Aim: assess of the pulmonary artery growth and the outcomes of complete repair after palliative treatment in infants with critical form of Tetralogy of Fallot.

Methods. This was prospective randomized two-center study (2019 to 2022). Its included infants with Tetralogy of Fallot who underwent palliation with either stenting of the right ventricular outflow tract (stent group, n=21) or modified Blalock-Taussig shunt (shunt group, n=21).

Results. In the stent group Nakata index increase from median 104.2 to 208.6 mm²/m², while in the shunt group, it increased from 107.3 to 169.4 mm²/m² (p<0,01). Mixed model analysis showed that in stent group the right pulmonary artery growth rate was 2.05*10^-2 z scores/day, which was 3.01 times higher than in the shunt group. In stent group the left pulmonary artery growth rate was 2.3*10^-2 z scores/day, which was 1.47 times higher than in the shunt group. In the stent group, there was one noncardiac-related mortality during the intermediate period. Transannular patch repair of the right ventricular outflow tract was performed in 12 patients (60%) in the stent group and in 15 patients (71.4%) in the shunt group (p=0.52) during complete repair. At 8 cases (40%) in the stent group and 6 cases (28.6%) in the shunt group, pulmonary artery replacement was performed. Time to surgical repair was shorter in the stent group (p=0.046), while the aortic cross clamp time (p<0,01) and cardiopulmonary bypass time (p<0,01) were significantly shorter in the shunt group.

Conclusions. Right ventricular outflow tract stenting provides hemodynamic stabilization and uniform growth of the pulmonary artery compared to modified Blalock-Taussig shunt.

Aim. To study the dynamics of social and labor status in patients with chronic thromboembolic pulmonary hypertension (CTEPH) 6 months after pulmonary endarterectomy (PEA) and to identify clinical factors affecting the resumption of labor activity in the late postoperative period.

Material and methods. The study included 79 patients with CTEPH scheduled for PEA. The average age was 48.6 (39.3-59.4) years. We analyzed clinical and functional data, as well as social and labor status before and 6 months after PEA. Using multivariate regression analysis, the factors influencing the resumption of labor activity after PEA, were studied.

Results. In the general cohort of patients with CTEPH, 6 months after surgery, a slight increase in the proportion of working patients of all ages was revealed - from 55.7% to 65.8% (p=0.19). In the subgroup of patients of working age (61 people), the proportion of working patients in dynamics increased significantly – from 67.2 to 85.2% (p=0.03). Multivariate regression analysis showed a protective effect of the 2nd stage of rehabilitation on social and labor status after PEA – OR 4.2 (2.9-8.3 95%CI) (p=0.01). The presence of atrial fibrillation and residual pulmonary hypertension negatively affected the resumption of labor activity in the long-term period after PEA – OR 0.6 (0.3-0.9 95%CI) (p = 0.03) and OR 0.7 (0.2-0.8 95%CI) (p=0.01) respectively.

Conclusion. The dynamics of the social and labor status of patients with CTEPH 6 months after PEA is characterized by a significant increase in the proportion of working patients of working age from 67.2 to 85.2% compared with the preoperative level. The leading protective factor for the renewal of labor potential in patients with CTEPH within 6 months after PEE is the underwenting of the 2nd stage of rehabilitation. Residual pulmonary hypertension and atrial fibrillation are limiting factors for resuming of labor activity.

Aim. To evaluate the antihypertensive therapy and adherence to treatment in patients with uncontrolled arterial hypertension.

Materials and Мethods. The study included 297 patients hospitalized at the E.I. Chazov National Medical Research Center of Cardiology over the period from September 2019 to March 2022. Patients were spread into two groups depending on the increase in the frequency of BP: group 1 (n=149) – increase in BP above 140/90 mm Hg 1 or more per week while taking antihypertensive therapy with clinical manifestations, and group 2 (n=148) – increase in blood pressure more than 140/90 mm Hg less than 1 per week while taking antihypertensive therapy clinical manifestations. Antihypertensive therapy and medication adherence were assessed at hospital discharge and after 12 months.

