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Abstract One of the physical methods for obtaining magnetite nanoparticles (NPs) is electron beam physical vapor deposition (EB PVD), which requires complex equipment, but allows obtaining a significant amount of pure (ligand-free) NPs. The biomedical application of such NPs is less studied than materials from other synthesis methods. The objective is to study the effect of pure magnetite NPs in the NaCl matrix obtained by EB PVD on hematological indicators, gases, electrolytes and parameters of iron metabolism in the blood of intact animals. The physical characteristics of NPs were studied using high-resolution transmission electron microscopy, scanning transmission electron microscopy, energy-dispersive X-ray spectroscopy mapping, electron energy-loss spectroscopy, selected area electron diffraction and fast Fourier transform. In vivo experiments were conducted on albino male rats, which were injected with solution of magnetite-sodium chloride NPs (1.35 mg Fe/kg). After 3 and 72 h, hematological parameters, blood gases, electrolytes, and serum iron were determined. The synthesized NPs had an average size of 11 nm. They were identified as magnetite, where polycrystals and single crystals were present. The absence of contamination in crystal boundaries, clear orientation and orderliness of atoms in crystals were established. The administration of NPs in the sodium chloride matrix to animals was characterized by a transient increase in the main indicators of red blood accompanied by an increase in the saturation of erythrocytes with hemoglobin and their mean volume after 3 h. It did not worsen blood gases and pH, but decreased blood Na+ content after 72 h. The investigated NPs caused changes in the parameters of serum iron characteristic to iron preparations, which after 3 h were smaller compared to the reference iron drug, and after 72 h—similar to it. More intense rapid effects on hematological parameters at lower serum iron indicate greater activity of the studied pure magnetite NPs obtained by EB PVD syntesis compared to the reference iron preparation.

Diagnosis and treatment of myocarditis can be challenging, including determining indications for heart transplantation. We present a 6-year medical history of a 54 years old patient with severe morphologically verified viral-negative lymphocytic myocarditis and systemic manifestations (onset of hemorrhagic vasculitis) combined with moderate coronary atherosclerosis, which regressed according to repeated coronary angiography. For 5 years, the patient received immunosuppressive therapy with methylprednisolone and azathioprine with a significant improvement. Repeated relapses of atrial fibrillation required correction of basic therapy and plasmapheresis. The disease was complicated by thyrotoxicosis and multi-organ dysfunction; the autopsy showed persistent myocarditis activity. The myocarditis is a chronic condition and requires a review of the treatment strategy at each stage.

Aim. To study the place of NCM in the structure of DCM, its clinical features and influence on prognosis in comparison with other forms of DCM syndrome.Methods. The NCM registry includes 125 patients, mean age 46.4±15.1 years, 74 men and 51 women, median follow-up 14 [4.0; 41.0] months. The DCM registry included 365 patients, mean age 46.4±15.1 years, 253 men and 112 women, median follow-up 14 [5; 43.75] months. The examination included electrocardiography (ECG), ECG Holter monitoring, echocardiography, blood anti-heart antibody level evaluation, and additionally cardiac computed tomography, magnetic resonance imaging, DNA diagnostics (in the MYH7, MYBPC3, TPM1, TNNI3, TNNT2, ACTC1, TAZ, ZASP (LDB3), MYL2, MYL3, DES, LMNA, EMD, TTR gene panel), coronary angiography, right ventricular endomyocardial biopsy.Results. The proportion of patients with DCM phenotype in the NCM registry was 40% (n=49), another 11% (n=15) had NCM diagnosed simultaneously with acute/subacute myocarditis. Lethality in these subgroups was 12.2% and 33.3%, respectively, and was significantly higher than in asymptomatic, ischemic and arrhythmic variants of NCM. In the DCM registry, the proportion of patients with NСM was 21% (n=78), and increased left ventricular (LV) trabecularity was detected in another 18% (n=64). DCM patients with and without NСM did not differ by baseline echocardiographic parameters, heart failure class, and cardiotropic therapy. Pathogenic mutations were detected in 14% of DCM patients with NCM and only 3% of other patients with DCM (p

September, 2021 marks the 175th anniversary of the opening of the Faculty Therapeutic Clinic (FTC) of the Imperial Moscow University, which is a successor of the First (1805) and Second (1820) Clinical Institutes with their therapeutic beds and chairs, and thus is the oldest therapeutic clinic in Russia. From 1930 the Clinic became part of the newly formed I.M. Sechenov First Moscow Medical Institute (now Sechenov University) and retains continuity to the present day. This publication is one of three research articles prepared by the staff of the Clinic for the anniversary and devoted to little-known pages of biography and scientific activities of the three heads of the FTC at the crucial stages of its history – A.I. Over, G.A. Zakharyin and the last prerevolutionary director – N.F. Golubov.

Aim. To study the late results of medical and interventional treatment in patients with morphologically verified nature of idiopathic arrhythmias.Methods. The prospective study included 20 patients (mean age 43.1±11.3 years, 10 female) with atrial fibrillation (AF), supraventricular and ventricular extrasystole, supraventricular and ventricular tachycardia, conduction disturbance without structural heart changes. In addition to the standard examination, the level of anti-heart antibodies was initially determined; endomyocardial biopsy (EMB) of the right ventricle with PCR study for the viral genome; DNA diagnostics (n=4), coronary angioraphy (n=6), skin biopsy (n=1) were performed. The median follow-up was 134 [128; 138] months.Results. By EMB in the initial examination were diagnosed: active (n=8)/borderline (n=3) infectious immune myocarditis; parvovirus-positive endomyocarditis (n=1); undifferentiated vasculitis (n=2); myocardial vasculitis (n=1); Fabry disease (n=1); arrhythmogenic right ventricular dysplasia (n=1); unspecified cardiomyopathy (n=2). Anti-heart antibodies were the most important in myocarditis diagnosis and monitoring. All patients with myocarditis/vasculitis (n=15) received its basic therapy: acyclovir (n=10); immunoglobulin G 10-12.5 g (n=2); hydroxychloroquine 200 mg/day (n=15); glucocorticoids (n=14); azathioprine 150 mg/day (n=2). The late results were evaluated in all patients with myocarditis. Initially, in 62.5% of patients a resistance of AF to all antiarrhythmic drugs was noted. After treatment the average frequency of AF paroxysms decreased (from 8 [5; 8] to 3 [1,25; 7,75] points). By the end of the follow-up, six patients underwent radiofrequency ablation (RFA) for AF, the full effect was achieved once. All patients without RFA have AF partially or completely resistant to drugs. Two patients (without RFA) died from ischemic stroke/ pulmonary embolism.Conclusion. Using EMB the causes of idiopathic arrhythmias (mainly AF) were diagnosed: immune inflammatory diseases in 75% and genetic in 25% of patients. As a result of complex treatment, the general burden of arrhythmias has decreased. But the presence of myocarditis and primary cardiomyopathy, without reducing the cardiac contractility and dilatation, does not allow achieving a stable antiarrhythmic effect. Lethality for 11 years was 10%. The causes of death were thromboembolic complications.

The possibility of heart inflammation (both myocardial and endocardial) months after a coronavirus disease 2019 (COVID-19) has not been practically studied, especially since approaches to the treatment of myocarditis in combination with various endocarditis forms have not been developed.Aim. To study the prevalence and mechanisms of SARS-CoV-2-associated endocardial injury in patients with morphologically verified post-COVID-19 myocarditis, as well as to develop approaches to comprehensive therapy.Material and methods. The study included 18 patients with severe morphologically verified post-COVID-19 myocarditis (men, 9; 51,1±9,4 years; 35 to 66 years). Patients with prior verified myocarditis/myocardial infarction, rheumatic heart disease, and systemic immune diseases were excluded. The average time after COVID-19 was 6,5 [3.5; 10] months The diagnosis of myocarditis was confirmed by endomyocardial biopsy (including immunohistochemical examination with antibodies to CD3, CD20, CD45, CD68, and to SARS-CoV-2 antigens; polymerase chain reaction for SARS-CoV-2 RNA, DNA of cardiotropic viruses). The blood level of anticardiac antibodies was determined by indirect immunofluorescence. In addition, echocardiography, magnetic resonance imaging (n=8), cardiac multislice tomography (n=1), and coronary angiography (n=14) were performed.Results. Biopsy revealed active (n=12) and borderline (n=3) lymphocytic myocarditis, eosinophilic (n=2) and giant cell (n=1) myocarditis. In 4 patients, nonbacterial thrombotic endocarditis (NBTE) with parietal and intravascular thrombosis was diagnosed, and in one patient — infective endocarditis (IE) of the bicuspid aortic valve. Myocardial persistence of SARS-CoV-2 was detected in 72% of cases (in 3 patients — with NBTE; in 1 — with IE; in 9 — without endocarditis). Titers of anticardiac antibodies increased by 3-4 times in 94% of patients. Patients with endocarditis were characterized by larger heart chambers, lower ejection fraction (27,5±6,6 vs 36,0±13,4%), more severe pulmonary hypertension, and valvular regurgitation. Intraventricular thrombosis according to echocardiography/magnetic resonance imaging and cardiac embolism was not observed. Treatment in all patients included methylprednisolone at an average dose of 24 mg a day. In 10 patients, the result was monitored for at least 3 months as follows: the ejection fraction was 46,0±12,7% and 44,3±7,3% in patients with and without endocarditis, respectively.Conclusion. Endocarditis in patients with post-COVID-19 myocarditis was detected in 28% (1 patient — IE; 4 — NBTE). The key mechanisms of post-COVID-19 myocarditis and NBTE are long-term (up to 18 months) myocardial persistence of SARS-Cov-2 and the development of an autoimmune reaction. Endocarditis was diagnosed in more severe patients, including those with giant cell and eosinophilic myocarditis. The effectiveness of steroid therapy in combination with anticoagulants in patients with NBTE requires further study. In case of IE, steroids can also be used in the treatment of myocarditis (in combination with antibiotics and immunoglobulin).

Aim. To establish the prevalence of myocarditis and primary (genetic) cardiomyopathies (CMP) among patients in a cardiology hospital.Material and methods. Medical records of 671 patients of the cardiology department were analyzed. The diagnosis at admission and at discharge was recorded. The diagnoses were divided into 7 following categories: hypertension, coronary artery disease, heart disease, idiopathic arrhythmias, cardiomyopathy, myocarditis and others. Types of myocarditis and cardiomyopathy, the presence of arrhythmias and heart failure were also recorded.Results. Myocarditis was diagnosed in 194 (28,9%) patients, cardiomyopathy — in 76 (11,3%) patients, combination of cardiomyopathy and myocarditis — in 26 (3,9%) patients. Myocarditis with the development of arrhythmia and heart dilatation prevailed as follows: 47,4 and 41,2%, respectively. The most numerous CMPs were left ventricular noncompaction (n=30), non-inflammatory dilated CMP (n=13), hypertrophic CMP (n=10) and arrhythmogenic CMP of the right ventricle (n=9). In the group with idiopathic arrhythmias, 64,3% of patients were diagnosed with myocarditis, and 19,4% — with cardiomyopathy.Conclusion. The prevalence of non-coronary myocardial diseases among patients in a cardiology hospital is high and amounts to 40,2%. The presence of arrhythmias, heart failure or dilated cardiomyopathy may be a manifestation of non-coronary myocardial diseases, and requires a comprehensive examination aimed, in particular, at ruling out or verifying the diagnosis of myocarditis.

