These Practical Guidelines are developed for general practitioners and cardiologists to demonstrate definition, diagnostic criteria and current approaches to treatment of familial hypercholesterolaemia.

Aim: The authors assess anemia as a predictor of poor outcome in patients with lesions of the left main coronary artery.

Material and methods. The study included 112 patients of both sexes with a lesions of the left main coronary artery, in combination with the defeat another one coronary artery, according coronary angiography, were hospitalized in RSCC in the 2015-2016 years.

Result. The authors found that in most cases, anemia in patients with coronary heart disease is not of iron and therefore iron administration is useless. In addition, anemia is an independent predictor of adverse outcomes in patients with coronary artery disease with lesions of the left main coronary artery. In the complex groups of patients needed correction and monitoring of hemoglobin levels, in order to prevent a decrease and increase it above the recommended values.

Modern concepts of pulmonary arterial hypertension (PAH) pathogenesis focus on the key role of endothelial dysfunction of pulmonary vessels. To control the activation of endothelin-1 system, endothelin receptor antagonists (ERA) are current ly used. Until recently, this class of drugs in our country and abroad was represented by two drugs: the sulfonamide derivative - a nonselective ERA bosentan and a non-sulfonamide derivative - ambrisentan, which blocks only ETA-receptors. Not the selectivity of ERAs, but their pharmacokinetic characteristics determine the differences in the profile of efficacy and safety. In 2015, in our country there appeared a new dual antagonist macitentan, which was created to optimize the tissue effects by increasing lipophilicity. In randomized SERAPHIN study, the use of macitental 10 mg compared with placebo contributed to a reduction of the risk of morbidity and mortality in patients with PAH by 45%, and the effect of therapy was not dependent on whether the patients received concomitant specific therapy with inhibitors of phosphodiesterase type 5, oral or inhaled prostanoids. In the paper there summarized indications for prescribing ERA, the data of the evidence base, as well as the concept of switching to optimize the ERA treatment. It is important to emphasize that in case of a satisfactory clinical response with ERA therapy, correction of therapy seems discrete. In the European guidelines 2015, this issue is not considered as having no extensive evidence base. On the other hand, it cannot be ruled out that the use of generic bosentan may lead to a decrease in the treatment effecacy and to provoke the clinical deterioration. At present clinical data intensively accumulate in favor of the strategy of switching from bosentan or ambrisentan to macitentan in PAH patients. It is necessary to assess the potential benefits associated with ERA switching to macitentan, in comparison with the possibilities of combination therapy.

Medical rehabilitation (MR) has become one of the important tasks of treatment in patients with coronary heart disease (CHD).

The aim - assessment of the effectiveness of spa resort MR in patients with CHD under spa resort treatment (SRT) in view of the provisions of the "International Classification of Functioning, Disability and Health" (ICF).

Material and methods. The study included 117 CHD patients treated with SRT at Yalta resort. Selection of patients for the study was carried out with informed consent of patients and duration of treatment of at least 18 days. Methods of investigation and treatment were applied in accordance with the features of the functional state of the patient, the individual indications and the standards of SRT at CHD. Psychological research and evaluation of quality of life (by the tests of Reeder, Beck, Spielberger-Hanin, SF-36) were additionally conducted. The analysis of the results was carried out by methods of variation statistics.

Results and its discussion. Spa resort MR in patients with CHD is characterized by high efficiency. Reliable positive trends were set for a number of domains: the sensation of pain and dizziness (b280 and b2401), blood pressure (b420), tolerance to physical exercises (b455), general metabolic functions (b540) and the sensations associated with the functioning of the cardiovascular and respiratory systems (b4601).

Conclusions. Statistically significant (p<0,05) reduction of six domain’s value by the end of the course of treatment indicates positive rehabilitation potential of SRT in patients with CHD.

The goal is to evaluate the efficacy and security of treatment of patients with arterial hypertension (AH) during the heat wave of a fixed combination of ARBS+ACC (losartan+amlodipine).

Materials and methods. Included 26 patients with hypertension 1 and 2 degrees from 42 to 81 years. All patients underwent measurement of office blood pressure, 24h ambulatory BP monitoring, Self-measurement of BP (Home BP ), electrocardiography, biochemical analysis of blood, estimation of osmolarity of blood, questionnaire: visual analogue scale, a questionnaire for patients exposed to heat, the test of adherence to treatment Moriscos-Green. Estimated diaries of self-control of blood pressure. The inclusion visit took place in spring 2016, 1st visit was in May-June 2016, 2nd - during heat waves, 3rd -September-October 2016.

