This dissertation based on the cross-sectional clinical study is the first research paper in Ukraine that analyses the status and develops health care programs for adult with congenital heart diseases (ACHD). The research problem is critically important because of the annual increase in the number of ACHD with unclearly defined sexual characteristics and factors impeding the adequate physical activity and worsening HRQoL. The clinical study of ACHD was performed in 2011-2016 within a larger observational study and taking into account the results of observation and treatment in previous years. The study was designed as prospective clinical research of 2044 ACHD (1059 men (52 %) and 985 women (48 %), ages 18-87 years (median age - 20 (25‰ -75‰) - 18-26 years) who visited the "Ukrainian Children's Cardiac Center" between April 1, 2011 and December 31, 2015. Adults with CHD who receive care at the specialized Cardiac Center are a heterogeneous group with a wide range of different primary pathological heart anomalies, the vast majority includes adults after surgical interventions for CHD (63.4 %), patients with varying degrees of complexity (32.2 % of patients with moderate and 16.2 % - with severe CHD). As for the CHD structure, 66.4 % of women had atrial septal defects, 56.7 % - ventricular septal defects, 67.5 % - patent ductus arteriosus, while most men had aortic valve stenosis - 74.8 % and coarctation of aorta - 64.2 %. The percentage of women with CHD having arrhythmias was higher compared to men (24.5 % and 20.1 % respectively); as well as the share of women with CHD with heart failure (HF) NYHA > 2 (46.8 % compared to 27.6 % of men), which required more frequent prescriptions and correction of drug therapy (67.4 % and 44.3 % respectively). Smoking, as a risk factor, appeared more frequently among men (16.7 % compared to only 3.4 % among women). Anxiety disorders among women with CHD were more manifest, were found in all age groups and had a clear tendency to deteriorate with age, namely, the level of anxiety of young women aged 18-20 years (5.4 ± 0.4 points), and 21-40 years (6.7 ± 0.3 points) were higher than the level of anxiety of men in the corresponding age groups (4.2 ± 0.3 and 5.5 ± 0.3 points respectively), while for elderly adults, in the absence of differences between genders, the level of anxiety was higher compared to young adults. Depressive disorders among ACHD arise later and more slowly, namely, the rate of depression was higher among women aged 21-40 years (5.3 ± 0.3 points) compared to men of this age group (4.1 ± 0.3 points; p<0.05), with significant higher depression among older adults in the absence of differences between genders. For adults with congenital aortic valve pathology in 5.8 years after the Ross operation, signs of HF NYHA II-III were associated with lower PWC170 (12.0 ± 0.8 and 14.1 ± 1.1 (kg · m)/min per 1 kg respectively, p<0,05) and maximum oxygen consumption (VO2max) (37.9 ± 2.1 and 43.6 ± 2.6 ml/(min · kg) respectively, p<0.05) compared with NYHA I, and was due to decreased right ventricular (RV) contractility according to TAPSE (17.9 ± 0.5 mm and 20.3 ± 0,9 mm respectively, p<0,05) in combination with significantly higher gradient on PA-RV conduit (37.8 ± 6.1 mm Hg and 27.3 ± 4.8 mm Hg respectively, p<0.05), as signs of RV dysfunction with preserved LV function. The peculiarity of ACHD was presence of two types of diagnoses: the "primary" diagnosis, such as the congenital anomaly of the heart in accordance with the general definition, and the "current" (preoperative) diagnosis were reflecting the acquired (residual/iatrogenic) pathology after the cardiac surgery procedures performed earlier. Current (preoperative) diagnoses, such as arrhythmologic pathology (4.5 %), RV outflow tract obstruction (3.7 %) and PA-RV conduits dysfunction (3.7 %) were an indication for repeated interventions for such patients and increased the difficulty of the disorder compared to primary pathology (severe - 11.5 % and 6.5 %, simple - 38.7 % and 45.6 % respectively).