Aim.To analyze the register of pericarditis in a therapeutic clinic, to evaluate their nosological spectrum, to optimize approaches to diagnosis and treatment. Materials and methods.For the period 20072018, the register includes 76 patients with the diagnosis of pericarditis (average age 53.115.7 years, 2085 years, 46 female). Patients with hydropericardium were not included in the register. Diagnostic puncture of pericardium was carried out in 5 patients, pleural puncture in 11 patients. Morphological diagnostics included endomyocardial/ intraoperative biopsy of myocardium (n=4/2), thoracoscopic/intraoperative biopsy of pericardium (n=1/6), pleural puncture (n=5), transbronchial (n=1), thoracoscopic biopsy of intrathoracic lymph nodes (n=2), lung (n=1), supraclavicular lymph node biopsy (n=1), salivary gland (n=1), subcutaneous fat and rectum biopsy per amyloid (n=6/1). The genome of cardiotropic viruses, level of anti-heart antibodies, C-reactive protein, antinuclear factor, rheumatoid factor (antibodies to cyclic citrullinized peptide), antibodies to neutrophil cytoplasm were determined, extractable nuclear antigens (ENA), protein immunoelectrophoresis, diaskin test, computed tomography of lungs and heart, cardiac magnetic resonance imaging, oncologic search. Results.The following forms of pericarditis were verified: tuberculosis (14%, including in combination with hypertrophic cardiomyopathy HCM), acute / chronic viral (8%) and infectious immune (38%), including perimyocarditis in 77%, pericarditis associated with mediastinum lymphoma/sarcoma (4%), sarcoidosis (3%), diffuse diseases of connective tissue and vasculitis (systemic lupus erythematosus, rheumatoid arthritis, diseases of Horton, Takayasu, Shegren, Wegener, 12%), leukoclastic vasculitis, Loefflers endomyocarditis, AL-amyloidosis, thrombotic microangiopathy (1% each), HCM (8%), coronary heart disease (constriction after repeated punctures and suppuration; postinfection and immune, 4%), after radiofrequency catheter ablation and valve prosthetics (2%). Tuberculosis was the main causes of constrictive pericarditis (36%). Treatment included steroids (n=39), also in combination with cytostatics (n=12), anti-tuberculosis drugs (n=9), acyclovir/ganclovir (n=14), hydroxychloroquine (n=23), colchicine (n=13), non-steroidal anti-inflammatory drugs (n=21), L-tyroxine (n=5), chemotherapy (n=1). In 36 patients different types of therapy were combined. Treatment results observed in 55 patients. Excellent and stable results were achieved in 82% of them. Pericardiectomy/pericardial resection was successfully performed in 8 patients. Lethality was 13.2% (10 patients) with an average follow-up 9 [2; 29.5] months (up to 10 years). Causes of death were chronic heart failure, surgery for HCM, pulmonary embolism, tumor. Conclusion.During a special examination, the nature of pericarditis was established in 97% of patients. Morphological and cytological diagnostics methods play the leading role. Tuberculosis pericarditis, infectious-immune and pericarditis in systemic diseases prevailed. Infectious immune pericarditis is characterized by small and medium exudate without restriction and accompanying myocarditis. Steroids remain the first line of therapy in most cases. Hydroxychloroquine as well as colchicine can be successfully used in moderate / low activity of immune pericarditis and as a long-term maintenance therapy after steroid stop.

Chronic infectious-immune myocarditis of severe course can potentially be considered as a factor that aggravates the course of new coronavirus disease (COVID-19) and increases the risk of adverse outcomes. The interaction of chronic myocarditis and COVID-19 during long-term immunosuppressive therapy has not been studied. We present a description of a 35-year-old female patient with chronic infectious-immune myocarditis (morphologically confirmed, with a history of infarction-like onset and thromboembolic complications), who had continuous immunosuppressive therapy with methylprednisolone and mycophenolate mofetil. The patient also received new oral anticoagulants and tenofovir (for chronic HBV infection). COVID-19 (SARS-Cov-2 RNA+) was diagnosed in May 2020. Risk factors for the adverse course of coronavirus infection included severe obesity, heart failure, and life-threatening ventricular arrhythmias. Correction of immunosuppressive therapy (withdrawal of the cytostatic agent, administration of hydroxychloroquine) and therapy with levofloxacin, an interleukin-17 inhibitor (netakimab) were performed. The severity of pneumonia and respiratory failure was moderate despite high fever and high levels of inflammatory markers in the blood (including interleukin-6). Signs of exacerbation of myocarditis, increased levels of troponin T and anticardial antibodies (compared with the initial ones) were not found. It can be assumed that supportive immunosuppressive therapy for myocarditis has a positive effect on the course of coronavirus pneumonia and avoids exacerbation of myocarditis. Careful continuation of immunosuppressive therapy with temporary withdrawal of aggressive cytostatics can be recommended in chronic myocarditis. Further study of the features of the course of previous myocarditis and COVID-19 pneumonia is necessary.

Patients with chronic myocarditis have a high risk of an unfavorable course of the novel coronavirus disease (COVID-19) due to the ability of the SARS-Cov-2 virus to independently cause acute myocarditis, to have a direct and cytokine-mediated cytopathic effect on the myocardium, as well as immunosuppressive therapy. At the same time, the features of the interaction of chronic myocarditis and COVID-19 have not been studied. The article describes a 31-year-old patient with a 10-year history of chronic recurrent infectious-immune myocarditis, who was on long-term immunosuppressive therapy (methylprednisolone and azathioprine in the past, then hydroxychloroquine). In May 2020, a serologically confirmed COVID-19 diagnosis was made. There were risk factors for the unfavorable course of coronavirus infection: heart failure and a history of persistent atrial fibrillation, male sex. Basic therapy with hydroxychloroquine (with an increase in its dose to 800-400 mg/day), ceftriaxone, and levofloxacin was carried out. The severity of pneumonia was moderate, despite febrile fever and severe intoxication. No relapses of arrhythmias, respiratory or heart failure were observed. Minimal laboratory (some increase in anticardial antibody titers) and echocardiographic signs of exacerbation of myocarditis without an increase in troponin T levels were revealed, which quickly regressed. It can be assumed that the maintenance immunosuppressive therapy of myocarditis with hydroxychloroquine had a positive effect on the course of coronavirus pneumonia and made it possible to avoid recurrence of myocarditis. Further study of the features of the course of the pre-existing myocarditis and pneumonia in COVID-19 is necessary.

Aim. To analyze the difficulties of diagnosis and the clinical features of the Danon disease in women.Results. An observation of Danon disease in a woman aged 18 years with an uncomplicated family history is presented. The early development of atrial fibrillation (at the age of not more than 15 years) in combination with atrioventricular blockade against the background of regular sports was not attracted due attention for 3 years. The examination revealed: a moderate degree of left ventricular hypertrophy (up to 17 mm), its diffuse nature and simultaneous involvement of the right ventricle, signs of heart failure due to severe restrictive disorders with preserved ejection fraction. Cardiac magnetic resonance imaging data (non-specific late gadolinium enhancement) became the basis for the assumption of amyloidosis and the implementation of a myocardial biopsy. An erroneous diagnosis of cardiac amyloidosis according to myocardial biopsy was refuted during a second study, the PAS reaction revealed signs of storage disease. The diagnosis of Danon disease was verified using DNA diagnostics (c.731delG mutation was detected). Due to the presence of unsustained paroxysmal ventricular tachycardia and a high calculated risk of sudden death, cardioverter-defibrillator was implanted. The analysis of literature data on the frequency and the manifestation of Danon disease in women, the place of this disease in the structure of the causes of myocardial hypertrophy is given.Conclusion. Atrial fibrillation at a young age and left ventricular hypertrophy syndrome can develop due to primary myocardial diseases not well known in the practice of a cardiologist. They require an in-depth diagnostic search; their identification is critical for determining treatment tactics and prognosis.

Russian Society of Cardiology (RSC)With the participation: Eurasian Association of Therapists (EUAT), Society of Specialists in Heart Failure (OSSN), Russian Scientific Medical Society of Therapists (RNMOT), Russian Society of Pathologists, Russian Society of Radiologists and Radiologists (RSR)Endorsed by: Research and Practical Council of the Ministry of Health of the Russian Federation

Aim. To study the efficacy and safety of mycophenolate mofetil (MM) in combination with corticosteroids in the treatment of lymphocytic myocarditis in comparison with a standard combination of corticosteroids and azathioprine.Material and methods. The study included 46 patients aged 18 years and older with severe and moderate lymphocytic myocarditis (men, 34; women 12; mean age, 53,5±13,0 years). The diagnosis was verified using endomyocardial biopsy. Symptom duration averaged 9,5 [4; 20.25] months. All patients had class 3 [2,75; 3] heart failure (HF). The main group included 29 patients who received MM 2 g/day, including six patients — instead of azathioprine, which was canceled due to cytopenia (n=3) or insufficient effect (n=3). The comparison group included 17 patients who received azathioprine 150 [100; 150] mg/day. Patients of both groups also received methylprednisolone at a starting dose of 24 [24; 32] and 24 [24; 24] mg/day and standard HF therapy. In 7/2 patients, the parvovirus B19 genome was detected in the myocardium. In all cases, an increase in anticardiac antibody titers was evidence of immune activity. The average follow-up period was 24 [12; 54] months (at least 6 months).Results. The groups were completely comparable in age, initial characteristics and standard drug therapy. In both groups, a comparable significant increase in the ejection fraction (EF) was noted as follows: from 31,2±7,6 to 44,7±8,3% and from 29±9,1 to 46±11,9% (p

This article describes evolution of criteria for arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C). The novel diagnostic criteria for ARVD/C published in 2020 are analyzed in detail, among which biventricular and leftdominant arrhythmogenic cardiomyopathy are identified for the first time. The need to develop novel criteria was fed on the accumulation of new data on ARVD/C, in particular, significant advances in magnetic resonance imaging technologies. The novel criteria retained high sensitivity and specificity in relation to traditional right ventricular disease form and became more sensitive in relation to the biventricular and left-dominant arrhythmogenic cardiomyopathy.Nevertheless, the addition of left-dominant disease forms reduces the criteria specificity in general, since left ventricle involvement with a similar clinical performance can have different etiology that goes beyond the ARVD/C, even when mutations are detected in typical genes, which is demonstrated by case reports described in the article. Like the previous two versions, the novel criteria will be fully assessed only with a large sample of patients after their introduction into the routine cardiology clinical practice.

