Amyloidosis is a systemic disease affecting not only the cardiovascular system. The paper discusses etiology, pathogenicity, features of clinical course of two types of amyloidosis AL- and ATTR. Data from updated international protocols and recommendations for the diagnosis of amyloidosis are summarized. A brief overview of invasive and non-invasive diagnostic methods and their combinations is provided, with an analysis of the evidence base for each of the diagnostic options and tools. The article describes an adapted algorithm for sequential diagnosis and differential diagnosis of patients with suspicion of ACM, taking into account the combination of clinical manifestations and examination data. The article covers the problem of specific treatment of AL- and ATTR, as well as symptomatic therapy, both heart failure, rhythm and conduction disorders, and neurological manifestations. The document will be useful both for primary level doctors and narrow specialists: cardiologists, neurologists, hematologists and nephrologists.

Aim of study. To analyze the state of the left ventricular geometry and structural features of the common carotid artery in men and women with chronic noncommunicable diseases.

Material and methods. Clinical and instrumental data of 475 patients with chronic non-communicable diseases were used. The average age of the participants was 52.3±13.4 years, all patients underwent general clinical examination. The frequency of various structural changes in the left ventricular (LV) myocardium and the intima-media thickness of the common carotid artery (CCA) were analyzed. Patients were divided into subgroups of men (n=237) and women (n=238).

Results. In the examined men, the levels of creatinine, cystatin C, and the LV myocardial mass index were significantly higher, while the glomerular filtration rate was significantly lower. In the subgroup of women, hemoglobin concentration and the number of erythrocytes were significantly lower, whereas the number of platelets was significantly higher. In individuals with chronic non-communicable diseases, structural LV changes were represented as follows: normal geometry (68.0%), concentric remodeling (6.1%), LV hypertrophy (25.8%), including concentric LVH (41.5%) and eccentric LVH (58.5%). Concentric remodeling was observed in 8.0% of men and 4.2% of women. The frequency of LVH was significantly higher in women (31.0%) compared to men (20.6%). Initial atherosclerotic changes were detected in 23.8% of patients, while atherosclerotic lesions of the CCA were found in 33.3%. In men, the frequency of CCA atherosclerotic lesions was significantly higher (41.3%) than in women (25.2%). In the subgroup of women with atherosclerotic lesions of the CCA, the incidence of LVH was significantly higher than in women without CCA lesions. Cystatin C positively correlated with the LV myocardial mass index (r=0.41143; p<0.005), relative LV wall thickness (r=0.27340; p<0.05), and CCA intima-media thickness (r=0.3027; p<0.05).

Conclusion. Left ventricular hypertrophy, especially the eccentric type, is frequently detected in patients with chronic non-communicable diseases, predominantly in women. Structural changes of the CCA are significantly more often detected in men. As the intima-media thickness of the CCA increases, the incidence of LVH also increases. Cystatin C concentration is associated with structural changes in the LV myocardium and CCA.

Aim. To evaluate the long-term results of percutaneous coronary intervention (PCI) plus optimal medical therapy (OMT) and OMT alone in patients with stable chronic coronary syndrome (CCS) and multivessel coronary artery disease who were not eligible for coronary artery bypass graft surgery (CABG).
Material and methods. This retrospective study included 374 patients with stable CCS and multivessel coronary artery disease, not eligible for CABG, hospitalized between 2013 and 2022. Patients were divided into 2 groups: Group 1 conservative therapy (OMT alone) and Group 2 invasive strategy (PCI+OMT). The primary composite endpoint of the study was the incidence of major adverse cardiovascular and cerebrovascular events (MACCE). In order to balance the groups according to their initial characteristics, Propensity score matching (PSM) was performed.
Results. Out of 374 patients, 321 patients were selected after PSM: 107 into the first group and 214 into the second group. The median age of the study patients was 69 [61; 76] years in group one and 66 [60; 74] years in group two (p=0,212). The median SYNTAX score was 32 [24; 40] versus 30 [24; 37], respectively (p=0,239). Long-term outcome analysis was performed in 306 patients out of 321 (95,3%). The incidence of the primary endpoint (MAСCE) in the conservative strategy group was 64,1% versus 51,8% in the invasive strategy group, p=0,034.
Conclusion. Invasive strategy (PCI+OMT) in patients with CCS and multivessel coronary lesions and not eligible for CABG is accompanied by a high rate of procedural success and a low rate of in-hospital complications. Myocardial revascularization by PCI in this cohort of patients leads to statistically significant reduction in the incidence of MACCE and cardiac mortality in the long-term follow-up period in comparison with conservative therapy.

