This article presents the current view on the structure and functions of nuclear lamin proteins, pathological phenotypes of persons with LMNA mutations and clinical problems of lamin-related dilated cardiomyopathy diagnostics and management. Dilated cardiomyopathy (DCM) caused by mutations in the LMNA gene is often associated with conduction disorders, cardiac arrhythmias and extracardiac features with discrete muscle disruption. This disease is characterized by a poor prognosis and high risk of sudden cardiac death due to conduction disturbances (life-threatening blockade) or ventricular tachyarrhythmias (fatal ventricular tachycardias\fibrillation). We describe here 2 cases of lamin-type DCM associated with definite LMNA mutations. Educational focus for reader-cardiologist is а recognition of potential carriers followed by molecular genetic testing for diagnostics of LMNA mutation and tissues regarding optimal management of patients, especially timing for prophylactic cardioverter-defibrillator.

Clinical and hemodynamic profile and neurohormonal indicators in patients with idiopathic pulmonary hypertension, depending on vasoreactivity reserve of pulmonary arteries. Objective: the main objective of our research work was an evaluation of clinical and hemodynamic profile, neurohormonal indicators in patients with IPH, depending on the results of vasoreactivity testing. Methods: the study included 131 patients with IPH (mean age 32,4±3,2 years. All the patients underwent the routine laboratory tests, 6-minuts walking test (6-MWT), transthoracic echocardiography, thorax organs radiography, right heart catheterization (RHC) with acute pharmacological test (APT) using one or two vasodilators (VD) In order to study neurohumoral status the analysis of the 6-keto-PgF1β, NO, ET-1, norepinephrine, epinephrine levels, NT-proANP, NT-proBNP (NUP), renin activity, AII, aldosterone (RAAS) activity was conducted. Results: significant differences of clinical and hemodynamic profile and neurohormonal indicators in patients with IPH depending on the availability (APT +) and absence (APT-) of the vasoreactivity reserve of pulmonary arteries (PA) were established. Among the APT- patients the number of patients belonging to FC III was significantly greater in comparison with APT+ patients. FC IV refered only to the APT- patients. Significantly more patients with APT+ belonged to FC I and II. The 6-MWT revealed that patients with IPH with intact vasodilation reserve overcame significantly longer distance. Doppler echocardiography showed that in APT-group a greater level of systolic pressure in the pulmonary artery (SPPA). When comparing the RHDC data all the hemodynamic parameters in the groups varied considerably, prognostically better data marked APT+ group. According to thorax organs radiography APT- group had significantly higher leel of KTI. Comparative evaluation of neurohumoral status, regardless of the outcome of the APT, showed a significant difference in the groups. Conclusion: the group of responders in comparison with the APT- patients group indicated more safe clinical-hemodynamic and functional status, and neurohumoral rates in the responders group were closer to the outcome in the control group.

Purpose: to assess the dynamics of the seasonal risk of death cases in metropolis on example of Baku city. Materials: medical certificates of all death cases registered in Baku during 2013 year (totally 12241, including 7971 cases connected diseases of the cir-culatory system). Methods: descriptive statistics of the average daily death cases, analysis methods of quality share signs of the circulatory system diseases amoung causes of death, correlation and regression of the relationship between with the possibility of death and the seasonal dynamics. Results: average monthly cases of death from all reasons and from BCSD made respectively: 1020±34,2 and 664,3±28,3. BCSD share among causes of death in a year made 65,1±0,4%. Average daily quantities of death cases from all reasons and from BCSD respectively made: 33,5±1,24 and 21,8±1,04. Higher than a median (33,4) average daily cases of death from all reasons were observed in February (43,0), March (37,2), April (34,9) and November (36,5). Above a median average daily cases of death owing to BCSD (20,0) were noted in February (29,3), March (24.3) , April (26,6), June (25,5), October (20,1) and November (20.4) . Average daily cases of death from all reasons for winter and spring period (35,8±1,57) statistically significantly (Р <;0,05) differed from average daily cases of death from all reasons for the summer-autumn period (31,3±1,49). The average daily quantity of death cases owing to BCSD was maximum in spring, and is minimum in fall (respectively: 23,6± 1,2 and 19,8±0,4; Р<0,05). It is obvious that the risk of mortality owing to BCSD is more in winter and spring period, especially in spring. The final result: despite the fact that the climate of Baku city is relatively mild, increasing of death risks in the winter and spring seasons are common.

Objectives. To define the presence and severity of anxiety and depression stress and assess the impact produced on the life quality in subjects with CHF secondary to registered macrofocal (Q wave) myocardial infarction in medical history. Material and methods. General clinical examinations, psychological testing. Results. A total of 118 patients with II, III FC NYHA chronic heart failure were examined. Anxiety and depression events were defined in 46.6% of cases. As heart failure progresses, anxiety and depression symptoms increase, with the prevalence of anxiety in the presence of FC II and depression in the presence of FC III. Life quality in patients with concomitant anxiety and depression stress is significantly worse compared to patients with normal psychoemotional state. Conclusion. Thus, individuals with CHF showed psychoemotional changes including anxiety and depression stress, which may adversely affect CHF course. Anxiety and depression symptoms increase as heart failure progresses.

This article reviews the possibility of evaluating intracardiac intervals using two-dimensional echocardiography and tissue Doppler study of the heart in predicting the likelihood of atrial fibrillation. It is shown that the determination of the duration of the aforementioned interval is sufficiently informative method of assessment of inter - and intra-arterial conductivity, which in turn makes it possible to determine the risk of new cases of arrhythmia or progression of paroxysmal atrial fibrillation in chronic form.

