Aim. To study the clinical manifestations, incidence of life-threatening complications, and their possible mechanisms and outcomes of left ventricular non-compaction (LVNC) in adults.
Material and methods. This study included 125 adult patients with LVNC, 74 men (59.2%) and 51 women (40.8%) aged 46.4±15.1 years. Echocardiography (EchoCG) (n=125), Holter monitoring (n=125), cardiac magnetic resonance imaging (MRI) (n=60), and contrast-enhanced multislice computed tomography (MSCT) of the heart (n=90), and, if indicated, coronary angiography (CAG) (n=33) and myocardial scintigraphy (n=27) were performed. The diagnosis of LVNC was confirmed in 74 cases using two methods, and in 21 cases, using three imaging methods. DNA diagnostics was performed in most patients. For most patients, the level of anticardiac antibodies and the genome of cardiotropic viruses were determined in the blood. Mean left ventricular (LV) ejection fraction (EF) was 38.6±14.0%; LV end-diastolic volume (EDV) was 158.1±67.8 ml; LV end-diastolic dimension (EDD) was 6.1±0.9 cm; and left atrial (LA) volume was 97.1±38.1 ml. The mean follow-up period was 14 months [4.0; 41.0]; from 1 month to 10 years.
Results. Death rate was 14.4%; heart transplantation was performed in 5.6% of cases. Nonsustained ventricular tachycardia (VT) was detected in 45.6% of cases and sustained VT in 13.6%. The presence of VT was associated with poor R-wave progression in the precordial ECG leads, low QRS voltage, QRS duration >105 ms, NYHA chronic heart failure functional class (CHF FC) ≥2-3, LV EF <40%; LV EDD >6.1 cm, the presence of myocarditis, and higher death rate. Cardioverter defibrillators, including cardiac resynchronization therapy defibrillators (CRTD), were implanted in 38 patients. Appropriate defibrillator shocks were associated with frequent premature ventricular contractions (PVCs). The incidence of thrombosis and embolism was 22.4%. Their predictors included CHF FC ≥2-3, RV anteroposterior dimension >3.1 cm, LA volume >98 ml, E/A >1.65, LV EDD >6.3 cm, LV EDV >153 ml, LV EF <35%, and myocardial necrosis of unknown origin (in patients without coronary atherosclerosis). The incidence of myocardial necrosis in LVNC was 16.0%. The mechanisms identified, in addition to coronary atherosclerosis, were embolism in unchanged coronary arteries, secondary myocarditis, and the presence of genetically determined thrombophilia.
Conclusion. LVNC is associated with a high risk of life-threatening conditions, such as ventricular arrhythmias, thrombosis and embolism, and myocardial necrosis, that are typical complications of LVNC in adults. Reassessing the predictors for the risk of thromboembolic and arrhythmic events, specifying the indications for implantable cardioverter defibrillator and anticoagulants, and actively identifying and treating concomitant myocarditis are essential.

Aim. The aim of this study was to evaluate the clinical and cost-effectiveness of computed tomography angiography (CTA), which includes CT coronary angiography and a “triple rule-out” protocol, in intermediate-risk patients with suspected non-ST-segment elevation acute coronary syndrome (NSTEACS) in the emergency room (ER) of the regional vascular center in the structure of a multidisciplinary hospital in Moscow.
Material and methods. This continuous single-site study included patients hospitalized in a multidisciplinary hospital with a referral diagnosis of NSTEACS within 69 days. Patients at intermediate risk who met the inclusion criteria underwent CTA after the initial examination in the ER. If coronary artery disease or an alternative significant diagnosis was excluded, patients were discharged from the hospital on the day of admission. As a comparison method, the costs of treating these patients were assessed if a standard protocol was used. According to this protocol, patients, after the initial examination, were hospitalized in the intensive care unit for patients with myocardial infarction (ICU-MI) and then in the cardiology department for observation and further examination. Clinical economic analysis was performed using the cost minimization method and the tariff method of cost estimation.
Results. For 69 days, 289 patients (59.5% men, mean age 71.7±8.6 years) were admitted to the ER with a referral diagnosis of NSTEACS. In 30 of them, a non-cardiological disease was identified that required routing to other specialized units. 37 (14.3%) of intermediate-risk patients underwent CTA. In 27 of them (10% of all patients), no significant coronary stenosis, signs of pulmonary embolism (PE), or aortic dissection were detected, and the patients were discharged from the ER. 10 patients (4% of all patients) who had significant coronary artery stenoses, PE, or aortic dissection were hospitalized. 72 intermediate-risk patients had exclusion criteria for CTA. The economic benefit from using CTA for excluding ACS in the ER, as compared to the standard approach, was 1,602,450 rubles for the study period. The estimated benefit per year was 8,476,728 rubles.
Conclusion. The introduction of CTA and the “triple rule-out” protocol for intermediate-risk patients in the ER can significantly improve the process of excluding the diagnosis of NSTEACS, reduce the number of unnecessary hospitalizations and optimize the use of hospital capacity. According to the results of our study, this approach is applicable in at least 14% of patients with suspected NSTEACS (at least 33% of intermediate-risk patients).