Aim. To assess the contribution of genetic and inflammatory factors to the development of arrhythmogenic right ventricular cardiomyopathy (ARVC).Material and methods. The study involved 54 patients with ARVC (age, 38,7±14,1 years; men, 42,6%; mean follow-up period, 21 [6; 60] months). All patients underwent electrocardiography (ECG), 24-hour ECG monitoring, echocardiography, determination of anticardiac antibodies and DNA of cardiotropic viruses in the blood, molecular genetic ARVC testing, as well as cardiac magnetic resonance imaging (n=49), high-resolution ECG (n=18), right ventricular endomyocardial biopsy (n=2), and autopsy (n=2).Results. Following four clinical types of ARVC were identified: I. Latent arrhythmic form: characterized by frequent premature ventricular contractions and/or nonsustained ventricular tachycardia (VT). II. Manifested arrhythmic form (n=11) — SVT/ventricular fibrillation (VF). III. ARVC with progressive heart failure (HF, n=8). IV. Combination of ARVC with left ventricular noncompaction (LVNC, n=8). Superimposed myocarditis was identified in 74%, 36%, 87,5% and 85,7% of patients in forms I-IV, respectively. Mutations were detected in 11%, 46%, 50%, and 38% of patients in forms I-IV, respectively. Clinical forms were stable: there was no transition from one clinical form to another during follow-up period.Conclusion. The contribution of genetic and inflammatory mechanisms to the clinical picture is different: in the latent arrhythmic form, the leading role belongs to inflammation; in the manifested arrhythmic form, the contribution of pathogenic mutations prevails, and in ARVC with progressive HF and in combination with LVNC, the contribution of genetic and inflammatory factors is equally important.

Aim. To study the nosological spectrum in the syndrome of primary left ventricle hypertrophy (PLVH) using morphological and genetic diagnostics and to compare the clinical course of true hypertrophic cardiomyopathy (HCM) and its phenocopy.Material and methods. Fifty five adult patients (29 men, 48.2±17.0 years) with PLVH (12 mm and more) were included. The exclusion criteria were athletic heart, hypertensive heart disease, severe valvular disease and other causes of secondary left ventricle (LV) hypertrophy. We performed 11 endomyocardial biopsy, 8 intraoperative biopsy, 1 study of explanted heart, 1 autopsy with virus investigation (real-time polymerase chain reaction) of the blood and myocardium. Mutational screening had included simultaneous sequencing of the MYBPC3, TAZ, TPM1, LDB3, MYL2, ACTC1, MYL3, MYH7, TNNI3 and TNNT2 genes based on NGS technology (Ion Torrent PGMTM) with following Sanger resequencing of potentially significant genetic variants. For patients with a phenotype of particular genetic syndrome the Sanger sequencing of target gene(s) for performed first. Clinical examination had included electrocardiography, Holter monitoring, echocardiography, coronary angiography, computer tomography/magnetic resonance imaging (by indication). The mean follow-up was 8 [3;32] month.Results. Isolated HCM was found in 28 patients, and 10 have a combination of HCM and noncompaction myocardium (NCM). Mutations in the MYH7 and MYBPC3 genes were detected in six cases. In 17 cases (30.9%) the non-sarcomeric causes of LVHS were detected. Three patients had Fabry disease, 2 ‒ had Danon disease, in 10patients we found amyloidoses, in 1 – Friedreich ataxia, and 1 patient was diagnosed with LEOPARD syndrome (all cases were confirmed by DNA diagnostics). Genotype-positive diagnosis was established in 23.6% of patients. In patients with HCM were significantly more frequent asymmetric septal hypertrophy with obstruction and muscle bridges, in other forms of primary hypertrophy – right ventricular hypertrophy, low QRS voltage, QS complexes and increasing of ejection fraction (EF) (55.7±12.5% vs 62.5±10.1% in HCM, p=0.08). The morphologic signs of myocarditis were in 46.7% of patients with HCM detected: in 3 patients with NCM and in 4 patients with isolated HCM. The viral genome in the myocardium was in 11 patients with HCM (73.3%) detected, previously human herpes virus type 6 (it was correlation with myocarditis) and parvovirus B19. Eleven patients died due to a stroke/heart failure without no significant differences between patients with HCM and phenocopy.Conclusion. The spectrum of causes of the primary left ventricular hypertrophy is very wide. The frequency of myocarditis associated with sarcomeric HCM was 46.7%. When lower EF and heart failure in patients with HCM can be result of myocarditis, in patients with storage disease they are the result of disease itself.

Loeffler's endocarditis remains is a very rare disease, develops due to eosinophilic inflammation predominantly of the endocardium with an outcome in fibrosis and massive thrombus formation and. He is generally characterized by an unfavorable prognosis. Clinical case of a 42-year-old patient with Loeffler endocarditis is presented. The development of the disease was preceded by a polyvalent allergy, mild dry eye syndrome and pansinusitis with a single eosinophilia of blood up to 16%. The reason for the hospitalization was the appearance of biventricular heart failure. During the previous year, the level of blood eosinophils remained normal, a threefold increase in the level of eosinophilic cationic protein was observed once. A 20-fold increase in the pANCA level, a 2.5-fold increase in the level of antibodies to DNA, an antibody to the nuclei of cardiomyocytes 1:160 were detected. The diagnosis was made on the basis of electrocardiography data (low QRS voltage, atrial hypertrophy), echocardiography, multispiral computed tomography and magnetic resonance imaging of the heart (thickening and delayed contrasting of the endocardium, massive thrombosis of the left ventricular apex with obliteration of its cavity, encapsulated fluid in the pericardium with compression of the right ventricle). Systolic dysfunction, severe signs of restriction and arrhythmias were absent. Trombectomy, tricuspid valve plasty, pericardial resection, suturing of an open oval window were performed. Signs of active inflammation with single eosinophils, vasculitis, perimuscular sclerosis, endocardial sclerosis were detected in morphological and immunohistochemical studies of endo-, myo-, pericardium. Viral genome was not found. The therapy with methylprednisolone 24 mg/day, azathioprine 75 mg/day was started. Six months after the operation, the symptoms of heart failure are completely absent, the thrombosis did not recur.

Aim. To study clinical forms of noncompact myocardium (NCM) in adults, the features of their manifestation, course and progression.Material and methods. The study included 116 adult patients with NCM of the left ventricle (LV) (67 men, mean age 46,3±15,1 years) and 42 patients with increased LV trabecularity (24 men, mean age 43,5±15,2 years). The mean LV end-diastolic diameter was 6,0±0,8 and 5,9±1,1 cm, LV ejection fraction was 38,6±14,0% and 44,6±18,3%, respectively. NCM was diagnosed using echocardiography, multispiral computed tomography (n=77) and magnetic resonance imaging (n=51), increased LV trabecularity was diagnosed according to echocardiography, multispiral computed tomography (n=11), multispiral computed tomography (n=24). DNA diagnostics was carried out according to the NGS method, followed by Sanger sequencing. The examination included the determination of anticardial antibodies, the genome of cardiotropic viruses by PCR, coronary angiography (n=29/2), scintigraphy (n=27/4). A morphological study of the myocardium was performed on 22/6 patients with NCM/increased LV trabecularity (14/6 endomyocardial biopsy, 1 intraoperative biopsy, 3 explanted heart studies, 6 autopsies).Results. Pathogenic mutations were found in 12 (10,3%) patients (MYH7, MyBPC3, LAMP2, DES, DSP, TTNgenes), variants of uncertain clinical significance (VUCS) — in other 5 (4,3%) patients; we detected VUCS in 1 patient with increased LV trabecularity. Familial cardiomyopathy may be diagnosed in 24 patients (22%). The combination of NCM with congenital heart defects was diagnosed in 11 (9,5%) patients. We identified six clinical variants of NCM: asymptomatic (2%), arrhythmic (15%), ischemic (7%), NCM in patients with dilated cardiomyopathy (42%), NCM in patients with acute/subacute myocarditis (12%) and in combination with other primary cardiomyopathies (22%) — hypertrophic cardiomyopathy, arrhythmogenic right ventricular dysplasia, restrictive cardiomyopathy, primary myodystrophy, cardiac sarcoidosis, Danon disease. Myocarditis was diagnosed in 51,7% of patients with various forms of NCM and in 59,5% of patients with increased LV trabecularity. The frequency of the main clinical manifestations of NCM (chronic heart failure, various cardiac arrhythmias, thromboembolic complications) and outcomes varied in groups of patients with different variants of the NCM course. In patients with increased LV trabecularity, similar clinical variants were noted with a less severe myocardial dysfunction, rare arrhythmias and embolism. A significant improvement in dynamics of EF and LV end-diastolic diameter was noted only in the group of patients with acute/subacute, most of which received basic myocarditis therapy. In other groups, there was an unreliable improvement.Conclusion. NCM can be detected in a patient of any age with a previously diagnosed heart disease (coronary heart disease, arterial hypertension, congenital heart defects, cardiomyopathy, myocarditis, etc.), and in the absence of any symptoms. Stable clinical forms are characterized by stability over time with a tendency to improvement against the background of complex medical therapy.