Results. Initially, the number of antihypertensive drugs was greater in patients with group 1 versus the patients with group 2 (p<0,001). The frequency of taking antihypertensive therapy initially and after 12 months was greater in group 1 (p<0,001). After 12 months, the number of antihypertensive drugs decreased in the group 1, and remained the same in group 2. When comparing the different classes of antihypertensive drugs, all classes, except angiotensin-converting enzyme inhibitors were most frequently prescribed in group 1 (p<0,05). After 12 months, a lower level of adherence was observed in group 1 patients compared to group 2 (6 [5-8] points vs. 8 [6-9] points) (p<0,001), as evidenced in the reduced intake of different classes of antihypertensive therapy.

Conclusion. Uncontrolled arterial hypertension is a release of hypertension associated with more antihypertensive medications to be prescribed and adherence to be monitored more closely.

Aim. To determine the reproducibility of the results of instantaneous wavefree ratio (iFR) and angiography coregistration in patients with coronary heart disease (CAD) with multilevel and diffuse lesions of the coronary arteries.

Materials and methods. The study included 34 patients with chronic ischemic heart disease (CAD) with diffuse and/or multilevel coronary stenoses >50%. In 41 coronary arteries, two consecutive iFR pullback of the conductor were performed and paired iFR angio-coregistrations were formed, reflecting changes in iFR gradients (Δ iFR) along the entire length of the studied artery. To compare the results of paired studies, the following values were used: 1) indices of distal iFR; 2) maximum Δ iFR; 3) Δ iFR of all stenoses ˃50%.

Results. Comparable values of distal iFR were obtained 0,84 [0,78; 0,89] vs. 0,85 [0,77; 0,88]) with an average difference of the first and second measurements of 0,001, maximum ΔiFR (0,08 [0,05; 0,14] vs. 0,08 [0,05; 0,13]) with a mean difference of -0,004 and ΔiFR of all stenoses >50% (0,05 [0,02; 0,11] vs. 0,05 [ 0,02; 0,10]) with an average difference of -0.001. Lin’s concordance correlation coefficient and Intraclass Correlation Coefficients calculated for the three parameters studied showed a good level of agreement (0,97; 0,98; 0,98) with excellent reliability (0,97; 0,98; 0,97).

Conclusion. In patients with multilevel and diffuse lesions of the coronary artery, iFR angio-coregistration has a fairly high reproducibility.

Aim: to study the influence of riociguat on the functional and hemodynamic status, remodeling of the right heart, as well as the safety of therapy in both previously untreated patients with idiopathic pulmonary arterial hypertension (IPAH) and inoperable chronic thromboembolic PH (CTEPH), and those not achieved treatment goals with sildenafil therapy and switching to riociguat.

Materials and methods. A total of 161 pts with precapillary PH were included in the study; 137 pts completed the three-year observation period. Of 55 IPAH pts riociguat was started after diagnosis verification in 39 pts (subgroup 1); 16 pts previously taking sildenafil who did not achieve treatment goals comprised subgroup 2 of switching to riociguat. Of 82 inoperable CTEPH pts riociguat was started in 45 naїve pts; a switching strategy riociguat was implemented in 37 pts after 24-hour withdrawal of sildenafil. The dose titration of riociguat was started from 1 mg TID according to the standard algorithm up to 7.5 mg/day. By month 36 92.4% and 94.8% of pts with IPAH and CTEPH, respectively, received 7.5 mg/day. At baseline, at month 12, 24 and 36 all pts underwent a 6-minute walking test (6MWT) with the assessment of the dyspnea index according to the Borg scale and SpO₂, echocardiography (Echo), right heart catheterization (RHC), and the safety profile was assessed.

