
The aim. To study of the features of clinical, functional indices and quality of life of patients with primary and secondary pulmonary arterial hypertension (PAH). Materials and methods. The study included 42 patients with PAH (34 women and 8 men, with an average age of 43.4 ± 11.7 years). At the same time, 16 (38%) patients had idiopathic PH, were further analyzed as I group (husband / wife 3/13, 39 ± 12.7 years), and 26 (62%) had secondary PAH caused by acquired defects heart, chronic thromboembolic PAH, and also ischemic and dilated cardiomyopathy (group II, husband / wife 12/14; 44.8 ± 10.6 years). The complex of diagnostic examination methods, in addition to objective examination, included: a 6-minute walking test, a standard 12-lead ECG, echocardiography, and direct tonsometry, with parameters such as pressure in PA, seizure pressure in PA. The quality of life was assessed using the international questionnaire EQ–5D. Results. Mean values of PA pressure in patients of Group I were 74.3 ± 10.7 mm Hg. At that, 4 (25%) of them were characterized by 2 degrees of LH, and 12 (75%) - the third. The majority of patients, at the time of enrollment, were characterized by a severe condition, not revealing intergroup differences, as evidenced by the mean of CHF (NYHA). The average duration of the disease at the time of inclusion in the study was more than 3 years in both groups. Gender distribution of patients as a whole in groups showed that patients with female PAH were the majority. In the case of intergroup comparison, the number of females was also significantly higher among patients with idiopathic PAH (81% versus 54%, p = 0.02). Among patients with secondary PAH, there were almost 3.5 times more patients with different forms of atrial fibrillation (38% and 12%, respectively, p <0.01). In a comparative analysis of the severity and severity of PAH in patients of both groups, it was found that among patients with Aerza’s disease, there were no persons with mild PAH. And, on the contrary, patients