Aim. To evaluate the effectiveness of myocarditis therapy depending on the diagnosis approach (with or without myocardial biopsy).Material and methods. The study included 83 patients ≥18 years old with severe and moderate myocarditis (25 women and 58 men; mean age, 45,7±11,7 years), established by myocardial biopsy (group 1, n=36) or by a non-invasive diagnostic algorithm (group 2, n=47), for which immunosuppressive therapy (IST) was carried out. Inclusion criteria were left ventricular (LV) end-diastolic dimension >5,5 cm and ejection fraction (EF)

Aim. To study the clinical spectrum of infectious-immune pericarditis, the potential for their invasive and non-invasive diagnosis, as well as long-term treatment with hydroxychloroquine (in comparison with other baseline therapy options).Material and methods. The study included 44 patients with infectious-immune pericarditis (28 women and 16 men aged 49,4±13,3 years). Patients with transudate and specific types of pericarditis were excluded. Levels of C-reactive protein and anticardiac antibodies were determined Multislice computed tomography of the lung (n=23) and heart (n=16), cardiac magnetic resistance tomography (n=9), scintigraphy (n=14), and if necessary — immunoelectrophoresis, DNA testing, Diaskin-test. Pericardio- and thoracentesis were performed in 3/3 patients, thoracoscopic pericardial biopsy — 1, endomyocardial biopsy — 7. The follow-up period was 14,5 [3; 39,5] months.Results. Isolated pericarditis was diagnosed in 10 patients (22,7%), myopericarditis — in 34 (77,3%). In 38 patients, pericarditis was exudative: in 24 (63,2%) with a small effusion (20 mm). Fibrin was detected in 18,2% of patients. Pericardial effusion was assessed as acute in 4, subacute — in 8, chronic — in 26 patients. The connection between the disease onset and infection was found in 56,8% of patents, and inflammatory blood changes — in 59,1%. In 80%, the punctate was lymphocytic; endomyocardial biopsy confirmed active/ borderline (5/2) lymphocytic myocarditis (virus-positive — in 3 patients). Anticardiac antibody titers were increased in 88,2%. Baseline therapy included NSAIDs (34,1%), colchicine (27,3%), hydroxychloroquine (43,2%), methylprednisolone (56,8%, 16 [16; 21] mg/day), azathioprine (20,5%). The treatment scheme was selected individually. In most cases, combined therapy was carried out. The results of treatment were assessed in 36 patients: an excellent effect was noted in 16 (44,4%) patients, stable effect — in 13 (36,1%), no stable effect — in 7 (19,4%). There were no cases of constrictive pericarditis, acute relapses, cardiac tamponade. Mortality of 6,8% was associated with myocardial injury.Conclusion. Criteria for the diagnosis of infectious-immune pericarditis were proposed. An increase in the titer of anticardiac antibodies was noted in all types of the disease. Prescription of corticosteroids is justified in many cases, including in combination with colchicine, cytostatics, hydroxychloroquine. Hydroxychloroquine monotherapy is effective for subacute/chronic pericarditis with moderate effusion.

Aim. To assess the differential diagnosis in a patient with a combination of coronary artery disease and myocarditis and the results of stepwise treatment (including immunosuppressive therapy (IST), and coronary stenting).Material and methods. A 56-year-old female patient with hypertension, obesity (body mass index, 31,6 kg/m2), diabetes and psoriasis developed shortness of breath after a respiratory viral infection. Primary echocardiography revealed left heart dilatation, ejection fraction (EF) of 21%. Coronary angiography revealed anterior descending artery stenosis of 75%, circumflex artery — 80%, right coronary artery (RCA) — 70%. RCA stenting was performed and cardiovascular and diuretic therapy was started. However, shortness of breath and low exercise tolerance persisted.Results. In the blood test, anti-endothelial cell antibodies were 1:320, anticardiomyocyte and anti-smooth muscle antibodies — 1:80, anti-cardiac conduction system fibers — 1:320 (N≤1:40). During myocardial perfusion scintigraphy with computed tomography, an uneven distribution of the indicator was noted. Signs of myocardial scarring and indications for further revascularization were not revealed. Cardiac magnetic resonance imaging confirmed a decrease in left ventricular (LV) contractility (LVEF 37%) and moderate dilatation. Biopsy was not performed due to dual antiplatelet therapy. The condition is regarded as infectious-immune myocarditis. IST was started with azathioprine 150 mg/day. We noted dyspnea relief and a stable increase in LVEF to 50-52%. The clinical course was complicated by sick sinus syndrome with pauses up to 6 seconds and presyncope; a pacemaker was implanted. After 5 years from the onset of IST, dyspnea episodes reappeared without exacerbation of myocarditis. As their cause, ischemia was diagnosed due to the progression of coronary atherosclerosis. Symptoms regressed after repeated coronary stenting.Conclusion. The presence of moderate coronary atherosclerosis without signs of ischemia and myocardial infarction should not be considered as the only cause of severe systolic myocardial dysfunction. Diagnosis and treatment of myocarditis in combination with coronary artery disease is carried out according to the standard principles and can improve LV systolic function and control the heart failure symptoms.

Aim. To study the efficacy of plasmapheresis as the main type of pathogenic treatment or in combination with immunosuppressive therapy in patients with dilated cardiomyopathy (DCMP) and arrhythmias of immune-inflammatory nature.Material and methods. The main group included 20 patients with arrhythmic myocarditis (with premature supraventricular / ventricular contraction >3000/day, n=3/8, atrial fibrillation (AF) n=9) and 14 patients with DCMP syndrome (enddiastolic volume (EDV) left ventricle (LV) 6,3±0,6 cm, ejection fraction (EF) 33,5±8,1%). The inclusion criterion was an increase of at least 2 types of anti-cardiac antibodies titers ≥ twice. Myocarditis is diagnosed using myocardial biopsy, magnetic resonance imaging, multispiral computed tomography, scintigraphy, coronary angiography. We used a course of discrete plasmapheresis. The comparison group included 26 patients with an arrhythmic myocarditis and 19 with DCMP syndrome (EDV 6,6±0,8 cm, EF 32,6±7,3%), which plasmapheresis was not used. Dynamics was assessed at 6 and 12 months.Results. In groups of patients with arrhythmias and DCMP, a significant decrease in anti-cardiac antibodies titers was observed immediately after plasmapheresis and in control studies (p0,05) at a dose of 8 [4; 16] and 16 [10; 24] mg per day, respectively, p>0,05. In patients with DCMP in the main group, a significant increase in EF (p

The goal is to present the possibilities of diagnosis verification, the features of the clinical picture of tuberculous pericarditis in the therapeutic clinic and the results of its treatment. Materials and methods. The paper presents clinical observation and a general analysis of 10 cases of tuberculous pericarditis in patients aged 31-79 (mean age 58.0 ± 15.1 years), 6 women and 4 men. Diagnostic puncture pericardium was performed on two patients, pleural puncture - on three Thoracoscopic biopsy of hilar lymph nodes and lung (n=1), pleura (n=1), supraclavicular lymph node biopsy (n=1). Dyskin test was carried out, as well as sputum examination, multispiral computed tomography, oncological search. Results. A 31-year-old patient with a massive effusion in the pericardial cavity, pleural lesion, arthritis of the left knee joint, whose results of the pericardial effusion and sputum were not diagnosed, tuberculosis was detected only with thoracoscopic biopsy of the lung and intrathoracic lymph nodes; the treatment via prednisolone and subtotal pericardectomy was performed. Among 10 patients with MSCT of the lung, changes were noted in general, but in only one case they were highly specific. Diaskin test is positive in 70%. In the study of punctata, bronchoalveolar flushing, Koch bacteria were not detected; at sputum in microscopy and biological sample BC was detected in two patients. The lymphocytic character of effusion in the pericardium / pleura is noted in 4 out of 5 cases. At a biopsy of lymphonoduses and a lung at 2 patients the picture of a granulomatous inflammation with a caseous necrosis. Pericarditis was predominantly large (from 2 cm and more) effusion, signs of constriction were noted in 50% of patients. Conclusion. Tuberculosis is one of the frequent causes of pericarditis in the Moscow therapeutic clinic. The most lymphocytic effusion with fibrin and the development of constriction. The negative results of all laboratory tests for tuberculosis do not exclude a diagnosis, It is necessary to use invasive morphological diagnostics, including thoracoscopic biopsy.

The aim of case presentation. To present a typical case of systemic autoimmune disease related to extremely aggressive medical alterations of the immune status which were carried out not only for life-saving indications, but for cosmetic reasons as well. Summary. The patient (32-year-old female) had a history of persistent irregular febrile fever for two years, renal disease (tubulointerstitial nephritis with papillary necrosis), hemorrhagic vasculitis-like skin involvement and mediastinal lymphadenopathy. Patient underwent multiple investigations aimed for ruling out infectious causes of the disease that yielded negative result, repetitive courses of antibiotics provided transient positive effect at onset of disease. Five years before the origin of symptoms patients underwent sleeve gastrectomy with biliopancreatic shunting for obesity (body weight - 130 kg, BMI - 43.4 kg/m2), cholecystectomy and appendectomy. After that within a year patient lost 55 kg of body weight and developed severe symptoms of malabsorption. In 1.5 years after the fever onset bilateral mammaplasty with silicone implants was carried following which the fever became constant. At transthoracic biopsy of mediastinal lymph nodes demonstrated the pattern of Wegener-type ANCA-negative necrotizing granulomatous vasculitis of small arteries (PCR for mycobacteria and acid fast stain were negative). Despite provided corticosteroid and cyclophosphan treatment, the disease progressed (focal affection of the lungs) and the patient died of brain stem hemorrhage. Autopsy revealed the signs of vasculitis with involvement of the brain, skin, mediastinal lymphnodes, lungs, as well as the signs of chronic inflammation, atrophy of breast gland lobules, embolization of the focal lymphatics by the implant substance, ulceronecrotic enteritis of defunctioning bowel, severe liver steatosis with the chronic vitamin deficiency. Conclusion. The relation of atypical necrotizing vasculitis with yet undescribed clinical presentation with previous bariatric surgery and long-standing malabsorption is highly probable; application of silicone implants with adjuvant-associated immune response were additional resolving factors. Incomplete effect of immunosuppressive therapy indicates the dominant role of autoinflammation in the course of disease. Selection of patients for bariatric and cosmetic surgery should be more careful and involve estimation of the general and immune status of the body.

Hypertensive heart disease with biventricular cardiac failure is not common in clinical practice. This diagnosis requires an extensive diagnostic search. We present the clinical case of the male patient of 38 aged. He was admitted to the clinic with heart failure 3-4 NYHA class. EchoCG revealed symmetric hypertrophy of the left ventricle up to 18 mm without its dilatation, a decrease in ejection fraction up to 42%, restrictive hemodynamics, overload of the right chambers, severe pulmonary hypertension (60 mm Hg). The clinical status included persistent arterial hypertension (180-220 and 120-150 mm Hg), effusion in both pleural cavities and pericardium, ascites, renal failure. During examination (multispiral computed tomography, magnetic resonance imaging, scintigraphy with 131I-MIBG), bladder paraganglioma was diagnosed (normatenafrin 1468 μg/day). The resection of the tumor was performed, according to immunohistochemical research – neuroendocrine carcinoma, G1. After 3 months a partial regression of hypertension and cardiac failure was observed with the preservation of a high level of creatinine. The criteria and differential diagnosis of the hypertensive heart disease and the syndrome of primary myocardial hypertrophy, diagnostics of the urinary bladder paraganglioma, complex mechanisms of myocardial damage within the pheochromocytoma and its prognosis are discussed.