The aim. The study and analysis of cardiac arrhythmias, heart rhythm variability in patients with ischemic heart disease, obstructive sleep apnea and primary snoring. Material and methods. 65 patients aged 40-68 with ischemic heart disease, obstructive sleep apnea and primary snoring have been included in research. Results. Obstructive sleep apnea and primary snoring leads to an increase in sympathetic and parasympathetic activity of autonomic nervous system on sinus node in patients with ischemic heart disease, obstructive sleep apnea and primary snoring.

Purpose: to assess the non-performance rate of primary PCI in STEMI patients and determine the factors influencing the choice of approach for the management of patients based on Kemerovo Cardiology Clinic's experience in 2012. Material and Methods: 492 patients aged 30 to 92 years, admitted to the MBHI "Kemerovo Cardiology Dispensary" (the Regional Vascular Center in 2012 with a diagnosis of ST-segment elevation myocardial infarction) were included in the single-center prospective registry study. The mean age of patients was 64.42 ± 10.86 years. Results: over 20% of STEMI patients admitted to the Kemerovo Cardiology Dispensary did not undergo emergency coronary angiography (CAG). These patients were older, predominantly female (64%), commonly with repeated MI (44%) and with the presence of renal and myocardial dysfunction. The group of patients, who were not selected for CAG, had significant patient delay between onset of symptoms and hospital admission (9 hours from onset of symptoms to hospital admission in the group of patients who were selected to emergency CAG and 30 hours - in patients who were not selected for CAG). Conclusion: according to the results of the study, we determined a group of patients (up to 20%), who did not receive high-tech medical services because of time delay and had less favorable outcomes after STEMI. Therefore, the development of algorithms is particularly important as they can reduce the time delay in the pre-hospital period, as well as active patient selection for coronary angiography during the in-hospital period.

Aim: to study hypotensive and organo-protective efficacy of cornbination therapy with perindopril and indanarnide in patients with rnild and rnoderate arterial hypertension. Material and methods. According to the protocol in the study included 23 patients with I-II grade of arterial hypertension (ESC/ ESH 2013), of both genders. All patients took the cornbination preparation Arnra forte (GMP, Georgia), containing 4 тд of perindopril and 1.25 rrig of indaparnide. At the stage before the treatrnent and 12 weeks of therapy was rneasured an office blood pressure by Korotkov's rnethod and carried out arnbulatory blood pressure rnonitoring (ABPM) ("BR-102 plus Recorder" (SCHILLER, Switzerland)). Antirernodeling efficacy was assessed by the dynarnics of cardiac echo geornetry and ultrasound (US) of vessels pararneters. Also was evaluated the clinical tolerance and rnetabolic effects of the drug. The pararneters of central hernodynarnics and vascular stiffness were rneasured by applanation tonornetry. Results are presented as M±SD. Results of research. 12-week cornbination therapy with perindopril and indaparnide is characterized by high antihypertensive efficacy, with a decrease in rnean blood pressure to 18%, and achievernent the target level of blood pressure in 100% patients with rnild and rnoderate arterial hypertension. According to the ABPM showed a significant decrease of average daily SBP and DBP, as well as a significant decrease in the speed of rnorning SBP surge. Thus it was not iriarked episodes of hypotension at night. Reliable antirernodeling efficacy of perindopril and indaparnide is expressed in reducing LVMI, reducing of EDD and reliably reducing the thickness of IMC against the background of 12-week treatrnent. 12-week cornbination therapy with perindopril and indaparnide showed renal protection efficiency, expressing in a significant decrease in blood creatinine level and alrnost twofold reduction of MAU level. Perindopril and indaparnide in the rnode of cornbination therapy in patients with rnild and rnoderate arterial hypertension showed rnetabolic neutrality in relation to lipid and carbohydrate metabolism, clinical safety. A side effect as dry cough was observed in one patient only at the end of 12-weeks treatrnent (4.3%). Conclusion. Therefore, our research showed a high antihypertensive efficacy of cornbination therapy with perindopril and indaparnide with the available cardio-, vaso-, and nefroprotection, rnetabolic neutrality and clinical safety that recommends the preparation for the treatrnent of patients with rnild and moderate arterial hypertension.

Article retracted 11.11.2019 г.

Hypolipidemic activity of statins is realized by inhibition of the alcohol cholesterol (CL) local pool production in hepatocyte endoplasmic reticulum. Before secretion of very low density lipoproteins (VLDL) into hydrophilic medium of the blood, CL covers the total hydrophobic mass of triglycerides (TG). The smaller the CL content in the monolayer between the enzyme (lipase) and substrate (TG), the higher the parameters of hydrolysis of palmitic and oleic VLDL. Statins act as follows: а) block hepatocyte production and decrease plasma content of nonesterified CL; b) activate TG hydrolysis in palmitic and oleic VLDL, formation of ligand VLDL and their uptake by insulin-dependent cells via apoE/B-100 endocytosis; c) activate TG hydrolysis in linolic and linolenic low density VLDL, formation of ligand low density lipoproteins (LDL) and their uptake by apoB-100 endocytosis; d) reduce blood content of equimolary esterified by the alcohol CL polyenic fatty acids, CL esters and CL-VLDL. Nonphysiological effect of impaired function of trophology (nutrition) on fatty acid (FA) metabolism in a population cannot be abolished by prescribing medicines. For lowering cardiovascular morbidity it is necessary to modify environmental factors, i.e., reduce dietary content of saturated FA (primarily of palmitic), trans-FA and palmitoleic FA to physiological levels and increase dietary content of unsaturated FA. Saturated FA block cellular uptake of unsaturated FA. Deficiency of unsaturated FA and excess of palmitic FA lead to the development of atherosclerosis.