Aim. To evaluate the efficacy, safety and adherence to therapy with a fixed combination of bisoprolol/perindopril in patients with arterial hypertension (AH), stable ischemic heart disease (IHD), and a history of myocardial infarction (MI) in clinical practice.

Material and methods. For patients with AH and concomitant stable angina and a history of MI, the treatment with beta-blockers and renin-angiotensin-aldosterone blockers is recommended as a part of therapy to reduce the risk of death from cardiovascular complications. This study included 504 patients. At baseline, systolic and diastolic blood pressure (SBP and DBP) and heart rate (HR) were 148.9±16.7 mm Hg, 87.7±11.0 mm Hg, and 77.4±10.5 beats/min, respectively. PRIDE was a multicenter, observational, ambispective study that included patients with hypertension, stable angina and a history of myocardial infarction, taking a fixed combination of bisoprolol/perindopril. The prospective observation period was 12 weeks from the date of inclusion in the study. The primary endpoint was a change in SBP and DBP at the end of the observation. Additional parameters of antihypertensive and antianginal efficacy and adherence to therapy were assessed as secondary variables. For exploratory purposes, an analysis was performed to evaluate predictors of achieving the target values of BP and HR, as well as adherence to therapy, including a fixed combination of bisoprolol/perindopril.

Results. The antihypertensive effect that was observed by the 12th week of observation was evident as a decrease in SBP and DBP by 24.9/12.2 mm Hg (p<0.001). The proportions of patients with low, moderate, or high adherence to treatment at 12 weeks were 21.7% (n=94), 25.3% (n=110), and 53.0% (n=230), respectively. The presence of grade 3 AH was a negative predictor for achieving the target BP <140/90 mmHg (odds ratio, OR, 0.11; 95% confidence interval: 0.01-0.64). Functional class III angina in the general population, patients younger than 65 years, and female patients, and grade 2 and 3 AH in male patients were factors associated with a lower likelihood of high adherence. In women, the presence of type 2 diabetes mellitus was a positive predictor for adherence to therapy. Despite the fact that 3.7% (n=18) of patients included in the study had a decrease in SBP below 120 mm Hg by week 12, no adverse events associated with such a decrease was noted, and the therapy was well tolerated by the patients.

Conclusion. Treatment of patients with AH in combination with stable IHD and a history of MI with a fixed combination of bisoprolol/perindopril was associated with significant antihypertensive efficacy and improved adherence to the prescribed treatment. The presence of grade 3 AH was associated with a significant decrease in the likelihood of achieving the BP goal, while grades 2 and 3 AH and functional class III angina negatively influenced the likelihood of high adherence to therapy.