Aim. To evaluate the prevalence of myocarditis in adult patients with non-compaction myocardium (NCM) of the left ventricle (LV), and its influence on the disease course, results of treatment and outcomes.Material and methods. To the study, 103 adult patients included, with NCM, 61 males, mean age 45,6±14,9 y. o. (from 18 to 78). Mean end diastolic LV size was 6,0±0,8 сm, EF LV 38,8±14,5%. Diagnosis of NCM had been done by echocardiography, multispiral computed tomography (n=81) and magnetic resonance tomography (n=39). DNA-diagnostics was performed by NGS method with further Senger sequencing. Pathogenic mutations were found in 9% of patients in the genes MYH7, MyBPC3, LAMP2, DES, DSP, TTN. The investigation also included anticardiac antibodies, genome of cardiotropic viruses by PCR, coronary arteriography (n=26), scintigraphy (n=25). Morphological assessment of the myocardium was done in 19 patients (12 endomyocardial biopsies), 1 intraoperation biopsy, 3 explanted hearts, incl. 2 after biopsy, and 5 autopsies. Mean follow-up 12 [2; 32] months.Results. Myocarditis was found in 53,4%, incl. virus-positive in 32,7% of those, with morphology done for 19 patients (active myocarditis in 10, borderline in 6; with minimal signs of activeness in 3). Viral genome in myocardium found in 8 patients (42,1%). The prevalence of myocarditis 44,4% in an arrhythmic variant of NCM, 12,5% in chronic ischemic, 57,5% in dilation cardiomyopathy, 50,0% in NCM patients with other cardiomyopathies. Special cases were acute/subacute myocarditis in NCM (10,7% of all), acute necrosis (infarction) in 4,9%. Comorbidity with myocarditis in NCM led to significantly more severe LV dysfunction (CHF FC 2 [1; 3] v 1,75 [0; 2], p0,05). Only in myocarditis patients, as a result of basis (antiviral, immune suppression) and cardiotropic therapy there was significant increase of EF (in acute myocarditis from 25,4±7,9 to 38,6±9,5%, p

Aim. To classify established clinical types of the right ventricle arrhythmogenic dysplasia (RVAD) taken a variety of genetic and inflammatory mechanisms, and to analyze the specifics of differential diagnostics and management of the respective types.Material and methods. Main group consisted of 50 patients with evident (n=26), probable (n=13) and possible (n=11) RVAD diagnosis, mean age 38,1±14,6 y. o., males — 20 (40%), follow up time 13,5 [4; 34] months. Comparison group consisted of 58 patients with some of the RVAD criteria insufficient for evident diagnosis. All patients underwent ECG, Holter ECG 24 hours, EchoCG; in the main group additionally — DNA-diagnostics (n=46), cardiac MRI (n=44), high definition ECG (n=16), endomyocardial biopsy of the RV (n=2), autopsy (n=2). In comparison group, MRI was done in 32 patients, biopsy to 7, and in 1 case — autopsy.Results. Based upon the clinical data and specifics of the disease course, 4 types of established clinical RVAD were selected, that do not tend to overlap: latent arrhythmic (50% patients), manifest arrhythmic (20%), RVAD with predominant biventricular chronic heart failure (CHF, 16%), and RVAD with non-compaction left ventricle myocardium (14%). The development of one or another type is based on genetic factors, as on comorbid myocarditis (in percent in the following, respectively). In diagnostics of the latent arrhythmic type (frequent right ventricle extrasystoly, VE and/or non-sustained right ventricular tachicardia, VT) the key role played female sex, syncopes in anamnesis (16%), family history of sudden death (12%), ECG-criteria and positive results of DNA diagnostics (24%). For manifest arrhythmic type (sustained VT) — sudden death family anamnesis (in 20%), MRIcriteria (enlarged RV with lower EF), ECG-criteria and positive DNA tests (50%). For RVAD with progressing CHF — sustained VT (50%), syncopes (37,5%), predominance of RV failure with its severely reduced EF (25,7±15,0%), major MRI- and ECG-criteria, decreased QRS-voltage and positive DNA test (38%). Comorbidity of RVAD and non-compaction myocardium differ by frequent VE, aggressive VT (57,1%), syncope (42,9%) and CHF with significantly lower than in DCMP EF LV. Mortality rate in I-IV types was, respectively, 0%, 10%, 25%, 14,3%, and relevant shocks in 8 of 13 (61,5%) patients with ICD.Conclusion. It is worthy to use the proposed RVAD classification in clinical practice to define the spectrum of diagnostical and management events and assess the individual patient prognosis.

Aim. To evaluate the exact efficacy of the implantable cardioverter-defibrillators (ICD) and combination devices (CRT-D), and to evaluate their influence on the rate of sudden death and overall mortality in patients with the dilation cardiomyopathy syndrome (DCMP), and to clarify the selection criteria for implantation.Material and methods. Totally, 220 DCMP patients investigated: 66 (30%) of them (mean age 48,5±12,8 y. o., 47 males) underwent implantation of ICD (n=37) and CRT-D (n=29), 154 (70%) patients were in comparison group (mean age 47,1±12,4 y. o., 104 males). In 60 patients (93,9%) the devices were implanted for primary prevention of sudden cardiac death (SCD). Follow-up lasted for 16 [6; 37] months. As primary endpoints, the following parameters were used: “death+transplantation”, mortality, SCD, “SCD+shock” and “death+transplantation+proper shocks of the defibrillators”.Results. Mortality in all DCMP patients was 19,1%, “death+transplantation” — 21,4%, SCD — 2,7%. There were no significant differences by these values in patients with both devices (19,7%; 22,7% and 1,5%), ICD (21,6%; 24,3% and 2,7%), CRT-D (17,2%; 20,7; and 0) and patients with no devices (18,8%; 20,8% and 3,2%). Significantly higher rates by “SCD+shocks” (18,2% v 3,2%, p

Aim. To investigate whether intravenous contrast-enhanced multislice spiral computed tomography (computed tomography) (MSCT) versus myocardial morphological examination can diagnose myocarditis and the non-inflammatory causes of dilated cardiomyopathy (DCM) and evaluate prognosis in patients with the latter. Subjects and methods. A study group consisted of 130 patients, including 95 men (46.8±11.9 years), with DCM (mean left ventricular (LV) end-diastolic dimension (EDD), 6.6±0.8 cm; mean LV ejection fraction (EF), 29.8±9.3%; NYHA functional class (FC) III (II; III)). All the patients underwent intravenous contrast-enhanced 320-slice CT of the heart; myocardial morphological examination was made in 48 patients (endomyocardial biopsy in 29 patients, intraoperative biopsy in 7, and autopsy in 9, and study of the explanted heart in 3). In addition, cardiotropic viral DNA in the blood and myocardium and the level of anticardiolipin antibodies were determined; echocardiography (in all the patients), scintigraphy (n = 45), magnetic resonance imaging (MRI) (n = 21), and coronary angiography (CG) (n = 46), and a genetic consultation were performed. A comparison group comprised 20 patients, including 14 men (69.3±9.2 years), with coronary atherosclerosis (40% or more stenoses) according to MSCT findings in the absence of criteria for DCM (mean LV EDD, 4.8±0.5 cm; mean LV EF, 59.4±4.6%). Results. Morphological/comprehensive examination showed that myocarditis as a cause of DCM was diagnosed in 76 (65%) patients; its concurrence with genetic cardiomyopathies was in 17 more patients (17%). MSCT of the heart revealed lower accumulation areas in 2 (1.5%) patients (type 1 based on the proposed rating scale), delayed myocardial contrast agent accumulation (DMCAA) in 81 (62.3%): subendocardial accumulation (type 2) in 8, intramyocardial accumulation in 4 (type 3), subepicardial accumulation in 52 (type 4), and transmural accumulation in 15 (type 5); DMCAA was not noted in 49 patients. DMCAA was not found in the comparison group. As compared with biopsy, the sensitivity, specificity, predictive value of positive and negative results of the tests in detecting active myocarditis for all the types of DMCAA were 77.4, 47.1, 72.7, and 53.3%, respectively; those for types 3-5 of DMCAA were 77.4, 52.9, 75.0, and 56.3%; those in detecting all the morphological types of myocarditis were 68.3, 28.6, 84.8, and 13.3%, and those for types 3-5 were 65.9, 28.6, 84.4, and 12.5%, respectively. Comparison of the data of MSCT and those of comprehensive examination in all the patients with DCM, the diagnostic significance in detecting myocarditis for all the types of DMCAA was 70.6, 67.9, 88.9 and 38.8%, respectively; that for DMCAA types 3-5 was 60.8, 67.9, 87.3, and 32.3%. In the study group, MSCT also identified the non-compacted myocardium (n = 31 (23.8%)), coronary atherosclerosis (n = 31 (23%)), which is confirmed by CG findings in 15 patients. The patients with DMCAA significantly more frequently showed a relationship with previous infection, acute onset, significantly higher NYHA FCs, end-diastolic and end-systolic LV volumes, and insignificantly lower LV EF. During a mean follow-up periods of 12 (6; 37.25) months, the overall mortality rate was 17.7% (23 deaths); the death + transplantation index was 20% (n = 26). All the types of DMCAA were found to be significantly related to prognosis: in the DMCAA group, the mortality rate was 21.5% versus 7.8% in the non-DMCAA group (odds ratio 3.22; 95% confidence interval, 1.02 to 10.21; p < 0.05). Conclusion. MSCT with the assessment of delayed contrast enhancement (and simultaneous CT coronary angiography) can be used for the non-invasive diagnosis of myocarditis in patients with DCM, including that in the presence of contraindications to MRI. DMCAA correlates with the presence of myocarditis, its activity, the degree of functional disorders, and prognosis.

Objective. To determine criteria for choosing management tactics in patients with ventricular arrhythmias (VA) in the absence of structural heart disease from the point of view of physicians and patients in clinical practice and to compare the immediate results of antiarrhythmic drug therapy (ADT) and radiofrequency ablation (RFA) with the trends in arrhythmic syndrome in the non-treatment group. Subjects and methods. Examinations were made in 90 patients (23 men and 67 women) (mean age, 44 (31; 57) years) with VA in the absence of structural heart disease. Preference was given to RFA (n = 32 (36%)), ADT (n = 37 (41%)), and follow-up tactics (n = 21 (23%)). At baseline and 1 month, Holter ECG monitoring was done; quality of life (QOL) was assessed; and anxiety and depression levels were detected using the SF-36 and HADS questionnaires. In addition, 71 physicians were surveyed about their preferences to the treatment of VA in individuals without structural heart disease. Results. In the total group of patients, VA was unambiguously accompanied by the symptoms only in 47%. The signs of anxiety and depression were identified in 41 and 14% of cases, respectively. The efficiency of RFA was comparable to that of ADT (p > 0.1): a positive antiarrhythmic effect was observed in 71.9% of the patients in the RFA group and in 67.6% in the ADT group. During one month, 38.1% of the patients in the follow-up group showed a spontaneous substantial reduction in the number of ventricular premature beats (VPBs) or disappearance of unstable ventricular tachycardia (UVT), which met the criteria for a positive effect. At baseline, the QOL indicators on a social functioning scale in the RFA group were worse than those in the ADT group. At the same time, most QOL indicators in the patients who have chosen a wait-and-see tactic were significantly higher than those in the RFA and ADT subgroups. The patients treated with ethacyzin in the ADT group more frequently achieved a positive effect. In the interviewed physicians’ opinion, the choice of a tactic depended on the impact of arrhythmia on health status (68%), the number of VPBs per day (61%), and the presence of UVT (56%). RFA or ADT was most often recommended when there were 10,000-15,000 or more VPBs per day ((49 and 35% of the respondents, respectively). 46.5% of the respondents stated that β-blockers were the drug of choice for idiopathic frequent VPBs. Only 30% of the respondents considered it appropriate to restrict to a follow-up in the presence of asymptomatic VPBs. Conclusion. Patient management in clinical practice generally complies with the current guidelines; however, much importance is attached to the severity of arrhythmia (the number of VPBs per day, the presence of UVT) in addition to the presence of symptoms. In the opinion of most physicians, the initiation of treatment is justified when there are 10,000-15,000 and more per day. QOL assessment may be promising in choosing the optimal management tactics for these patients. Treatment should not be initiated immediately in patients with a high level of QOL, especially in those with arrhythmia lasting less than 12 months, by taking into account that there can be a spontaneous improvement in 38% of cases within the next month. The immediate results of ADT and RFA are comparable in patients with VA in the absence of structural heart disease. The Class IC antiarrhythmic drug ethacyzin is the most effective agent that ensures positive changes in arrhythmic syndrome in 66.7% of cases with the rate of side effects being in 17.8%.

