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Aim: To evaluate the long-term safety and efficacy of goflkicept treatment in patients with idiopathic recurrent pericarditis (IRP)., Materials and Methods: This report presents the interim analysis of an ongoing open-label extension (OLE) clinical trial of goflkicept in patients with IRP (NCT05673902), as a continuation of the core study (NCT04692766). The study assessed the frequency of pericarditis recurrence, time to recurrence after 12 and 60 weeks of goflkicept therapy, changes in C-reactive protein level, chest pain intensity, pericardial effusion size, and adverse events (AEs)., Results: All patients remained in clinical-laboratory remission during the 60 weeks of goflkicept treatment. The recurrence frequency was 31.3% (5/16) after 60 weeks and 90% (9/10) after 12 weeks of goflkicept treatment (p<0.001). A total of 64 AEs were reported in 16 patients (94.1%), mostly of mild to moderate severity. The most common AEs were infections, occurring in 11 patients (64.7%). Nine serious adverse events were reported in 5 patients, none of which were considered drug-related. There were no deaths., Conclusion: Long-term goflkicept therapy resulted in a significant reduction in the risk of recurrence and prolonged remission without an increase in adverse events.

This paper summarizes the data from updated international protocols and guidelines for diagnosis of transthyretin amyloid cardiomyopathy (ATTR-CM). The invasive and non-invasive diagnosis techniques and their combinations are briefly reviewed; the evidentiary foundations for each diagnostic option and tool are analyzed. The paper describes a customized algorithm for sequential diagnosis and differential diagnosis of patients with suspected ATTR-CM with allowance for the combination of clinical signs and diagnostic findings. Along with the awareness of primary care providers about the red flags of the disease and visualization criteria, as well as providing information to the patients about the possibility of performing therapy of ATTR amyloidosis and the risks of delayed diagnosis, the proposed algorithm enables timely patient routing and prescribing specific treatment.

Objective: the aim of the study was to characterize the mechanical properties of the pulmonary arterial wall (PA) in patients with pulmonary arterial hypertension (PAH) using magnetic resonance imaging (MRI) of the heart, and to determine their diagnostic and prognostic value., Materials and Methods: 57 patients with PAH were examined. The diagnosis of PAH was verified according to the recommendations of the ERS/ESC from 2015. All patients underwent a detailed echocardiographic (ECHO) study, MRI of the heart and right heart catheterization (RHC). To calculate the stiffness of the pulmonary artery wall, the MRI and RHC data were used., Results: We identified a correlation between the functional class of PAH and the parameters of hemodynamic, physical performance, ECHO parameters of the right chambers. There were no differences in the stiffness of the pulmonary artery wall, depending on the functional class of PAH. Among the six stiffness indicators, only pulsation index was associated with the structural and functional parameters of the right ventricle and pulmonary vascular resistance., Conclusion: The MRI pulsation index is the simpleststiffness index of the pulmonary artery wall and the most promising one for evaluating the prognosis of patients with PAH.

Aim: To provide a pharmacoeconomic estimate of macitentan versus bosentan in therapy for pulmonary arterial hypertension (PAH)., Subject and Methods: An analysis was carried out on the basis of a social perspective for patients, whose mean age was 50 years. A budget impact analysis was performed without discounting; with the time horizon of the study being 5 years. Assessing the cost- effectiveness of endothelin receptor antagonists used a Markov model based on the meta-analysis of clinical trials. The cost of bosentan was calculated from the 2016 registered prices with VAT. That of macitentan was estimated from the expected price of 170,000 rubles per 10-mg dose pack #28 if the drug is included in the List of Essential Medicines with VAT. The cost of sildenafil and iloprost was consistent with the January-to-November 2016 auctio., Results: At cost-effectiveness assessment costs and outcomes were both discounted at an annual rate of 3,5%., Results: After 5 years of therapy with macitentan in patients with baseline Functional Class (FC) II PAH, the proportion of patients with FC I-II was shown to be 2.6% more than that during therapy with bosentan (20.1 and 17.5%, respectively), and that of the died patients was 1.5% lower (69.5 and 71%, respectively). In baseline FC III PAH following 5 years, the proportion of patients with FC III PAH on initial macitentan treatment was 1% more than that on bosentan therapy (8.1 and 7.1%, respectively), and that of the died patients was 0.5% lower (87.2, and 87.7%, respectively). The cost-effectiveness analysis shows that therapy with macitentan versus bosentan not only causes some increase in life expectancy in terms of quality of life (by 0.414 and 0.230 QALYs in FC II and III PAH, respectively), but also results in a small cost decrease in FC II and III PAH (by 11,000 and 16,000 rubles per patient, respectively). Thus, macitentan is a dominant alternative versus bosentan. The budget impact analysis indicates that when bosentan is replaced with macitentan, the reduction in health care costs in the Russian Federation will amount to 1.9 million rubles over 5 years, and in all budgetary costs will be 14.7 million rubles., Conclusion: Treatment with macitentan in patients with FC II-III PAH is more cost-effective than that with bosentan and does not require an increase in budget costs.