Aim. To evaluate the efficacy and safety of intracoronary epinephrine for the treatment of refractory no-reflow phenomenon in patients with ST-segment elevation myocardial infarction (STEMI) during percutaneous coronary intervention (PCI).
Material and methods. A single-site prospective controlled study “Intracoronary administration of epinephrine for refractory no-reflow phenomenon in patients with acute myocardial infarction” was conducted (registration on ClinicalTrials.gov: NCT04573751). The study included 40 patients with refractory no-reflow phenomenon, which was identified when it was not resolved with at least one of the following means: nitroglycerin, adenosine, papaverine, platelet receptor inhibitors IIB/IIIA, or thromboaspiration. Patients were divided into 2 groups: patients of group 1 (n=18) were injected with intracoronary epinephrine 100 μg, patients of group 2 (n=22) received standard therapy without epinephrine. The groups did not differ in the main baseline clinical and anamnestic characteristics, with the exception of the predominance of men in the control group: 86.4% vs. 55.6% (p=0.03). 
Results. In the epinephrine group, TIMI 3 blood flow was more often achieved: 55.6% vs. 0% (p<0.01); reduction in ST elevation >50% within 1 hour after PCI: 72.2% vs. 31.8% (p=0.01). Concentrations of troponin I 12-24 h after PCI were significantly lower in the epinephrine group than in the control group: 15.2 (6;25) ng/ml vs. 25 (10;40) ng/ml (p=0.03). No life-threatening hemodynamic disorders or cardiac arrhythmias were recorded after the administration of epinephrine. No statistically significant differences were found in cardiac ultrasound data and MACE (Major Adverse Cardiovascular Events) during 30 days of follow-up.
Conclusions. Intracoronary epinephrine 100 μg in STEMI patients with refractory no-reflow phenomenon during PCI is a safe and effective method for improving the blood flow in the infarct-related coronary artery. The prevalence of refractory no-reflow phenomenon among STEMI patients in our study reached 4.6%.

Aim. To study the association of the rs386000 polymorphic variant in the LILRA3 gene with the risk of developing obliterating atherosclerosis of the lower extremity arteries (OALEA).

Material and methods. 1277 individuals of Slavic origin were examined (629 patients with OALEA and 648 healthy volunteers). Genotyping of the LILRA3 gene rs386000 was performed with a MassARRAY-4 genomic mass spectrometer. Polymorphic variants of the LILRA3 gene, that encodes the leukocyte immunoglobulin-like receptor A3, may be attractive objects for studying the mechanisms of atherosclerosis.

Results. The study showed that the rs386000 polymorphic variant in the LILRA3 gene was associated with the risk of developing OALEA. However, this association was characterized by sexual dimorphism: in men, carriage of the rs386000-C allele (p=0.03) and the rs386000-C/C genotype (p=0.01) was protective against the risk of developing OALEA, while in women, this polymorphism did not influence the susceptibility to the disease. Single nucleotide polymorphism (SNP) annotation showed that carriage of the rs386000‑C allele was associated with an increased expression of the LILRA2, LILRB5, LILRA6, LILRP1 and TSEN34 genes and a decreased expression of the LILRA3 and LILRA5 genes in the blood.

Conclusion. The present study revealed for the first time an association of the rs386000‑C allele of the LILRA3 gene with a reduced risk of developing OALEA. Further studies, including experimental studies, will determine the specific mechanisms mediating the involvement of the LILRA3 gene rs386000 polymorphism in the molecular mechanisms for the development of obliterating atherosclerosis, as well as the nature of the sex-specific association of the polymorphism.

Aim. To compare the antiarrhythmic activity and safety of two Russian-made drugs, Laporitmin (PharmVILAR) and Allapinin (Pharmcenter VILAR) in premature ventricular contractions (PVCs) in patients without structural heart pathology.

Material and methods. The study included 100 subjects divided into 2 groups; the follow-up period was 3 weeks. The drug efficacy was assessed based on the results of ECG monitoring and Holter ECG monitoring (ECG-HM).

Results. A comparative analysis of the antiarrhythmic activity and safety of the drugs containing lappaconitine hydrobromide showed a comparably high clinical efficacy and good tolerability of the original drug Allapinin and the generic drug Laporitmin for PVCs. According to the results of ECG monitoring, the number of PVCs significantly decreased from 2 (2-6) to 0 (0-1) in patients of the main group (Laporitmin treatment group) and from 2 (2-7) to 0 (0-2) in patients of the control group (Allapinin treatment group) (p>0.05); according to the ECG-HM data, the number of PVCs significantly decreased by 88.2 Δ% in the main group and by 87.5 Δ% in the control group (p <0.01). There were no statistically significant differences in the primary effectiveness criterion between the main and control groups (F statistic = 1.41; p = 0.23).