Aim. To determine the diagnostic value of different clinical, laboratory, and instrumental signs in the diagnosis of myocarditis in patients with the picture of idiopathic arrhythmias, dilated cardiomyopathy (DCM) and in a comparison group when comparing with myocardial morphological examination. Subjects and methods. A study group included 100 patients (35 women; mean age, 44.7±12.5 years) with idiopathic arrhythmias (n=20) and DCM as a syndrome (n=100). All underwent myocardial morphological examination: endomyocardial biopsy (EMB) (n=71), intraoperative biopsy (n=13), study of the explanted heart (n=6), and autopsy (n=11). A comparison group consisted of 50 patients (25 women; mean age, 53.7±11.7 years) with non-inflammatory diseases of the heart (left ventricular end-diastolic dimension 50%) who underwent open-heart surgery (n=47), EMB (n=2), or autopsy (n=1). The investigators also performed polymerase chain reaction for cardiotropic viral DNA in the blood and myocardium, anticardiac antibody (ACA) identification, myocardial scintigraphy (n=26), coronary angiography (n=47), magnetic resonance imaging (MRI) (n=25), and multislice computed tomography of the heart (n=45). The diagnostic value of the extended spectrum of clinical, laboratory, and instrumental markers for myocarditis was estimated. Results. Active/borderline myocarditis was diagnosed in 76% of the patients in the study group (75.5% in the arrhythmia subgroup and 76.3% in the DCM one) and in 24.3% of those in the comparison group (p

Aim. To evaluate the efficiency of immunosuppressive therapy (IST) in virus-negative (V–) and virus-positive (V+) patients with lymphocytic myocarditis (LM). Subjects and methods. 60 patients (45 males) (mean age 46.7±11.8 years) with dilated cardiomyopathy (mean left ventricular (LV) end diastolic size (EDS) 6.7±0.7 cm; ejection fraction (EF) 26.2±9.1%) were examined. The diagnosis of active/borderline LM was verified by right ventricular endomyocardial biopsy in 38 patients, by intraoperative LV biopsy in 10, in the study of explanted hearts from 3 patients and at autopsy in 9. The investigators determined the genomes of parvovirus B19, herpes viruses types 1, 2 and 6, Epstein—Barr (EBV), zoster, and cytomegalovirus in the blood and myocardium and, if antibodies were present in the blood, hepatitis B and C viruses, as well as antibodies against antigens in the endothelium, cardiomyocytes and their nuclei, smooth muscles, fibers of the conducting system. IST was used in terms of histological, immune, and viral activities. IST was performed in 22 V+ patients (Group 1) and in 24 V– patients (Group 2); this was not done in 10 V+ patients (Group 3) and V– patients (Group 4). IST comprised methylprednisolone at a mean dose of 24 mg/day (n=40), hydroxychloroquine 200 mg/day (n=20), azathioprine at a mean dose of 150 mg/day (n=21); antiviral therapy included acyclovir, ganciclovir, intravenous immunoglobulin (n=24). The follow-up period was 19 (7.3—40.3) months. Results. The viral genome was detected in the myocardium of 32 patients who made up a V+ group. The degree of histological activity did not differ in relation to the presence of viral genome in the myocardium. The degree of immune activity (anticardiolipin antibody titers) in the V+ patients was as high as that in V– ones. At baseline, the V+ patients had a significantly higher LV EDS and a lower EF than the V– patients. Overall, IST only could lead to a significant increase in EF (from 26.5±0.9 to 36.0±10.8%; p

The primary diagnosis of “dilation cardiomyopathy” is syndromal, and demands clarification of nosological origins. In the article, the specifics of such diagnostics is discussed. Clinical case is provided that illustrates the ways of management, diagnostics and treatment of the essential (primary, genetically determined) dilation cardiomyopathy. Patient of 22 year old with no family history, at the ages 20 and 21 y.o. had cardioembolic strokes. Paroxysmal atrial fibrillation was found, raised creatine kinase levels up to 349-1045 U/L, decreased ejection fraction 17%. Heart failure rapidly progressed. In endomyocardial biopsy there was homogenisation of cardiomyocytes, subendocardial lipomatosis with borderline virus-negative myocarditis. By Senger direct sequencing, the novel variant p.E372 in gene LMNA was found, heterozygous. Implantation of CRT-D was done, and in 4 months — cardiac transplant.

Takayasu’s disease (nonspecific aortoarteritis) affects the absolute majority of 20—40-year-old women; its most common manifestations include aortic arch branch lesions. The specific features of the described case is the male gender of the patient, signs of mesenchymal dysplasia, a concurrence of tuberculosis, pulmonary hemorrhage, and myocarditis at onset, the presence of IgG4, peculiar ocular symptomatology, and its rapid regression after initiation of therapy.

The problem of non-coronary diseases of myocardium (NCDM) and, particularly, the classification of them, is one of the most complicated in cardiology and internal medicine. Until recently, there is no unified official classification in Russia, and there are various in use for practical and scientific purposes: American (2006), European (2008) and the latest issue by Paleev N.R. and Paleev F.N. (2008). The article critically examines and compares these classifications. The analysis provided, of evolution of the term “cardiomyopathy” (CMP), European and Russian classifications of myocarditis and MOGE(S) of 2014; the principles for an “ideal” classification are proposed, at modern stage, and our original synthetic variant.Main aspects of the proposed classification are introduction of syndromal approach (the selection of primary clinical syndrome) as a first step of nosological diagnostics when NCDM suspected; retaining of nosological principle with distinction of NCDM to myocarditis, CMPs and myocardiodystrophies; selection of combination NCDM; parallel application of etiological (incl. genetic) and structure-functional CMP classification; selection of CMP with mostly right chambers lesion; selection (together with genetic and idiopathic) of the special CMPs, showing combinational nature; combinations of non-rheumatic myocarditis with peri- and endocarditis; the grade of myocardium diagnosis reliability (if biopsy impossible) with the original criteria of its diagnostics; the level of myocarditis activeness — histological and immune; two types of infarction-like myocarditis (as well as ischemic); paraneoplastic myocarditis and myocarditis as non-classified (latent) systemic immune disorder; introduction of a detailed morpho- and virological characteristics of myocarditis into classification; distinction of myocardiodystrophies by the level of systolic function compensation; creation of clinical classification of arrhythmogenic right ventricle dysplasia and non-compaction myocardium syndrome (incl. in the presence and absence of myocarditis).

In amyloidosis AL-type the heart involvement is most common; this type is specific with predecessor proteins of light immunoglobulin chains, transthyretin (TTR), mutant and wild (senile) types. Most common clinical form is restriction cardiomyopathy.Aim. To assess the recent specifics of diagnostics, due to broader abilities of novel methods.Material and methods. Five 40-79-year old patients with various morphofunctional variants of heart involvement and typical ECG signs (low QRS voltage, QS, non-sufficient R increase, absence of left ventricle (LV) hypertrophy signs) to verify the suspected amyloidosis, underwent EchoCG, immunohistochemistry of blood and urine for light chains of immunoglobulines, biopsy of subcutaneous fat and mucose of the gum/gut; MDCT of the heart (n=3), MRI (n=1), scintigraphy with 99Tc-pyrophosphate with assessment in 1 hour after indicator injection (n=1), endomyocardial biopsy (n=2), titer of anticardial antibodies assessment (n=2), DNA-diagnostics (n=1).Results. The diagnosis of amyloidosis was confirmed in all cases. Its morpho-functional types were RSMP with LV hypertrophy, hypertrophic cardiomyopathy (HCMP) with no restriction but progressing fall of ejection fraction (EF), dilation cardiomyopathy (DCMP), severe hypertrophy with low EF, minimal hypertrophy with no restriction and systolic dysfunction. There was AL-type diagnosed (n=2, one case with myopathy mimicking “dermatomyositis”), mutant TTR (n=1, novel mutation Thr40Asn) and wild TTR (n=2) types. The leading clinical signs were biventricular heart failure and atrial rhythm disorders: sustained atrial fibrillation in 3 patients (in one, before amyloidosis verification, the RF-modification was done as “labyrinth” surgery, isthmus block with no established efficacy) and frequent supraventricular extrasystoly in one another. To the patient with mutant ATTR the ICD was implanted with further replacement by CRT-D, and increase of EF from 24% to 31% was achieved (patient is followed-up for 8 years). As the morphological equivalent of severe systolic dysfunction in this patient the amyloid deposition in myocardial arteries could be suspected. MDCT revealed typical subendocardial delayed deposition in 2 from 3 patients, in one case there was also diffuse deposition of 99Tc-pyrophosphate in myocardium. Antibodies to the nuclei of cardiomyocytes (specific ANF) was found in a female patient with AL-type and DCMP, which made not to rule out myocarditis.Conclusion. Cardiac amyloidosis might present as any structural and functional variant of cardiomyopathy, including DCMP. The most specific is diffuse hypertrophy with restriction and EF decrease, but with no LV dilation. Early fall of contractility, ischemia symptoms and LV dilation might be the result of amyloid lesion of small arteries. In the presence of any systemic presentations together with “HCMP”, “RCMP”, “DCMP” there must be amyloidosis ruled out. Myocardial scintigraphy with 99Tc-pyrophosphate is a method of use for the diagnosis of ATTR; MDCT of the heart — for any type of amyloidosis. Cardiac amyloidosis might be followed by significant type of the titer of specific ANF (secondary reaction or concomitance with myocarditis?).

