histological examination of the aortic wall and aortic and
mitral valve presented in this research. A total of 52 patients were enrolled. Bentall–De Bono operation has
been performed in all cases. In 8 cases it was added with mitral valve replacement.
Results . It was found that the morphology weakness substrate of the aortic wall consisted mainly in two processes.
The first process is the focal elastics media degradation and destruction of smooth muscle with disorganization
structure was called cystic media aortic degeneration. And the second is diffuse and widespread
destruction of the elastic structures of the aorta with basophilic ground substance and the overall decrease in
the number of smooth muscle cells in the media. The degree of histological changes of the aortic and mitral
valves correlated with their macroscopic changes.
Conclusion . The revealed features of histological structure of the aortic wall and valve in case of Marfan syndrome
can be the additional criteria for choosing a method of surgical treatment of patients with ascending
aortic aneurysm.
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About the authors
- Bockeria Leo Antonovich, Academician of Russian Academy of Sciences and Russian Academy of Medical Sciences,
- Director of A.N. Bakulev Scientific Center for Cardiovascular Surgery;
- Serov Roman Andreevich, MD, PhD, Professor, Chief of Department;
- Rychin Sergey Vladimirovich, MD, PhD, Senior Research Associate