Results. At baseline, the proportion of pts with FC III-IV in CTEPH group compared to IPAH group, was significantly higher (70.7% vs 41.8%)); the distance in T6MX (d6MWT) was 291 [232;385] m vs 379 [300;448] m (p<0.001). CTEPH pts had lower values of sPAP, PVR, SaO2 and SvO2 assessed by RHC. In the switching subgroups 2 of pts with IPAH and CTEPH, achieved levels of sPAP (p=0.01), sRAP (p=0.001) and PVR (p=0.01) (RHC) were significantly lower than in subgroups 1. The baseline levels of CI and SV were significantly higher in subgroups 2 (p<0.05). During riociguat treatment in both subgroups of IPAH, a significant increase in d6MWT was achieved by 6 months with FC I-II (WHO) in 75% and 70% of patients. In the subgroup 1 to 36 months the greatest increase by 97m was achieved; in the switching subgroup the increase in d6MWT was noticed by month 6, which was maintained by month 36. In CTEPH patients, there was a significant improvement in FC (p=0.001) with ∆dT6MX 48m (p=0.001). In subgroup 1 with CTEPH, the d6MWT increase was 68.2m (p = 0.001), reaching 82.6m by month 36. In subgroup 2 the significant change of d6MWT were noted by the 1st year of FU, reaching 136.6m by month 36. In IPAH and CTEPH pts by month 36 of riociguat therapy, a significant decrease of mPAP, SPAP were found by echo, which was confirmed by RHC; there was a decrease in the basal size of the right ventricle (RV) (p=0.04) and an increase in RV FAC (p=0.04 and p=0.03, respectively). In subgroups 1, ∆SvO₂ in ts with IPAH and CTEPH were significantly higher compared to subgroups 2. During FU period the proportion of low-risk pts increased to 26.7% in IPAH group and 44.8% in CTEPH group at month 36. In subgroups 2 riociguat therapy resulted in maintenance of treatment regimens throughout the year. Only 6.2% of pts by month 24 and 36 required the prescription of a 3d specific drug. No serious adverse events (AEs) were observed during treatment. The most common AEs were nasopharyngitis, nasal congestion, and dyspnea.

Conclusions: Riociguat therapy for 36 months demonstrated a persistent positive effect on the functional and hemodynamic status, remodeling of the right heart both in previously untreated patients with IPH and inoperable CTEPH, and in patients from the switching groups who did not achieve treatment goals with sildenafil therapy.

Aim: to assess the effectiveness of PAH-specific therapy, including selexipag, based on data from a comprehensive examination and assessment of oxygen transport function in patients with IPAH using Raman spectroscopy.

Materials and methods. The study was performed on fourteen (n=14) patients diagnosed with IPAH who were on combination PAH-specific therapy, including selexipag. The diagnosis was established in accordance with the algorithm proposed in the Russian guidelines for the diagnosis and treatment of PAH (2020). A comparative analysis of clinical and hemodynamic parameters, including an assessment of hemoglobin conformation using Raman spectroscopy, was performed in all participants.

Results. The use of Selexipag as part of a combined PAH-specific therapy caused significant changes in the conformation of Hb including a decrease in the probability of a “dome-shaped” heme conformation due to changes in the group vibrations of heme pyrrole groups (I₁₃₇₅^/I₁₁₇₂) and stretching vibrations of vinyl groups (I₁₅₈₀/I₁₅₅₀), as well as an increase globin density.

Conclusion. The use of the prostacyclin receptor agonist selexipag as part of a combination PAH-specific therapy for patients with IPH was associated with improvement in clinical, functional and molecular parameters (taking into account the assessment of oxygen transport function and hemoglobin conformation by Raman spectroscopy).

The course of heart failure, regardless of its etiology, is associated with local and systemic activation of inflammatory signaling cascades. Identification of the association of candidate cytokine genes with the risk of developing coronary heart disease and chronic heart failure remains a subject of interest for many researchers. The review presents an analysis of studies of the rs1143634 polymorphism of the IL-β gene in relation to the risk of atherosclerotic cardiovascular complications. The presence of the minor allele T is associated with an increase in the concentration of IL-1β in the blood serum. Data on the role of the rs1143634 polymorphism of the IL-β gene in atherosclerotic coronary heart disease, including in combination with chronic heart failure syndrome, are presented, taking into account ethnic characteristics and gender of patients. The carriage of the T allele was associated with the risk of developing atherosclerosis and myocardial infarction. At the same time, it is reported that the C allele and the CC genotype of the rs1143634 polymorphism of the IL-1β gene were a genetic risk factor for the development and progression of heart failure. In a number of studies and meta-analyses, the association of this polymorphism with diseases of the cardiovascular system was not confirmed, but certain trajectories of the risk of complications were identified due to differences in the rs1143634 polymorphism of the IL-β gene in heart failure.