Aim. To study the role of contrast-enhanced multispiral computed tomography (MSCT) of the heart in diagnostics of myocarditis in patients with the syndrome of dilation cardiomyopathy (DCMP) comparing to morphological investigation of myocardium.Material and methods. Into the main group of study, 127 patients included (92 males, 46,9±11,8 y.o.) with DCMP syndrome (mean end diastolic size (EDS) of the left ventricle (LV) 6,6±0,8 cm, mean ejection fraction (EF) 29,7±9,5%, 3 [2; 3] FC by NYHA). All underwent 320-slice MSCT of the heart with i.v. contrast, 50 underwent morphological investigation of myocardium (endomyocardial byopsy in 30, intraoperational in 7, autopsy in 9, explanted heart study in 4). Also, viral infection markers were studied, as the level of anticardiac antibodies, EchoCG (all patients), scintigraphy (n=42), magnete-resonance tomography (MRI) (n=21), coronary arteriography (CAG, n=48). Comparison group included 18 patients (12 males, 69,2±8,5 y.o.) with coronary atherosclerosis (stenosis form 40%) by MSCT and absence of DCMP criteria (mean EDS LV 4,7±0,5 cm, mean EF LV 59,3±4,9%, 0 [0; 2] FC by NYHA).Results. By the data from complex investigation, myocarditis as the main cause of DCMP syndrome was diagnosed in 79 (62,2%) patients of the main group, its comorbidity with genetic cardiomyopathies — in 19 else (15%). In MSCT of the heart the areas of lower accumulation were found in 4 patients from main group (3,1%, type 1 by the proposed evaluation score), delayed accumulation of the contrast in myocardium — in 72 (56,7%) patients: in 12 subendocardial (type 2), in 4 intramyocardial (type 3), in 44 subepicardial (type 4), in 12 transmural (type 5); in 51 patient there was no delayed accumulation. Sensitivity and specificity of all types of delayed accumulation in diagnostics of myocarditis were 63,3% and 78,7%, positive and negative predictive value 86,1% and 50,7%, subepicardial and transmural types — 49,0%, 83,0%, 85,7%, 43,8%, respectively. While comparing the data of MSCT directly with morphological study of myocardium, diagnostic significance of all types of delayed accumulation in myocarditis revealing was 66,7%, 84,6%, 87,5%, 61,1%, subepicardial and transmural types — 52,4%, 92,3%, 91,7%, 54,5%, respectively.By MSCT in the main group also the non-compaction myocardium was found (n=29, 22,8%), coronary atherosclerosis (n=33, 26,0%), confirmed by CAG in 16 patients. Presence/absence of delayed accumulation by our proposed score correlated with 1) diagnostical signs: duration of illness (r=-0,185, p

Non-compaction myocardium syndrome of the left ventricle (NCM) is relatively new diagnosis. There is no consensual approach to classification, diagnostics, as treatment and management of patients with NCM till recently. Predictive value of the observed genetic mutations is not studied good enough too.Aim. The article is focused on a clinical case of female patient with NCM and lifethreatening ventricular rhythm disorders, revealed two independent mutations in genes coding sarcomere proteins of myocardium.Material and methods. Clinical and instrumental study, except standard methods, included serological diagnostics of myocarditis, defining a genome of cardiotropic viruses in blood, and MSCT of the heart; genetic study included sequencing on the platform Ion Torrent of the coding sequences and attached inthrone regions on 16 genes, which mutations might lead to NCM.Results. In current study we performed clinical, instrumental and genetic investigation of 59-year old female patient with NCM and sustained VT paroxysms of unknown duration that had been being registered since her 47 age, with frequent VE (up to 24 thousands per day). Ventricular dysrhythmias did not resolve regardless two interventions on RFA of the focus of arrhythmia and drug treatment. At the age 56 cardioverter-defibrillator was implanted, and during 3 years of follow-up there were 13 relevant shocks. There were high titers of antimyocardial antibodies. Differential diagnostics was about myocarditis, ACRV and NCM. Genetic test revealed two mutations heterozygous: p.Q1233* gene MYBPC3 and p.R237W in gene MYH7. These genes’ mutations are common findings in NCM. If two mutations found, it is a factor of higher risk of SCD and progradient course of the disease.Conclusion. Genetic changes (mutations) that influence morphology of myocardium proteins, determine primary cardiomyopathies. However, the velocity of a disease course and real conditions of a patient do determine non-genetic factors as well, including sex, age, comorbidities, infectious or immune myocarditis, range and effectiveness of treatment.

The main clinical presentation of noncompaction myocardium (NCM) as nosologic unit are thromboembolic syndrome, heart failure, mostly ventricular arrhythmias and ischemia itself, related to insufficient myocardium blood supply under the noncompacted layer. Also, there are only sporadic cases of myocardial infarction described (MI), including “idiopathic”, in NCM.Aim. To check the prevalence of acute MI in patients with NCM, the specialties of clinical picture, diagnostics, and its probable specific mechanisms, influence on prognosis and its ways of prevention.Material and methods. Totally, 10 patients included, 7 males and 3 females, mean age — 46,3±15,8 y. o., (30 to 76 y. o.) among 85 patients with verified NCM diagnosis, set in accordance with harmonized visual criteria, and developed MI on this background. In 4 cases the NCM is confirmed with three visualizing criteria (EchoCG, MSCT, MRI of the heart), in 4 other — with the two. Mean follow-up was 10,5 [1,75; 32,25] months — from 1 month to 1 year. All patients underwent ECG, ambulatory ECG recording by Holter, EchoCG, assessment of antibodies against various heart antigens, PCR for DNA of parvovirus and B19, as well as herpes group, in blood; 7 coronary arteriographies, 7 MSCT of the heart, measurement of Troponin (n=7), morphological investigation of the heart with PCR-diagnostic of viral infection (n=6), MRI (n=5) and myocardium scintigraphy with 99mТс (n=6).Results. In 4 among 10 patients the development of MI was the first presentation of NCM. Prevalence of coronary atherosclerosis in those with MI and NCM was 20%, however in most cases development of MI was not related with coronary atherosclerosis. Intracardiac thrombosis was verified in 60% of patients with MI, embolism to other organs in 30%. The following mechanisms of MI established: 1) thromboembolism to coronary arteries if thrombi are present in the left chambers of the heart (atrium a. w.a. ventricle), verified in 1 patient at autopsy, and is suspected in other five; 2) concomitance of myocarditis, incl. viral, with microvasculitis development and thrombosis of intramyocardial arteries and focal necrosis in ischemized myocardium (n=6); 3) thrombosis of coronary arteries with presence of hemodynamically significant atherosclerosis (probably 1 patient); 4) sudden worsening of blood supply under noncompacted layer under the circumstances of low cardiac output by secondary origins.Conclusion. MI is typical and unrare complication of NCM: its prevalence reached 11,8% in separate registry of 85 patients with NCM syndrome. Four probable mechanisms of MI (necrosis) in NCM (embolism, thrombosis, myocarditis, microcirculation disorder) might be comorbid. The development of MI leads to serious worsening of the baseline systolic dysfunction and ventricular rhythm disorders: mortality among MI patients with NCM is 20% with the mean time of follow-up 10,5 months. As a preventive matter against MI in NCM should be concerned the anticoagulants at least for atrial fibrillation and in systolic dysfunction, on-time diagnostics and myocarditis management.

Aim. The nosological diagnostics in patients with the baseline syndromic diagnosis “dilated cardiomyopathy” (DCMP) with the use of myocardial biopsy and evaluation of complex treatment (cardiotropic, antiviral, immune suppressing, surgical), including virus-positive and virus-negative patients with myocarditis.Material and methods. Totally, 220 patients included with DCMP syndrome, 69 women and 151 men (31,4% and 68,6%), from 16 to 77 y. o. (mean age — 47,5±12,5,) with dilation and systolic dysfunction of the left ventricle (LV); end diastolic size (EDS) more than 5,5 cm; ejection fraction (EF) less than 50%, and absence of exclusion criteria. Mean EDS was 6,5 [6,0; 7,1] cm, EF — 30,3±10,1%, dp/dt 659 [535; 774,25] mmHg, VTI — 10,8±3,9 cm.The assessments were done of the anticardial antibodies via immune enzyme assay (IEA) and PCR-diagnostics of viral genome in blood of 95,5% patients, tread-mill test (5,9%), coronary arteriography (41,8%), MSCT (67,7%), MRI (22,3%), scintigraphy with 99mТс-MIBI (31,4%), genetic consultation and DNA-diagnostics via sequencing by Senger (21,8% and 16,8%). Morphological study of myocardium was done in 84 (38,2%) patients, incl. 52 endomyocardial biopsies (EMB) with PCR for parvovirus B19 genome and herpes viruses.Results. By the data of clinical-morphological assessment, viral genome in blood was revealed in 21% patients, in myocardium — 52%, definite myocarditis was set in 48%, possible — 8%, genetical DCMP — in 12%, myocarditis with genetic cardiomyopathy — 23% (incl. those with pathogenic mutations in genes MYBPC3, DSP, DSG2, LMNA, EMD, DES, TTR, DTNA), idiopathic DCMP — in 9%. In 59% patients myocarditis was primarily chronic, in more than one third of patients there was combination of several causes for DCMP (incl. alcohol). Short-term and long-term results of treatment were evaluated in 127 patients with myocarditis: 52 virus-positive by blood and/or myocardium (myocarditis morphologically proved in 73,1%) and 75 virus-negative (biopsy in 30,7%), that differed by a little lower EDS LV and lower EF. Significant differences by immunity activation (titres of anticardial antibodies) were not found; in 7% there was isolated viral myocarditis, in 34% — viral-immune; in 59% — immune. If viruses were found, therapy ordered with acyclovir, gancyclovir, entecavir, i. v. immunoglobulines; elimination of virus from the blood reached in 81% of patients. If there was tolerance to cardiotropic therapy and high immune activity, immune suppression therapy (IST) was ordered: in 51% of virus-positive and 62,7% virus-negative patients: steroids 30 [22; 40] and 24 [16; 32] mg/day, azathioprin 1-2 mg/kg, hydroxychloroquine 200 mg/day. In 12,0 [6,0; 20,0] months it was found that only in IST group there was remarkable decrease of CHF FC, LV sizes, MPPA and increase of EF LV from30,3±10,6% to 39,2±11,9% independently from presence or absence of viral genome. In absence of IST such dynamics was not found. The best results were found in virus-negative patients receiving IST (mortality 9,8%, need for surgical treatment 21,3%), the worst — in virus-positive patients without IST (66,7% and 61,1%, respectively).Conclusion. Nosological nature of DCMP syndrome can be defined in most cases; usually there is myocaditis in its origin. IST in high immune activity of myocarditis (high titers of anticardial antibodies) leads to significant increase of EF, decrease of the left ventricle size, MPPA, mortality and necessity of surgery in virus-negative, as in virus-positive patients.