Introduction. The pathogenesis of slow flow coronary syndrome and its reversibility are not fully understood, which makes it difficult to treat these patients. Recently, there has been increasing evidence that inflammation plays a significant role in the development of these syndromes. The aim of this study was to investigate the functional or fixed nature of slow coronary blood flow and the potential reversibility of these changes in a chronic inflammation model.

Material and methods. The study included 19 patients with pulmonary tuberculosis who had signs of slowdown in coronary blood flow according to coronary angiography. A modified TIMI scale was used to assess the rate of movement of the radiocontrast agent. To assess the reversibility and possible functional nature of the changes, all angiographic studies were performed before and after intracoronary administration of 100 μg of nitroglycerin. The effects of antituberculosis chemotherapy and surgical treatment were evaluated.

Conclusions. The syndrome of slow coronary flow in patients with long-term chronic inflammation is not functional in most cases, but rather fixed. Conservative or surgical treatment aimed at eliminating the source of the inflammatory response is not accompanied by normalization of coronary flow.

Objective: to study the contribution of the soluble guanylate cyclase stimulator riociguate to the overall effect of combined treatment of patients with inoperable chronic thromboembolic pulmonary hypertension.

Material and methods. The study included patients with an inoperable form of CTEPH who were treated with riociguat and underwent balloon pulmonary angioplasty (BPA). The patients were divided into 2 groups of 50 people "Riociguat+BPA" and "BPA+Riociguat". Patients of the "Riociguat+BPA" group received PAH-specific therapy with riociguat at the first stage of treatment, after which they underwent several sessions of BPA. Patients of the "BPA+Riociguat" group recieved a series of BPA, and then riociguat was initiated. Patients took riociguat for 6 months at a target dose of 7.5 mg/day in both groups. The average number of completed BPA sessions was 4. A control invasive measurement of hemodynamic parameters was performed at three points: before the start of treatment, after the first stage of treatment, and 6 months after the completion of the entire course of treatment.

Results. Following the administration of riociguat, there was a statistically significant increase in the 6MWT distance by 36 m in the “Riociguat+BPA” group and by 17 m in the “BPA+Riociguat” group. Improvement in key hemodynamic parameters was observed: in particular, after initiation of riociguat therapy, a decrease in mPAP by 6 mmHg and PVR by 1,97 Wood units was observed in the “Riociguat+BPA” group, while in the “BPA+Riociguat” group, a decrease in mPAP by 5 mmHg and PVR by 1,7 Wood units was observed. A smaller influence of riociguat was found with respect to gas exchange parameters. When comparing the results achieved at the end of the course of treatment, both groups demonstrated a good effect from the combined approach, regardless of which type of treatment was initiated first.

Conclusion. The contribution of riociguat is manifested in the improvement of the functional status and hemodynamic parameters both when prescribing the drug before a series of BPA sessions, and when adding the drug after endovascular treatment. The sequence of initiation of treatment methods within the framework of the combined approach should be determined individually.

Objectives. To characterize the state of the cardiovascular system in patients carrying the HFE gene mutations in the Russian Federation.
Materials and methods. An evaluation of 39 cases of HFE gene mutations confirmed by biochemical and genetic methods was carried out.
Results. When analyzing the medical records of 39 patients with HFE gene mutations, changes in the structural and functional state of the cardiovascular system were detected in 20 (51.3%) patients. There was a history of arterial hypertension in 11 (28.21%) patients. Acute cerebrovascular accident was indicated in the anamnesis in 2 (5.13%) patients. A history of myocardial infarction was detected in 3 (7.69%) patients. Heart failure of non-ischemic genesis was diagnosed in 5 (12.82%) patients. In 14 (35.9%) patients in the form of atrioventricular block, left bundle branch block, right bundle branch block, intraventricular conduction disturbances, shortening of the PQ interval and prolongation of the QT interval, in 1 (2.56%) patient a paroxysmal form of atrial fibrillation was noted.
Conclusion. Half patients with HFE gene mutations had various changes in the cardiovascular system. In this regard, patients with cardiac pathology of unclear etiology require screening for timely diagnosis of hemochromatosis and joint management of patients with confirmed hemochromatosis by hematologists, cardiologists and hepatologists to prevent cardiovascular complications.

Aim. To evaluate the methods used for therapeutic compression of complications and the use of anticoagulants during arrhythmia catheter interventions and in the early postoperative period at stages of thrombotic disease development and treatment outcomes, as well as at stages of hemorrhagic complications.

