Idiopathic recurrent pericarditis (IRP) is an orphan inflammatory disease of the pericardium, characterised by the absence of pathognomonic symptoms and specific biomarkers. The diagnosis is complicated by the absence of characteristic clinical signs and the lack of specific markers for the recurrence of IRP, which hampers the timely initiation of pathogenetic therapy and significantly worsens the prognosis. To address the challenges of complex differential diagnosis, low physician awareness, and the lack of unified guidelines for IRP for general practitioners, internists, and cardiologists, it was necessary to establish a specialized expert center. This center aims to provide synergy among highly qualified doctors from various specialisations for precise diagnosis confirmation.

Chronic heart failure is a leading cause of increased mortality rates globally, including in the Russian Federation. Guideline-directed medical therapy plays a key role in the management of this patient cohort, particularly among individuals with reduced left ventricular ejection fraction. Of special interest is the subgroup with improved left ventricular ejection fraction, for which data remain limited and the issue of further medical treatment remains unresolved. This review aims to categorize this patient population and address the feasibility of discontinuing or reducing medical therapy.

AIM:To evaluate the efficacy and safety of the use of sodium-glucose cotransporter type 2 (SGLT-2) inhibitors in patients with chronic heart failure (CHF) due to amyloid cardiomyopathy (ACM). MATERIALS AND METHODS:50 patients with CHF due to different types of ACM were included and randomized in two groups at a ratio of 1:1. In the 1st group (n=25), SGLT-2 inhibitors were added to basic therapy. In the 2nd group (n=25) patients received only basic therapy. The observation period was 6 months. Clinical function status, laboratory marker dynamics, echocardiography parameters, frequency of hospitalizations and presence of cardiovascular events were evaluated. RESULTS:SGLT-2 inhibitors therapy was well tolerated, safe. The frequency of hospitalizations due to decompensated heart failure was higher in the 2nd group (p=0.048), cases of hospitalization in the 2nd group occurred statistically significantly earlier (p=0.017). Mortality in the 1st group was 4% (1 case), in the 2nd group - 16% (4 cases), due to progressive CHF. In the 1st group, the distance of a 6-minute walk test increased (p<0.001), the quality of life assessed by the Minnesota Living with Heart Failure Questionnaire improved (p<0.001), the expression of shortness decreased (p<0.001), there was an improvement in clinical status according to the clinical assessment scale (p<0.001), while in the 2nd group there was a statistically significant deterioration of clinical functional status. The dynamics of the N-terminal pro-Brain Natriuretic Peptide and troponine levels were positive in the 1st group and negative in the 2nd group (p=0.001 and <0.001 respectively). Significant changes in the glomerular filtration rate in the 1st group were not detected (p=0.475), in the 2nd group there was a decrease of this parameter (p<0.001). According to the results of echocardiography in the 2nd group a significant reduction of the left ventricular (LV) ejection fraction (p=0.001) was observed, and in the 1st group was a tendency to decrease of the LV ejection fraction (p=0.238). Regardless of the SGLT-2 inhibitors intake, there was a decrease in the end-diastolic dimension of LV (p=0.046 for 1st group and <0.001 - for 2nd group), as well as a progressive thickening of heart walls (p=0.011 and <0.001 for interventricular septum thickness, p=0.004 and <0.001 - for posterior wall thickness of LV). CONCLUSION:The administration of SGLT-2 inhibitors to patients with CHF due to ACM leads to an improvement in quality of life, a decrease in the frequency of hospitalizations due to decompensated heart failure of CHF, but does not affect mortality. Further study of therapeutic efficacy in more patients is needed.

