Developed on the instructions of the Russian Health Ministry, approved by the Society of Emergency Cardiology and the leading committee of Cardiology National clinical guidelines on diagnosis and treatment of pulmonary hypertension working group: Chair of working group - corresponding Member of the Russian Academy of Sciences, prof. Chazova I.Ye. (Moscow), deputy chair of working group - PhD Martynyuk T.V. (Moscow), prof. Avdeev S.N. (Moscow), PhD Volkov A.V. (Moscow), prof. Nakonechnikov S.N. (Moscow)

The patient with the verified diagnosis of idiopathic pulmonary hypertension, functional class III (WHO) was treated by anticoagulants, diuretics, calcium channel blocker. After one year of adding endothelin receptor antagonist bosentan there was noticed the negative dynamic with right heart chambers dilation, appearing of heart failure signs. By 11 months of the combined pathogenetic therapy with bosentan and phosphodiesterase type 5 inhibitor sildenafil there was achieved the significant improvement of the functional and hemodynamic status. This positive dynamics remained within the next 3 years of observation.

The currentguidelines on chronic thromboembolic pulmonary hypertension (CTEPH) treatment consider pulmonary thrombendarterectomy as the first therapy choice. In case of inoperability due to severe patient's condition, distal lesions of pulmonary vessels, extremely high pulmonary vascular resistance, medical treatment for CTEPH includes life-long anticoagulation therapy and reduction of heart failure symptoms. This clinical case of the 59-y-old patient M. with inoperable CTEPH demonstrates the efficacy of phosphodiеsterase-5 inhibitor Sildenafil usedin high doses. The diagnosis was confirmed by the results of the complete diagnostic process including right heart catheterization and pulmonary angiography. The initial functional class was assessed as III in accordance with WHO classification. Sildenafil 60 mg daily was added tothe standard therapy (anticoagulant, calcium channel blocker) for initial four weeks. To the 4wk visit Sildenafil dose was increased to 240 mgdailytaking into account good tolerance of the treatment.The significant improvement of functional class, positive dynamic ofhemodynamic and echo parameters were found to 4 month of follow-up.

The aim of the multicenter prospective open study: to assess demographic and clinical characteristics of PAH patients, regions of their accommodation, features of diagnostic and medical strategy, and survival. Materials and methods. In the study we included PAH patients (group 1) aged >18 years observed in 10 expert centers of the Russian Federation from 01.01.2012 to 30.04.2014. Patients' data were brought in the electronic forms on the registry site www.pul-hyp.medibase.ru. Results. 198 patients (160 women/38 men) from 44 regions of the country were included in the study: 55.0% - IPAH, 35.9% - PAH associated with congenital heart disease, 7,6% - PAH associated with connective tissue disease, 1.0% - heritable PAH, 0.5% - PAH associated with portal hypertension. At the time of including in the register the patients' age median was 38.1 (28.753.8) years, the disease duration median was 5.9 (2.3-13.7) years. The 6MW distance was 394.84 ± 111.10 meters, dyspnea Borg index - 3.34 ± 1.41 points. 9.2% had FC I, 43.7% of FC II, 36.2% of FC III, 10.9% of FC IV. According to RHC mean PAP was 55 (43.25-64.0) mm Hg, CV 3.5 (2.9-4.33) l/min, CI 2.0 (1.82.3) l/min/m², PVR 1079 (762-1424.8) dinхс/см⁵. Positive acute vasodilator testing was found in 25% of patients. The spectrum of the most frequent associated diseases included gastrointestinal diseases (27%), arterial hypertension (14%), urolithiasis (9%) were. PAH-specific therapy was prescribed to 68% of patients (sildenafil - 75.4%, bosentan - 40.4%, iloprost - 9.5%). 9.6% of patients were observed in clinical trials. PAH monotherapy was prescribed to 66.7% of pts, the combined two-component therapy was recommended to 31.7% of pts, triple therapy- to 1.6% of pts. 51% of pts were treated with calcium channel blockers, 59% - diuretics, 82% - antitrombotic drugs. In PAH group the 1-y survival was 98%, the 2-y survival - 96%. Conclusion. Harmoniously built system network of expert centers will allow to improve early diagnostic of PAH, providing proper observation and lowering mortality rate.

The results of influence of long-term therapy with Ewator® and Predisin® on morphofunctional rates and painless myocardial ischemia in 57 premenopausal women with hypoestrogenemia and hypertension of 2 stagesare presented. The results of the present study show that this therapy in premenopausal women with hypertension can stop or even cause regression of left ventricular myocardial hypertrophy, endotelian disfunction, left heart dilations and has positive effect on frequency of painless myocardial ischemia (PMI) events.

Aim of the study: to study antihypertensive and organ-protective efficacy of indapamide and valsartan combined therapy hypertensive patients with high cardiovascular risk and metabolic disorders. Methods: The studyincluded 37 patients with stage I-III hypertension (ESH 2007), with an average age of 47.67±10.02 years, 48.6% men and 51.4% woman. Blood pressure was measured by Korotkov method. All patients were performed by M- and B-mode echocardiography. Flow-mediated endothelium dependent vasodilatation was measured during reactive hyperaemia due to 5 minute brachial occlusion.A 7.5 MHz highresolution ultrasound was used to measure carotid artery intimaemedia thickness (IMT). Blood lipid and glucose level, serum creatinin and uric acid level were estimated by enzyme assay method on biochemical analyzer “Daytona TM”. Results: 12-weekly combined therapy with indapamide and valsartan were shown very good antihypertensive efficacy in average daily doses 2.5 mg and 80±40.6 mg respectively, with reducing average blood pressure on 19.7±7.0% and attained goal level of BP in 92% cases. During the treatment were found significantly regress of left ventricular hypertrophy by reducing left ventricular mass index on 16.2±11.3%, effective vasoprotection, without significantly changes of metabolic disorders, which characterized indapamide and valsartan combination like metabolic neutral drug combination. Conclusion: Indapamide and valsartan combined therapy characterize with high antihypertensive, cardio- and vasoprotective efficacy andmetabolic neutrality choosing for treatment hypertensive patients with high cardiovascular risk and metabolic disorders.