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The authors examined the state of patients suffering from Marfan syndrome (MS) who endured operation for ascending aorta aneurysm with replacement of the ascending aorta and aortic valve (Bentall operation), studying alterations of the skeleton, face, heart and eyes, as well as pathomorphological restructurings in the aortic wall. The study was carried out 7.0 ± 4.2 years after the operation. We examined a total of 39 patients with MS - 27 (69.2%) men and 12 (30.8%) women aged from 22 to 70 years old (average age - 42.1 ± 13.4 years). All patients were operated on for dissecting aneurysm of the ascending aorta accompanied by a considerable degree of aortic valve insufficiency or aortic ostium stenosis. The mean diameter of the aorta at the level of the sinuses of Valsalva amounted to 7.0 ± 1.3 cm (minimal - 5.0 cm, maximal - 12.0 cm), the Z-score prior to operation was 12.7 ± 6.5. The time form making the diagnosis of MS to surgical intervention for aortic aneurysm amounted to 9.6 ± 5.9 years. The condition after operative treatment in all patients was satisfactory, with the haemodynamic indices stable: systolic AP - 133.5 ± 19.1 mm Hg, diastolic AP 85.1 ± 12.9 mm Hg, heart rate 74.8 ± 7.2 bpm. The average systemic score for the symptoms and tests of MS patients amounted to 8.2 ± 3.3 points. Pathohistological alterations of the aorta in patients with Marfan syndrome consisted in pronounced restructuring of the wall with deep irreversible alternative changes. The pathological process extended in the middle aortic layer all alone the length, but not only in the portions of rupture and dissection. The main pathomorphological signs in MS were as follows: focal accumulations of mucoid substances, dystrophic alterations of smooth-muscle cells, ribbon-like anuclear zones, formation of cystlike cavities, alterations of elastic fibres - fragmentation, hyperelastosis, multiplication, thinning and straightening, zones of elastolysis.

Twenty four cases of Gsell-Erdheim syndrome observed in 1999-2001 have been examined. There were 20 men (83.4%) and 4 women (16.6%). The age ranged from 22 to 69 years but 6 men (25%) died at age under 50. The following variants of aortic lesions were revealed in autopsy material: intimal tear with the following dissection which extends along the aorta to variable distances--in most cases into iliac arteries. The morphologic changes, associated with were: anuclear zones, elastolysis, thinning, dyschromia and fragmentation of elastic fibers, focal hyperelastosis. Such changes were revealed in all parts of the aortic wall, independently of the site of rupture.

24 cases of Gsell-Erdheim syndrome observed in 1999-2001 have been analysed. There were 20 men (83.4%) and 4 women (16.6%) aged 22-69 years. Six men (25%) died at the age under 50 years. At necropsies the following characteristic variants of stratification were observed: a total damage with stratification of the aorta at all length or, less often, local damage. Destruction of an elastic skeleton of the media is the basis of aorta stratification: zones free of the nuclei, foci of elastolysis (medianecrosis), thinning, dyschromia and fragmentation of elastic fibers, focal hyperelastosis. The pathological process involves all the aortic length not only the area of aneurysm and rupture. Manifestations of variable severity of the same process were seen in different parts of the aortic wall.

101 necropsies of aortic dissection (AD) made in 1991-2000 have been analysed. Among the deceased men prevailed (76.3%), 23(22.8%) of them were under 50 years of age. An accurate intravital diagnosis was made only in 40.6 patients. The most common clinical masks were the following: coronary, valvular, vascular, gastrointestinal, pulmonary, thromboembolic, mediastinal, cerebral, renal and anemic. Most often AD masks as acute myocardial infarction. The knowledge of encountered clinical masks of AD promoted improvement of early AD diagnosis to correct AD surgically and reduce lethality.

Three observations (2 males, aged 44 and 63, and a female of 47) of chronic dissecting aortic aneurysms are described. In all the cases the patients didn't die immediately from the acute dissection, as the blood accumulated in the canal formed, broke back into the aortic lumen, thus a lower orifice of the chronic aneurysm was formed. It might be possible that in some aortic dissections the hematomas fused, forming subsequently a canal. The new canal joined the main trunk (the aorta) by 2 or 3 orifices. The duration of the dissection was not identified clearly, but the canals formed were in all cases organized and had a smooth inner surface. Histology revealed striate-like necroses in the aortic wall, as well as fragmentation, swelling and lysis of the elastic fibers, resulting in the formation of slits, cavities with accumulation of metachromatic substances, i. e. the changes, characteristic for the medianecrosis (Gsell-Erdheim syndrome). A possible primary injuring effect of xenobiotics on the aortic wall is suggested.

By means of lectin-peroxidase technique histotopography of receptor sites for Ricinus communis agglutinin (RCA), peanut agglutinin (PA), soybean agglutinin (SA), Sophora japonica lectin (SJL), wheat germ agglutinin (WGA), Laburnum anagyroides lectin (LAL), Lotus tetragonolobus lectin (LTL) and concanavalin A (con A) in normal aortic tissue sections as well as in dissecting aortic aneurysms (Gsell-Erdheim syndrome) has been investigated. It was demonstrated that fucose-specific LAL and LTL possess high selectivity of binding to aortic endotheliocytes, SA to neurocytes of autonomic nervous nodes. Gsell-Erdheim syndrome is accompanied by appearance of SA-binding sites around smooth myocytes with formation of specific SA-positive multivesicular shapes in aortic media. Possible role of the observed phenomena in pathogenesis of dissecting aortic aneurysm is discussed.