Introduction. In recent years, the detection rate of transthyretin amyloid cardiomyopathy has been rapidly increasing. The only drug registered as a pathogenetic therapy in Russia is tafamidis. To date, there is no single protocol for assessing disease progression, and the role of scintigraphy with phosphate complexes is not reflected in current documents due to the lack of evidence.Brief description. The article presents a case series including patients who received tafamidis therapy for at least 12 months. During therapy, there were no signs of disease progression, and in some cases, according to myocardial scintigraphy with phosphate complexes, a decrease in radiopharmaceutical uptake was noted.Discussion. The role of myocardial scintigraphy with phosphate complexes for monitoring the effectiveness of tafamidis therapy was discussed.

Early right ventricular failure after left ventricular assist device (LVAD) implantation is one of the main causes of a worse postoperative prognosis. The problem of de novo right ventricular failure requires the search for methods of primary prevention and improvement of treatment strategies in its development. Preoperative therapy with levosimendan has a positive effect on right ventricular function and may be considered as a method of preoperative preparation for LVAD implantation.

Background. Acute decompensation of heart failure (ADHF) is a period of chronic HF, which is characterized by a rapid aggravation/onset of HF symptoms, requiring urgent hospitalization for intensive care and worsening the patient’s prognosis. Aim. To evaluate the efficacy of allopurinol in patients with ADHF, hyperuricemia, and reduced glomerular filtration rate compared with standard of care for 6 months. Materials and methods. We present the results of a 6-month follow-up for 72 patients with ADHF, hyperuricemia, and reduced filtration function of the kidneys, calculated using the CKD-EPI formula, divided equally into two groups: group 1 received standard of care for HF combined with a xanthine oxidase inhibitor – allopurinol, and group 2 received standard of care only. Allopurinol was administered to group 1 patients at an initial dose of 50 mg, on average, 24-48 hours after admission to the hospital, in addition to standard of care. The study included 72 patients, with an average age of 71.67 years in group 1 and 70.28 years in group 2. The indicators at the time of enrollment in the study, when HF compensation was achieved, and after 6 months were analyzed. Results. The median length of hospital stay in the allopurinol group was 13 [12; 18] days vs. 14 [10; 15] days in the standard of care group (p = 0.283). At 6 months of follow-up, repeated hospitalizations due to ADHF were reported in both groups at a similar rate. During the entire follow-up period, one death was reported in the allopurinol group. Statistically significant decreases in the levels of N-terminal precursor of brain natriuretic peptide were observed in both groups (p 0.001). Significant changes in C-reactive protein levels were found in both groups at discharge. The analysis of echocardiographic parameters revealed a significantly increased left ventricular ejection fraction, a reduction in inferior vena cava size, and a decrease in systolic pressure in the pulmonary artery in both groups. During the therapy, there was a significant increase in the walking test distance in both groups (p 0.001), with no significant difference between the groups. Conclusion. The results of the study indicate a positive effect of allopurinol on uric acid levels, with no additional benefits and effects on endpoints.