Material and methods. The study was prospective. The study included 688 patients who underwent intracardiac electrophysiological studies, radiofrequency, or cryothermic catheter ablation. Patients used non-drug and drug-based thrombosis prevention methods in various treatment options. All patients underwent ultrasound examination of the access site before and within 24 hours after the procedures. The presence of hemorrhagic and thrombotic interventions, as well as the treatment methods for the latter lesions, were repeated after duplex intervention.

Results. Venous thrombosis was detected in 113 patients (16.4%), and serious hemorrhagic signs requiring additional treatment were detected in 18 (2.6%). Cautious use of therapeutic compressions and intraoperative heparin administration demonstrated efficacy in preventing the risk of thrombosis. The effect of postoperative enoxaparin sodium administration was dose-dependent. The use of anticoagulants did not affect the risk of hemorrhagic complications. Oral and parenteral anticoagulants significantly resulted in complete or partial thrombolysis in most patients.

Conclusion. Patients, according to arrhythmia catheter principles, may benefit from the active use of one or more DVT prophylaxis methods without an officially standardized risk of increased hemorrhagic complications.

Aim. The purpose of this study is to compare the single use of a fixed minimum dose of cavutilid (refralon; Russian Federation, E.I. Chazov National Medical Cardiology Research Centre) 350 μg with amiodarone in patients with paroxysms of atrial fibrillation and flutter.

Materials and methods. The study included 62 patients with paroxysmal atrial fibrillation and flutter, divided into two groups (31 patients each). The first group received single fixed dose of cavutilid 350 μg, the second — amiodarone at a starting dose of 5 mg / kg with a possible continuation of the infusion up to 1200 mg in case of failure to achieve sinus rhythm recovery. The patients did not differ in main clinical and instrumental parameters, except for the higher weight and duration of medical history in the amiodarone group. The effectiveness of cardioversion (restoration of sinus rhythm within 1 and 24 hours, time to restoration of sinus rhythm, reduction of heart rate, achievement of " lenient " heart rate control, absence of sustained relapses) and safety (presence of serious cardiovascular complications, ventricular arrhythmias, prolongation of QT/QTc intervals, severe bradycardia and pauses >3,0 sec, arterial hypotension).

Results. In the cavutilid group, sinus rhythm restoration within 1 hour was achieved in 87.1% of patients, in the amiodarone group — in 19.4% (p<0.001). After 24 hours, sinus rhythm was registered in 93.3% and 64.5%, respectively (p=0.011). Time to sinus rhythm restoration was significantly faster in patients of the cavutilid group (median 8.00 [5.00; 13.00] minutes versus 140.00 [60.75; 240.00] minutes for amiodarone; p<0.001). The arrhythmia recurrence rate, heart rate reduction, and achievement of "linient" frequency control did not differ statistically between the groups compared. Major adverse cardiovascular events and ventricular tachycardia were not reported in any of the groups. Prolongation of the QT/QTc interval was less common (in 2 patients, 6.5%, versus 5 patients, 16,1%; p=0,425) and lasted significantly less time after the use of cavutilid. Arterial hypotension was observed in 2 patients (6.5%) only in the amiodarone group.

Discussion. The high efficacy and safety of a single fixed dose of cavutilid 350 μg for pharmacological cardioversion of atrial fibrillation and flutter paroxysms demonstrate low risk of arrhythmogenic effects of the drug and the possibility of reducing patient follow-up time both in intensive care units and in general inpatient setting. Taking these advantages into consideration, we may suggest that fixed dose of cavutilid 350 μg has potential for outpatient use.

Congestive heart failure (CHF) is a highly prevalent clinical syndrome that winds up any starting point of a cardiovascular continuum. It is therefore a precious point on the agenda for the world leading scientists and investigators, who aim to understand the full spectrum of its underlying molecular and pathophysiological defects. In spite of a wide range of modern diagnostics techniques, it is still a clinical burden to correctly diagnose CHF among other clinically similar nosology’s. As a consequence, extreme attention is now brought to the epigenetics of a sustaining heart homeostasis. Small non-coding RNAs, called microRNAs (miRNAs) are considered to play an important role in cardiac metabolism management. MiRNA do not only regulate transcription and post-translational gene expression patterns but also account for the matrixRNAs (mRNAs) translation. Various types of miRNAs are involved in certain pathophysiological processes, such as cardiac structural remodeling, cellular apoptosis, angiogenesis and persistent inflammation – all accounting for the CHF progression. Potential use of miRNAs as a CHF diagnostic biomarker is now actively proposed by a large number of studies. Reasons behind include their prevalence in all types of investigative fluids, sample stability and most importantly, an ability to be precisely spotted with the aid of modern techniques. Current article is primarily dedicated to the underlying biology of miRNAs action and their role in CHF development.