Conclusion. The comparative analysis of the original drug Allapinin and the generic drug Laporitmin for PVCs in patients without structural heart pathology who had indications for antiarrhythmic therapy did not detect statistically significant differences in the primary criterion of effectiveness. Therapy with either drug showed a comparably high efficacy in reducing the number of PVCs by more than 75∆%, which was established as a criterion for primary effectiveness in the clinical trial protocol. Both drugs were well tolerated.

Aim. To assess the effect of the availability of specialized, including high-tech, medical care for patients with ischemic heart disease (IHD) on mortality from IHD in the Russian Federation.

Material and methods. To achieve the predetermined goal, we used cointegration of time series characterizing the mortality from IHD and the availability of specialized, including high-tech, medical care for patients with IHD in the Russian Federation for the period from 2015 to 2021: availability of cardiac beds; availability of cardiac surgery beds; availability of beds in regional vascular centers (RVC); availability of beds in primary vascular departments (PVD); availability of cardiologists in polyclinics; availability of cardiologists in hospitals; availability of cardiovascular surgeons (CVS) in hospitals; availability of interventional radiologists for endovascular diagnosis and treatment (EVDT) in hospitals; availability of therapeutic procedures of percutaneous coronary intervention (PCI) in acute coronary syndrome (ACS); availability of elective PCI procedures; availability of coronary artery bypass grafting (CABG).

Results. Cointegration tests showed a relationship between IHD mortality and the availability of beds at RVCs and PVDs and of CABG operations. Furthermore, an increase in the availability of RVC beds by 1 unit in each period results in a decrease in IHD mortality by 22.8 per 100,000 population during the year; an increase in the availability of PVD beds by 1 unit in each period results in a decrease in IHD mortality by 64.4 per 100,000 population during 2 years; and an increase in the availability of CABG by 1 unit in each period of time results in a decrease in IHD mortality by 34.8 per 100,000 population during 2 years.

Conclusion. Thus, the most promising directions for concentrating healthcare resources to quickly reduce IHD mortality are the further deployment of a network of RVCs and PVDs, as well as increasing the number of CABG operations

Aim. Epicardial adipose tissue (EAT) is a layer between the myocardium and the epicardium, similar to the intra-abdominal adipose tissue. Many cardiovascular diseases have been associated with increased EAT. Limited proof exists that EAT contributes to ventricular extrasystoles (VES). In this study, we aimed to examine the role of EAT on VES.
Material and methods. 266 subjects were included in this prospective study between April 2022 and March 2023. They underwent a 12‑lead electrocardiogram, 24‑hour Holter monitoring, and echocardiography. The subjects were divided into two groups: the VES Group (n=134) (>60 VES / hr) and the non-VES Group (n=132) (<10 VES / hr) group. In addition, severe VES were defined as ≥10.000 VES / 24‑hr. EAT and other variables were compared between the non-VES and VES groups. Logistic regression analysis was performed to find the factors affecting VES, and an ROC analysis was used to determine the cut-off values of the variables.
Results. EAT was higher in the VES group (p<0.001). In pairwise comparisons, higher EAT in the VES group was independent of ventricular frequency (p=0.552). Variables affecting the presence of VES were left ventricular mass index (p=0.031), QT dispersion (p=0.010), and EAT (p<0.001). The EAT predicted the presence of VES at a cut-off value of 4.05 with a sensitivity of 54.5 % and a specificity of 81.3 %.
Conclusion. This research indicated that increased EAT might be an independent predictor of VES.

DMD is a gene located on X chromosome that is responsible for the formation of the dystrophin protein. Pathogenic variants in the DMD gene cause diseases such as Duchenne muscular dystrophy (DMD) and Becker muscular dystrophy (BMD). DMD is characterized by progressive muscle weakness, leading to loss of motor and respiratory functions, as well as cardiomyopathy and progressive heart failure due to the complete absence of dystrophin in the body. Patients with BMD synthesize a reduced amount of dystrophin, which distinguishes it from DMD by a milder clinical picture and an older age of onset. Cardiomyopathies are a common and, in some cases, the main manifestation of these pathologies. This review focuses on studies of diseases associated with dystrophinopathies, in which the main symptom is heart injury, cardiomyopathy, and also provides information about modern approaches to gene and targeted therapy for these diseases.