Acute decompensation of heart failure (ADHF) is a period of the course of chronic heart failure (CHF), which is characterised by rapid aggravation/appearance of heart failure (HF) symptoms, requiring emergency hospitalisation of the patient and intensive care. ADHF is an urgent problem for modern healthcare and is associated with unfavourable prognosis and high mortality in this group of patients. Aim. To evaluate the safety of allopurinol prescription in patients with ADHF, hyperuricaemia and reduced level of glomerular filtration rate irrespective of left ventricular ejection fraction. Materials and methods. The results of standard therapy of HF with diuretic therapy in combination with xanthine oxidase inhibitor – allopurinol in the starting dose of 50 mg in 36 patients with ADHF of functional class II–IV, hyperuricemia, reduced renal filtration function calculated by the CKD-EPI formula are presented. Patients were hospitalised from February 2023 to January 2024 in the department of myocardial diseases and heart failure of FGBU ‘E.I. Chazov NMICC’ of the Ministry of Health of Russia. The study included 36 patients, the mean age was 71.6±9.8 years. Of them 72% of patients were male. The indices at the moment of inclusion in the study and at achievement of HF compensation were analysed. Results. The median number of days spent in hospital was 14.7±5.7. On the background of optimal drug therapy of CHF in combination with allopurinol by the time of discharge the level of uric acid statistically significantly decreased – median before treatment 509 [460; 563], after 384 [330; 418] μmol/l, р0.0001; decreased concentrations of N-terminal precursor of brain natriuretic peptide: pre-treatment median 3972 [2322; 9272], post 2132 [983; 3867] pg/ml, p=0.0001; decreased Creatine Kinase median pre-treatment 73 [55; 108], post 63 [47; 83] U/L, p=0.0011; decreased urea concentration pre-treatment 8.4 [7; 11], after 8.3 [7; 10] mmol/L, p=0.01; decreased total bilirubin median pre-therapy 23.2 [15.5; 28.8], after 18.5 [15; 25.6] µmol/L, p=0.025; C-Reactive Protein – median before treatment 4.3 [2; 14.2] mg/l, after 2.8 [1.1; 11.2] mg/l, p=0.036; there was a significant increase in distance test – six minute walk (T6X) – median before treatment 158 [149; 185], after 301 [283; 321], р0.0001. No acute kidney injury and progression of Chronic Kidney Disease were observed during the period of hospitalisation. No dyspeptic phenomena and allergic reactions were noted on the background of allopurinol administration. Statistically significant reductions in echocardiographic parameters were observed in NPV measurement – median before therapy 2.3 [2; 2.5] cm, after 2 [1.9; 2.4] cm, p=0.0002; decrease in Systolic Pulmonary Artery Pressure before therapy 50±14.8 mmHg, after 44±13.2 mmHg, p=0.0001; statistically significant increase in LVEF-median pre-therapy 30 [25; 53], after 34 [27;55]%, p=0.0002. Conclusion. The use of allopurinol reduces uric acid concentration, which is accompanied by improvement of renal function as HF signs are compensated, and also has a favourable safety profile when used in patients with ADHF, hyperuricemia and Chronic Kidney Disease.

Aim. To determine the echocardiographic variants of phenotype presentation of amyloid cardiomyopathy (ACM) in patients with different types of amyloidosis. Materials and methods. The study included 54 patients with ACM: 27 with light chain amyloidosis (15 [56%] males; the median age was 63.0 [56.5; 67.0]) and 27 patients with transthyretin amyloidosis (20 [74%] males; the median age was 73.0 [65.5; 78.5]). Standard echocardiographic parameters and the left ventricular (LV) global longitudinal strain in both groups were evaluated. Results. Among patients with ACM, the following phenotypes were reported: hypertrophic phenotype (HP), a combination of hypertrophic and restrictive phenotypes (HP+RP), a combination of hypertrophic, restrictive phenotypes and ejection fraction (EF) less than 50% (HP+RP+EF50%), and patients with minimal structural changes. In patients with HP+RP+EF50%, significantly greater thickness of the LV posterior wall (p=0.025) and the relative wall thickness (p=0.010) were found; the LV global longitudinal strain was lower (p=0.001). There were significant differences in the size of the right atrium (p=0.036), the systolic pressure in the pulmonary artery (p0.001), and the presence of a thickening of the free wall of the right ventricle (p0.007) in the HP+RP group. Involvement of the heart valves was more common in patients with ATTR ACM. Conclusion. After reviewing the echocardiographic data of patients, various phenotypic presentations of ACM and several correlations between echocardiographic characteristics depending on the type of amyloidosis were determined. Further study of echocardiographic parameters in patients with various types of amyloidosis may be promising for early diagnosis and proper treatment of ACM.

Currently, the proportion of patients with heart failure is progressively increasing. Despite the progress in drug therapy, additional manipulations are often necessary to correct cardiovascular pathology in these patients. Given the high comorbidity, the use of classical surgical techniques is impossible in some cases. Implantation of high-tech devices, catheter ablation of arrhythmogenic areas, transcatheter mitral valve plastic surgery are minimally invasive surgical methods. Proper interaction between a cardiologist and a cardiovascular surgeon allows for the maximum personalization of the approach to the treatment of each patient, achieving maximum results with significantly reduced risks.

