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.