Pulmonary arterial hypertension (PAH) is a rare disease, diagnosed at a late stage with low functional class III or IV (WHO). PAH leads to severe right heart failure and ultimately, death. The modern researches aim at exploring the potential therapeutic targets, as at developing new drugs that can affect the previously set target. Impaired NO production plays an important role in PAH pathogenesis; this is determined by the powerful vasodilatory action, as well as anti-inflammatory, anti-proliferative, and antiaggregatory effects. Riociguat is the first in a new class of soluble guanylatecyclase stimulators to have proved efficacy in phase II of clinical trials. In the randomized, double-blind, placebo-controlled phase III study PATENT-1 (Pulmonary Arterial Hypertension soluble Guanilatcyclase-Stimulator Trial) study, 443 patients with PAH symptoms were randomized to receive placebo of riociguat in a single dose of 2,5 mg (with a dose titration based on tolerability to 2,5 mg TID a day) or a dose of 1,5 mg (with a dose titration according to portability to 1,5 mg TID three times a day). The study included naïve patients treated with endothelin receptor antagonists or prostanoids (except for parenteral ones). To 12wk of riociguat treatment the mean distance in 6-MWT increased by an average of 30 m in the group treated with the maximum single dose of 2,5 mg TID, or decreased by an average of 6m in the placebo group (difference between groups, 36 m, 95% confidence interval 20-52 m, p p<0,001). Riociguat improved 6-MWT in patients not previously treated with PAH-specific therapy (38 m), and in patients, taking endothelin receptor antagonists or prostanoids (36 m). In riociguat groups compared with placebo a decrease in PVR and PAPm (p<0,01) was noted, as well as an increase in cardiac index (p<0,0001) , a reduction in NT-proBNP (p<0,0001) , FC (p=0,003) and in the Borg index (p=0,002), ), the time of development of clinical deterioration (p=0,005) prolonged as well. Riociguat therapy was also characterized by good tolerance. Efficacy of treatment was maintained during long-term observation of PATENT-2study. The mean value of 6-MWT changed to 51±74 м

The pulmonary hypertension is characterized by increase of pulmonary vascular resistance, pressure in a pulmonary artery, remodeling of heart and vessels and the lowest survival of patients. It agrees offered in 2009 the European society of cardiologists to algorithm a ventilation/perfusion scintigraphy takes the central place in differential diagnostics between various forms of a pulmonary hypertension. The scintigraphic picture is well studied at patients with the pulmonary hypertension which has developed owing to the transferred thromboembolism of branches of a pulmonary artery. The distributions of RFP given direct comparison and nature of changes of perfusion depending on a genesis of pulmonary hypertension and gravity of a disease in literature aren't presented. At the same time the knowledge of features of scintigrams can be useful in differential diagnostics and a forecast assessment at patients with a pulmonary hypertension of a various etiology.

Objective. Available data on the diagnostic value of determination of the concentration N-terminal pro-C-type natriuretic peptide (Nt-proCNP) to predict pulmonary hypertension (PH) in patients with COPD are few. Aim. To determine the concentration of NT-proCNP in COPD patients and evaluate its diagnostic value for the prediction of PH in COPD. Methods. The study involved 47 patients with COPD (GOLD II-IV, age 59,49±0,63 years, disease duration 13,7±0,63 years, smoking history 23,09±0,93 packs/years). NT-proCNP concentration in serum was assessed by ELISA technique (test Biomedica Medizinprodukte GmbH & Co KG, Austria). The pulmonary artery systolic pressure (SPAP) was analyzed by using a color-Doppler technique. Results. Concentrations of NT-proCNP were significantly higher in COPD patients with PH (SPAP 40-55 mmHg, n=16) or severe PH (SPAP ≥55 mmHg, n=10) than in patients without PH (SPAP < 40 mmHg, n=21): 4.14±0.51 pg/ml, 5.26±0.21 pg/ml and 1.42±0.03 pg/ml (p<0.001), respectively. A significant correlation was found between the concentration of NT-proCNP and SPAP (r=0.53; p<0.05). The AUC for Nt-proCNP levels to predict PH and severe PH in COPD were 0.928 and 0.932, respectively. Conclusion. The present study shows that the serum levels of NT-proCNP are significantly elevated in COPD patients with PH and can be used as a non-invasive specific biomarker for predicting PH in COPD.

The review is devoted to a problem of the pulmonary arterial hypertension associated with a systemic sclerosis. Data on prevalence, genetic aspects, clinical features and the diagnostic algorithms used for early diagnostics of this fatal manifestation of a system sclerosis are submitted.

The patient with the verified diagnosis of idiopathic pulmonary hypertension, negative acute vasoreactivity testing by RHC, functional class III (WHO) was treated by phosphodiesterase type 5 inhibitor sildenafil. After one year there was achieved the significant improvement of the functional and hemodynamic status.

Idiopathic pulmonary arterial hypertension (IPAH) is a rare cardiovascular disease of unknown etiology, characterized by pulmonary vascular resistance (PVR) and pulmonary artery pressure (PAP) increase due to pronounced remodeling of small pulmonary arteries and arterioles, with progressive deterioration and the rapid development of right ventricle failure. The key pathogenetic feature is the endothelial dysfunction, which leads to the development of vasoconstriction, thrombosis in situ, proliferative and inflammatory changes in the lung microvasculature. Optimization of drug therapy is associated with the clinical implementation of highly effective pathogenetic drugs which act on the main disease targets - activation of endothelin-1 (ET-1) system, the lack of endogenous prostacyclin and nitric oxide. The role of ET-1 as a powerful vasoconstrictor that induces cell proliferation and differentiation, production of cytokines, growth factors, biologically active substances. Endothelin receptor antagonists (ERA) represents the important class of PAH-specific therapy, including two non-selective ERAs-nonselective bosentan and selective ambrizentan. Presented clinical case demonstrates the long-term reliable efficacy and safety of ambrizentan for 10 years in the IPAH patient with the baseline functional class III (WHO).