Background. To determine predictors of early and late right ventricular failure (RVF) according to transthoracic echocardiography (TTEchoCG) and right heart catheterisation (RHC) in patients with left ventricular assist device (LVAD). Materials and methods. Twenty-three patients with LVAD were included in the study. Before implantation, all patients underwent TTEchoCG with comprehensive evaluation of the right ventricle (RV) using speckle-tracking echocardiography (STE) and 3D-echocardiography (3D-RVEF), as well as RHC with measurement of standard indices and calculation of pulmonary artery pulsatility index (PAPi). Results. The highest area under the ROC curve was the RV ejection fraction determined by 3D-RVEF (0.841 with 95% CI 0.677–1.006, sensitivity 0.889, specificity 0.786; p0.001) with a cut-off value ≤42% (OR 29.3 with 95% CI 2.6–336.4; p=0.007) and PAPi (area on ROC curve 0.869 with 95% CI 0.503–0.975, sensitivity 0.778, specificity 0.857; p0.001,) with a threshold value ≤2.2 (OR 20 with 95% CI 1.2–333.3; p=0.035). The combination of these parameters was the most accurate prognostic model (sensitivity 0.778, specificity 1). The combination of echocardiographic parameters – 3D-RVEF and systolic velocity of the tricuspid valve fibrous ring according to tissue myocardial Doppler (TMD: S’ml) has similar sensitivity (0.778) and slightly lower specificity (0.929). Conclusion. The optimal independent echocardiographic predictor of early RVF is 3D-RVEF. The combination of 3D-RVEF and PAPi proved to be the most accurate model, but the combination of 3D-RVEF and S’ml-TMD echocardiographic parameters alone is only slightly inferior in specificity, which allows preliminary assessment of the risk of RVF.

Idiopathic recurrent pericarditis is a rare pathology characterised by recurrent inflammation in the cardiac cavity. Treatment of recurrent pericarditis is empirical and based on the use of drugs with anti-inflammatory properties. First-line drugs are non-steroidal anti-inflammatory drugs and colchicine, second-line drugs are glucocorticosteroids. This is associated with the development of undesirable side effects, which makes it impossible to continue therapy in a number of patients. This article presents two clinical cases, describes the course of the disease and the development of complications at different stages. This article demonstrates the complexity of selecting the optimal therapy in real clinical practice.

Amyloid cardiomyopathy is an infiltrative disease with extremely adverse prognosis. New treatments are now emerging, however they are not aimed at eliminating the existing deposits of amyloid, but at preventing new ones. Given the difficulties of diagnosing the disease at an early stage, patients often end up in the hospital with advanced heart failure. In such cases, the efficacy of a pathogenic therapy is extremely low, which makes the treatment of an advanced stage of the disease very important. The article discusses the possibility of heart transplantation for patients with amyloid cardiomyopathy. A clinical case of a patient with hereditary transthyretin amyloidosis who was performed biatric orthotopic heart transplantation is presented.

Dilated cardiomyopathy (DCM) is a leading cause of heart failure, sudden cardiac death, and heart transplantation in young patients. The causes of DCM are varied and include genetic factors and metabolic, infectious, toxic and others factors. Today it is known that germline mutations in more than 98 genes can be associated with the occurrence of DCM. However, the penetrance of these genes often depends on a combination of factors, including modifiable ones, i.e. those that change under the influence of the environment. About 20–25% of genetically determined forms of DCM are due to mutations in the titin gene (TTN). Titin is the largest protein in the body, which is an important component of the sarcomer. Although titin is the largest protein in the human body, its role in the physiology of heart and disease is not yet fully understood. However, a mutation in the TTN gene may later represent a potential therapeutic target for genetic and acquired cardiomyopathy. Thus, the analysis of clinical cases of cardiomyopathy in patients with identified mutations in the TTN gene is of great scientific interest. The article presents a clinical case of manifestation of DCM in patient with a revealed pathogenic variant of mutation in the gene TTN and reverse left ventricular remodeling of the against the background of optimal therapy of heart failure in a subsequent outpatient observation.