Transcatheter aortic valve implantation (TAVI) is commonly accepted method of treatment of aortic stenosis in most patients with currently increasing number of TAVI procedures. Patients after TAVI frequently have indications for percutaneous coronary intervention (PCI) that might be technically challenging due to the presence of transcatheter valve. Compromising coronary ostia involves such issues as design of the prosthesis and its position in the aortic root with respect to the commissures of native aortic valve. Since TAVI with commissural alignment can mitigate risk of compromising coronary ostia, this option should be considered while planning TAVI, especially in younger patients with coronary atherosclerosis and relatively high probability of PCI in the future. The algorithm of commissural alignment in clinical practice of TAVI up to date has been proposed only for several transcatheter heart valves reported in this paper.

Uncovering the secrets of genome flexibility not only contributed to the development of research in this area, but also served as an impetus for the development of new treatments for human diseases. A better understanding of the biology of chromatin (DNA/histone complexes) and non-coding RNAs (ncRNAs) has enabled the development of epigenetic (epi) preparations capable of modulating transcriptional programs associated with cardiovascular disease. This is especially true in heart failure, where epigenetic mechanisms have been shown to underlie the development of several pathological processes such as left ventricular hypertrophy, fibrosis, cardiomyocyte apoptosis, and microvascular dysfunction. Targeting epigenetic signals may represent a promising approach, especially in patients with heart failure with preserved ejection fraction (HFpEF), where the prognosis remains poor and effective treatments are not yet available. Under these conditions, epigenetics can be used to develop individualized therapeutic approaches, paving the way for personalized medicine. Although the beneficial effects of epi-drugs are gaining more attention, the number of epigenetic compounds used in clinical practice remains low, suggesting the need to develop more selective epi-drugs. In this review, we present a list of new promising epi-drugs for the treatment of cardiovascular diseases, with a focus mainly on HFpEF. The therapeutic effect of these drugs is due to the impact on at least one of the three main epigenetic mechanisms: DNA methylation, histone modification, and non-coding RNA.

Presented clinical case report illustrates nonstandart approach use repeat thrombolytic therapy patient 49 y.o. with acute circular – apical myocardial infarction, after noneffective primary systemic thrombolytic therapy rtPA. Moreover, repeat thrombolytic therapy in hospital stage was vital alternative in case with significant tamely delay mechanical reperfusion. After 90 minutes then introduction rtPA has been done, we got negative dynamic on ECG in the form of additional ST-segment elevation at other (early intact) lead, and reccurent anginous pain refractory to standard doses narcotic analgesic. In connection with remote PCI – center, we supposed significant tamely deIlay to mechanical reperfusion. In view of this factors, high risk to undesired event, refractory anginous pain, we made decision of repeat thrombolytic therapy with non-immunogenic staphilokinase, and it has been effective. At a later time the disease is taking it’s normal course.

Caseous calcification of the mitral annulus (CCMA) is a rare form of degenerative lesion of the mitral valve fibrous ring. It’s a biochemical transformation of calcification area accompanied with formation of masses of a curdled consistency (caseosis). It is usually located in the area of the posterior mitral valve. In most cases, it is asymptomatic or minimally symptomatic, and is an incidental finding during imaging studies. The causes and pathogenesis of CCMA are unknown, however, there is a connection with disorders of calcium and phosphorus metabolism and there is no connection with an valve infection. There is no specific treatment. In cases where CCMA leads to significant disturbances of cardiac hemodynamics and/or there is a high probability of ebolism, it needs to be treated surgically.

Differential diagnosis is carried out with other cardiac mass.