Tashova S. Eisenmenger syndrome: clinical course, morpho-functional status of the heart and results of treatment during the long-time follow-up.

The thesis contains the results of complex study of congenital heart disease (CHD) with increased pulmonary blood flow in phase of Eisenmenger's syndrome (ES). The choise and the trend of the study were motivated by the frequency of this pathology in adults, absence of diagnostic criteria for medical patients, unknown cause of SE development in patients with CHD and absence of standards of medical treatment of ES. Therefore, the aim of the study was determination the role of endothelial dysfunction and of interleukins in the pathogenesis of ES, complex evaluation of cardiohaemodynamics and of morpho-functional status of the right and left parts of the heart depending on the type of CHD, as well as evaluation of the results of treatment by long-acting calcium channel blockers during 2-year follow-up. Evaluation of blood levels of endotheline-I (ET-I) and interleukins in ES patients revealed the increase of ET-I level in arterial blood. Taking into consideration its inactivation in pulmonary vessels, weconclude that its increase in arterial blood is related to the increase of its endothelial production under the impact of haemodynamic injury. The increase of pro-inflammatory cytokines' level (IL-1?, IL-2 and IL-6), as well as highly positive correlation between IL-1? and IL-6 --r=0.72 (p<0.001), IL-6 and TNF-a - r=0.28 (p<0.05) and between ET-I and IL-6 was revealed. The results obtained supply the up-to-date scientific data about the role of ET-I and cytokines in the pathogenesis of pulmonary hypertension and in sclerothic changes in ES patients, open new perspectives in diagnostics and evaluation of functional status of the heart in patients with CHD in phase of Eisenmenger's syndrome. They extend the ways of medical management of these patients.