Aim. To assess the safety and efficacy of a single intravenous bolus of non-immunogenic staphylokinase compared with alteplase in patients with massive pulmonary embolism and hemodynamic instability. Non-immunogenic staphylokinase is a modified recombinant staphylokinase with low immunogenicity, high thrombolytic activity and fibrin selectivity.Material and methods. This multicenter, open-label, randomized, comparative clinical trial FORPE in two parallel groups was conducted in 23 clinical centers in Russia. A total of 310 patients aged 18 years and older with hemodynamic instability and computed tomography pulmonary angiography verified massive pulmonary embolism and right ventricular dysfunction were included. The patients were randomly assigned in either non-immunogenic staphylokinase (15 mg) or alteplase (100 mg) group. Both medicines were administered intravenously. An independent biostatistician created a randomization sequence using computer-generated random numbers. Randomization was performed using the envelopes. The study was open-label, and emergency unit staff, investigators, and patients were informed about the assigned drug. The primary efficacy endpoint was 7-day all-cause death after randomization. The non-inferiority margin was set at 10% for the difference in 7-day all-cause mortality after randomization between the compared groups. Non-inferiority was tested using the Welch t-test for the primary efficacy endpoint. Secondary efficacy endpoints were analyzed in both the intention-to-treat and per-protocol populations.Results. Of 348 patients screened between December 25, 2020, and July 31, 2023, 310 (89%) were included in the study. Of the total number, 155 (50%) patients were randomized to the non-immunogenic staphylokinase group and 155 (50%) to the alteplase group. In the non-immunogenic staphylokinase group, the primary efficacy endpoint, 7-day all-cause death, was 2% in the intent-to-treat population and 2% in the per-protocol population, whereas in the alteplase group it was 3% (odds ratio (OR) 0,75, 95% confidence interval (CI) 0,11-4,49; p=1,00) and 3% (OR 0,75, 95% CI 0,11-4,52; p=1,00), respectively. The difference in the primary efficacy endpoint was 0,6% (95% CI -2,8 to -4,0) in the intent-to-treat population and 0,6% (95% CI -2,9 to -4,2) in the per-protocol population. Thus, the lower limit of the 95% CI did not cross the non-inferiority margin (p<0,001). There were no cases of hemorrhagic stroke in the non-immunogenic staphylokinase group, whereas there were three cases (2%) of hemorrhagic stroke in the alteplase group (p=0,25). Serious adverse events were experienced by 11 patients (7%) in the non-immunogenic staphylokinase group compared with 12 patients (8%) in the alteplase group (p=1,00).Conclusion. Non-immunogenic staphylokinase is at least as effective as alteplase in the treatment of patients with massive pulmonary embolism with hemodynamic instability and has a higher safety profile. Future observational studies of non-immunogenic staphylokinase are needed to continue assessing its safety and efficacy. Given the high safety and efficacy of non-immunogenic staphylokinase, its use should be studied in patients with moderate-to-high risk pulmonary embolism.

The burden of heart failure (HF) has been increasing worldwide in recent decades. Early diagnosis of HF based on the outpatient measurement of natriuretic peptide (NP) concentration will allow timely initiation of the treatment and reducing the incidence of adverse outcomes in HF. Unfortunately, the frequency of NP testing remains low worldwide. At the online expert meeting held on March 15, 2024, the features of the N-terminal pro-brain natriuretic peptide (NT-proBNP) test (Elecsys proBNP by Roche) were discussed along with the interpretation of test results and presentation of results in laboratory reports. The experts addressed the features of the Elecsys proBNP test in patients with suspected HF in various clinical scenarios (chronic and acute HF). The limits of clinical decision for the NT-proBNP test were established depending on the clinical scenario. Changes in the Elecsys proBNP test results depending on the comorbidities were addressed. The experts suggested ways to optimize the format of the Elecsys proBNP test result reports in the Russian Federation, which will accelerate the implementation of the test in clinical practice and optimize the management of HF patients.