Aim: To assess whether the serum levels of autoantibodies (auto-Abs) interacting with myocardial and vascular wall antigens might be used for the differential diagnosis of myocardial inflammatory diseases. Subjects and methods. Seventeen patients with morphologically documented myocarditis were examined. A comparison group comprised 9 patients with acute myocardial infarction (AMI). A control group included 18 apparently healthy individuals and 8 patients with chronic erosive gastritis (CEG). Serum auto-Abs levels were estimated by the ELI test systems standardized for enzyme immunoassay., Results: The individual profiles that reflect relative changes in the levels of 16 study auto-Abs and characterize the integral autoreactivity of a patient have clear intergroup differences. Using the profiles of auto-Abs could confirm the diagnosis of myocarditis in 94% of cases or assign the patient to a group of apparently healthy individuals. The informative value of the method in patients with AMI and CEG was 81.8 and 87.5%, respectively., Conclusion: The proposed method may be used for the noninvasive screening diagnosis of myocardial inflammatory diseases.

Aim: To elucidate whether endothelium-dependent vasodilation (EDVD) is a method of early diagnosis of endothelial dysfunction or is a sign of established morphological changes in the vascular wall., Material and Methods: Endothelialfunction was studied in 44 males with essential hypertension (EH) (mean age 46.9 +/- 0.9 years), in 52 patients with coronary heart disease (CHD) (mean age 50.0 +/- 0.9 years) after mammary-coronary and aortocoronary bypass operation with use of radial artery (RA), and in 28 healthy males (mean age 44.2 +/- 1.2 years). Intraoperative RA biopsy results for patients with ischemic heart disease after coronary artery bypass grafting with RA as an arterial graft were analysed., Results: Patients with CHD showed thicker complex intima-media (TIM) of the common carotid arteries compared to control and hypertensive patients free of CHD clinical symptoms. This is associated with lowering of EDVD in RA and, therefore, suggests systemic occurrence of endothelial dysfunction. The histological study of RA has found hyperplasia of the internal vascular intima of 1.1 +/- 0.2 degree in 97% of CHD patients. Combination of CHD with hypertension showed thicker intima, higher index of intima thickness and RA narrowing percentage vs such parameters in CHD free of hypertension., Conclusion: Impaired EDVD in the test with reactive hyperemia is due to morphological changes in the radial artery, its intima. The degree of changes in endothelial vascular reactions in RA is closely related to severity of systemic atherosclerosis. In CHD, RA has a marked intimal hyperplasia which leads to vasospasticity and may result in development of stenosis in its use as a graft for myocardial revascularization. One of the basic factors influencing the degree of intimal hyperplasia of the radial artery is severity of arterial hypertension.

Aim: To compare efficacy of long-acting nifedipin (cordipin-XL) and beta-blocker atenolol (tenormin) in hypertensive patients; to assess efficacy of antihypertensive treatment on functional activity of blood and endothelial cells, remodeling of the myocardium and vessels., Material and Methods: A total of 35 patients with essential hypertension stage II entered the trial. Eighteen of them received cordipin-XL, 17--tenormin., Results: Contrary to tenormin, cordipin-XL significantly improved blood rheology, reduced functional activity of leukocytes, concentration of Willebrand factor and intercellular adhesive molecules in blood plasm. Positive hemodynamic and non-hemodynamic effects of cordipin-XL were accompanied with reversion of left ventricular hypertrophy., Conclusion: Non-hemodynamic effects of cordipin-XL associated with improvement of endothelial function and blood rheological properties seem to have an independent role in prophylaxis of cardiovascular complications.