Results. The observed decrease in systolic blood pressure and diastolic blood pressure (p=0.000) to the target values, preserved for the whole period of observation. According to the diaries of selfcontrol of blood pressure controlled blood pressure 81% of patients. During a heat wave, this value decreased to 58%, in autumn - to 63%. The 3rd visit achieved a reduction in heart rate at -6.0 (-11.1; and 2.8) beats/min; p=0.007; the decline in uric acid level c of 415.3 to 346.2 mmol/l (p=0.04) and creatinine on the 2nd visit compared to baseline (p=0.02). According to 24h ambulatory BP monitoring, 24h ambulatory BP monitoring, during a single visit, the mean SBP decreased from 139 to 121 mm Hg. (P=0.04) and DBP from 88 to 76 mm Hg. (P=0.04) in the daytime and the tendency to decrease SBP variability from 12 to 10 mm (p=0.07). At the end of the study, the indicators remained stable. The mean value of SBP decreased at night to the third visit from 116 to 110.5 mm Hg. (P=0.04). During the heat wave, a larger mean SBP and DBP was recorded compared with the third visit (p=0.04). A correlation was found between the mean DBP value (r=0.725, p=0.03), the SBP time index (r=0.695, p=0.04) and the SBP time (r=0.787, p=0.02) at night and Quality of life Electrolyte shifts and increasing the osmolarity of the blood during heat waves have not been identified. There is a growing commitment therapy (p=0.04) and quality of life to 20.0 (7.4; 23.3); p=0.000 by visual analogue scale.

Conclusion. Fixed combination of losartan and amlodipine (Lorenza) is an effective, safe and may be recommended for hypertensive patients during heat wave and seasonal cold snap.

According to the recommendations of the European Society of Cardiologists, transthoracic echocardiography (TTE) is the most informative diagnostic method in patients with chronic heart failure (CHF) [3]. Echocardiography provides accurate information on the size of heart chambers, wall thickness and indices of systolic and diastolic function. Nowadays, in view of the new technological development, the most perspective technologies for the integrated assessment of the functional condition of the myocardium of the left ventricle (LV) are the ones that assess not only the ejection fraction (EF), but also the deformation parameters and rotational properties of the LV myocardium, including technologies of nonDoppler myocardial images in 2D and 3D modes (2D and 3D Speckle Tracking Echocardiography or STE). Based on the 2D Speckle Tracking Echocardiography data, reduction of the global longitudinal deformation has more prognostic significance in risk of death assessment in patients with heart failure than the EF of LV and the index breach of the local contractility of LV. However, the method of evaluating myocardial deformation in 3D mode STE and its advantages over the 2D mode STE is insufficiently explored. Thus, for the assessment of LV systolic and diastolic dysfunctions in patients with CHF with preserved and reduced EF of LV, a great interest represents the application of the new echocardiographic technologies, such as the 2D and 3D Speckle Tracking Echocardiography.

Introduction. To improve the diagnosis, to examine the prevalence, structure, evaluation of effectiveness of therapy it is necessary to create register of pulmonary hypertension.

The purpose of the study: to study the efficacy, the safety of the drug bosentan in patients with idiopathic pulmonary hypertension (ILG) according to the register of the Voronezh region.

Material and methods. The Material was the data base of the register. Results. Analyses the prevalence, the incidence of ILG. Peculiarities of clinical course depending on the hemodynamic and functional status of patients of ILG.

Conclusion. The efficacy and safety of the drug bosentan in ILG.

Idiopathic pulmonary hypertension (IPAH) is a rare cardiovascular disease of unknown etiology, but the most common form of pulmonary arterial hypertension, defined as mean pulmonary arterial pressure >25 mmHg and pulmonary vascular resistance >3 Wood Units. Endothelial dysfunction seems to play an integral role in the pathogenesis of IPAH: the activation of endothelin-1 causes the hypertrophy of smooth muscle cells and endothelial cells, fibrotic changes, immune inflammation and vasoconstriction which leads to small pulmonary arteries and arterioles remodeling. The endothelial system activation is the reason for using endothelin receptor antagonists (ERA) to block the endothelin receptors type A (ETA) or both types (ETA, ETB). ERA, as the main class of PAH-specific therapy, is presented by bosentan and macitentan, dual endothelin receptor antagonists, and ambrisentan, non-sulfonamide, ETA-selective ERA. This clinical case demonstrates the successful treatment with ambrisentan as the part of combination therapy in the patient with severe IPAH.