Aim. To evaluate the features of diagnosis of amyloid cardiomyopathy (ACMP), differential diagnosis of different types of amyloidosis and its clinical manifestations. Materials and methods. Were analyzed 150 cases of patients who consulted at the Expert Center for Amyloidosis with suspicion of the presence of ACMP. 63 patients were diagnosed with ACMP: 25 (39.7%) – women, 38 (60.3%) – men, with an average age of 64.1±1.5. 36 (57.1%) patients had AL-amyloidosis (immunoglobulin amyloid light-chain amyloidosis), 25 (39.7%) – ATTR-amyloidosis (transthyretin amyloidosis), 2 (3.2%) – AA-amyloidosis with heart failure (reactive systemic amyloidosis caused by hypersecretion of á-globulin). The analysis of clinical manifestations depending on the type of amyloidosis, data of laboratory and instrumental methods of diagnosis is carried out. Results. In most cases, 53 (84.1%) patients, amyloidosis manifested as signs of heart failure. Among cardiac manifestations, shortness of breath (95.2%), general weakness (93.7%), lower limb edema (76.2%) were the most common. To confirm the diagnosis, despite the high accuracy of the speckle-tracking echocardiography and magnetic resonance imaging of the heart with gadolinium, in rare cases a biopsy is required (e.g. there is a combination of clinical signs of several types of amyloidosis). Biopsy of the affected organ was performed in 31 (49.2%) patients. The strategy for further pathogenetic treatment depends on the determination of the type of amyloidosis. Free light chains of immunoglobulins were detected in 57.1% of cases, which allowed diagnosis of AL-amyloidosis. In 17 (38.6%) patients myocardial scintigraphy with 99mTc-pyrophosphate showed signs of ATTR-amyloidosis, which with a negative result of immunochemical studies allows non-invasive diagnosis of it. Conclusion. ACMP is a disease with an extremely adverse prognosis. Raising the awareness of specialists about ACMP is an important goal. With timely diagnosis, pathogenetic therapy can be started early, which will improve the quality of life and prognosis of patients with ACMP.

Russian Society of Cardiology (RSC)With the participation: National Society of Myocardial Diseases and Heart Failure, Society of Heart Failure Specialists, Russian Scientific Medical Society of Internal MedicineEndorsed by the Research and Practical Council of the Ministry of Health of the Russian Federation (12.09.2024)

Aim. To study the effectiveness of virtual reality (VR) relaxation to correct symptoms of depression, anxiety, emotional and cognitive disorders in patients with heart failure (HF).Material and methods. The study included 100 patients with HF aged 21 to 83 years. A main group of 58 people was randomly selected, each of whom received a course of 5 daily VR sessions of a relaxation program. Patients in the control group underwent VR sessions containing distracting visual images without a relaxation program. During the study, mental status of all patients was assessed using questionnaires sensitive to the cognitive and emotional areas.Results. After completing five daily VR sessions with a relaxation program, patients with HF noted a decrease in stress (p=0,031) and improved sleep (p=0,002), and 2 days after completing the VR course — improved well-being (p=0,006), mood (p=0,001), sleep (p=0,003) and decreased stress (p=0,005). In addition, patients in the main group had improved daily activities (p=0,004), pain/discomfort (p=0,007), anxiety/depression (p=0,016) and general well-being (p=0,009).Conclusion. VR relaxation is effective for reducing stress, improving mental status and cognitive functioning in patients with HF.