Part I (Diagnostics), was published in the journal “Rational Pharmacotherapy in Cardiology” 2014;10(1):62-72Aim. To develop a comprehensive clinical and morphological approach to the nosological diagnosis and treatment of "idiopathic" arrhythmias (IA) and the syndrome of dilated cardiomyopathy (DCM).Material and methods. Patients (n=320) with IA (n=190; 117 women, age 45.3±14.8 years) and DCM (n=130, 41 women, age 46.9±12.5 years) were included in the main group. 51 people (patients with ischemic heart disease; heart valve disease, hypertrophic cardiomyopathy, who underwent open-heart surgery; healthy volunteers) were included in the control group. Along with the standard tests evaluation of the level of anti-heart antibodies (185 patients with IA and 122 with DCM), viral serology (166 and 122), multispiral computed tomography (42 and 88), cardiac magnetic resonance imaging (41 and 22), coronary angiography (19 and 54), myocardial biopsy/autopsy (19/0 and 33/9) were performed.Results. According to morphological study infectious-immune myocarditis was found in 78.9% patients with IA and 66.7% patients with DCM, arrhythmogenic right ventricular dysplasia in 5.3% and 4.8% of patients, respectively. Other genetic cardiomyopathies, including combination with myocarditis were revealed in other patients. The frequency of the viral genome detection in the myocardium in IA, DCM and the control group was 17.6%, 66.7% and 77.1%, respectively. However in the control group the incidence of myocarditis and anti-heart antibodies titers were significantly lower than in the main group, where a strong correlation between myocarditis and anti-heart antibodies titers was found. The algorithm of noninvasive nosological diagnostics was developed; it allowed to verify diagnosis in 95% of IA patients and 89% DCM patientsThe basic therapy (antiviral drugs, corticosteroids, hydroxychloroquine, azathioprine) was performed in some patients with myocarditis. Improving of the antiarrhythmic effect of treatment in patients with IA, a significant increase in ejection fraction, a reducing of the size of the heart chambers and of pulmonary artery pressure in patients with DCM, a reducing of the need in surgical treatment (including in the virus-positive patients) occurred as results of treatment.Conclusion. Nosological cause of IA and DCM syndrome can be diagnosed in most patients by using an integrated clinical and morphological approach. This allows managing effective etiotropic and pathogenetic treatment.

PAim. To develop a comprehensive clinical and morphological approach to the nosological diagnosis and treatment of "idiopathic" arrhythmias (IA) and the syndrome of dilated cardiomyopathy (DCM).Material and methods. Patients (n=320) with IA (n=190; 117 women, age 45.3±14.8 years) and DCM (n=130, 41 women, age 46.9±12.5 years) were included in the main group. 51 people (patients with ischemic heart disease; heart valve disease, hypertrophic cardiomyopathy, who underwent open-heart surgery; healthy volunteers) were included in the control group. Along with the standard tests evaluation of the level of anti-heart antibodies (185 patients with IA and 122 with DCM), viral serology (166 and 122), multispiral computed tomography (42 and 88), cardiac magnetic resonance imaging (41 and 22), coronary angiography (19 and 54), myocardial biopsy/autopsy (19/0 and 33/9) were performed.Results. According to morphological study infectious-immune myocarditis was found in 78.9% of IA and 66.7% of DCM-patients, arrhythmogenic right ventricular dysplasia in 5.3% and 4.8% of patients, respectively. Other genetic cardiomyopathies, including combination with myocarditis were revealed in other patients. The frequency of the viral genome detection in the myocardium in IA, DCM and the control group was 17.6%, 66.7% and 77.1%, respectively. However in the control group the incidence of myocarditis and anti-heart antibodies titers were significantly lower, while in the main group a strong correlation between myocarditis and anti-heart antibodies titers was found. The algorithm of noninvasive nosological diagnostics was developed; it allowed to verify diagnosis in 95% of IA patients and 89% DCM patients.Conclusion. Nosological cause of IA and DCM syndrome can be diagnosed in most patients by using an integrated clinical and morphological approach.

Aim. To analyze the results of first 50 endomyocardial biopsies (EMB) in general care clinic, to clarify the selection criteria and procedure role in management decision in patients with various noncoronarogenic diseases of myocardium. Material and methods. Totally 50 patients included, who had been underwent EMB during 2007-2014 (31 men, mean age 44,3±13,5). Investigation included the level of anticardiac antibodies (n=49), DNA of herpes group viruses and parvovirus B19 by PCR in blood (n=40) and myocardium (n=49), scintigraphy of myocardium (n=20), MDCT (n=21), MRI (n=17), coronary arteriography (n=22), genetician consultation (n=15). EMB of the right ventricle was done using biopsy forceps STANDARD 5.5 F 104 FEMORAL (Cordis). The materials were coloured with hematoxyline-eosine, picrofucsine by Van Hison, Schiff reactives, Pearls, red congo. The study was approved by the Ethics Comitteeof FMSMU. Results. Indications for EMB were set for 3 groups of patients: 1. With dilated cardiomyopathy (DCMP) as a syndrome, including the probability of acute/ subacute myocarditis (n=7), allergic myocarditis (n=1), myocarditis with myocardial infarction (n=1), vasculitis (n=3), possible myocarditis in long-lasting course of DCMP (n=9), in patients with non-compact myocardium, myodystrophy of Emeri-Dreyfus and possible arrhythmogenic dysplasia of the right ventricle (n=7). 2. With "idiopathic" rhythm and conduction disorders (n=18), including high probability of myocarditis (n=13), vasculitis (n=2), necessity for differentiation of myocarditis and genetic cardiomyopathies (n=3). 3. With the syndrome of primary myocardial hypertrophy (n=3). EMB was informative in all patients, myocarditis found in 82,6%.Conclusion. The criteria for high diagnostic value of EMB is nosological way of selection. In addition to current scenarios it is aimfull to perform EMB if clinically significant ischemia with non-changed coronary arteries, with suspected myocarditis in patients with genetic cardiomyopathies, inborn defects, CHD and other established heart diseases. The EMB results define the tactics of medication and surgical treatment.

Contemporary myocarditis classifications are usually made up by morphological criteria due to a critical role of biopsy in its diagnostic. However in general medical practice this method is quite rare, though in the real practice internists and cardiologists meet this disease more and more ofted, including its non-classical forms.Clear connection of cardial symptomathic with an infection is the most specific sign of myocarditis, though its absence does not rule out the diagnosis. Using biopsy and complex algorhythm of diagnostics developed on the basis of biopsy we have usually seen these types: 1. Latent (without decompensation and general inflammatory changes) myocarditis in patients with so-called idiopathic rhythm and conduction disorders including atrial fibrillation; 2. Various clinical course and immunity-involvement types in patients with the dilated cardiomyopathy; 3. Paraneoplastic myocarditis; 4. Myocarditis with infarction-like manifesting (as acute and benevolent in its course and prognosis, as chronic with the outcome into severe heart failure), microvascular angina, and combination of myocarditis with coronary

A case is reported of a 23-year-old male patient who developed, after severe blunt injury of the lumbar region, massive thrombosis of the vena cava inferior (VCI), both renal veins, bilateral pulmonary artery thromboembolism (PATE), nephrоtic syndrome (NS). In spite of anticoagulant therapy, the condition of the patient progressively aggravated for 1.5 year: thrombosis involved the ileac and femoral arteries on the right, thrombus floated in the right atrium with PATE recurrent episodes, pulmonary hypertension reached 120 mm Hg with formation of decompensated cor pulmonale, proteinuria and hypoalbuminemia deteriorated, anasarca edema developed. Multigenic thrombophilia was diagnosed (1 homozygous and 5 heterozygous mutations). A radical one-stage operation was successful: thromboectomy from the VCI, right ileac and left renal veins, thrombendarterectomy from the pulmonary arteries, suture of the interatrial septum defect, installation of cava-filter. After the operation pulmonary pressure lowered to 40-45 mm Hg, right heart volume normalized, immunosuppressive therapy with prednisolone and cyclosporine led to nephropathy remission. The discussion covers mechanisms and factors (including genetic) of thrombosis progression, correlations between intravascular thrombosis, NS and chronic glomerulonephritis (possible NS development due to bilateral thrombosis of the renal veins and nephropathy role in thrombosis progression), approaches to conservative and surgical treatment of such patients. Global experience in conduction of pulmonary thrombendarterectomy and thrombectomy from VCI is reviewed (one-stage operations were not described earlier).

Aim. To study possibility of nosological diagnosis in patients with dilated cardiomyopathy (DCMP) with use of myocardial biopsy. Material and methods. The trial enrolled 62 patients (23 females) with DCMP syndrome (end diastolic left ventricular size > 5.5 cm, ejection fraction < 55%). Mean age of the patients was 46.0±12.8 years. The examination included diagnosis of viral infections (Herpes virus, parvovirus B19), measurement of anticardial antibodies titer, 99Tc-MIBI single photon emission computed tomography of the myocardium, multislice computed tomography, MRT of the heart, coronarography, morphological study of the myocardium (n = 20) with application of polymerase chain reaction (PCR) for H.simplex viruses of types 1, 2 and 6, herpes zoster, Epstein-Barr, cytomegalovirus, parvovirus B-19, adenoviruses. The control group (20 operated patients with valvular heart disease and coronary heart disease) was examined for viral genome in the blood and myocardium. Results. Complex examination of DCMP patients showed the following distribution by nosological entuities: myocarditis (n = 41, 66.1%) including virus-positive (n = 14), primary DCMP (n = 16, 25.9%) including with non-compact myocarditis (NCM) in 3, with debute at delivery of the child - in 3. Arrhythmogenic right ventricular dysplasia combined with viral myocarditis (n = 2), genetic myopathy (n = 1) and Takayasu disease (n = 1) combined with NCM, isolated NCM (n = 1) were diagnosed in the rest cases. Morphological investigation of the myocardium was made in 20 patients: diagnosis of myocarditis and primary DCMP were made in 70% (including in 2 patients with CHD) and 20%. Detection of viral genome was 20 and 15% in the study and control group, respectively, in the myocardium - in 57.9 (test for parvovirus B19 was not made in 26%) and 65.0% (complete diagnosis).All the virus-positive patients with DCMP were diagnosed to have signs of active/borderline myocarditis. Diagnostic criteria and poor prognosis factors were defined. Conclusion. The nosological diagnosis of DCMP was made in all the examinees basing on the complex of clinical, case history and device evidence. The diagnosis was morphologically verified in 33.9% patients. Treatment approaches are developed.

Primary lymphoma of the heart (non-Hodgkin's lymphoma primarily of B-cells affecting first the heart and pericardium) is a rare disease which accounts for 1.3-2.0% of all primary tumors of the heart. A case is reported of a 43-year-old male patient with a cardioverter-defibrillator implanted at the age of 38 years for management of ventricular tachycardia paroxysms and AB-block of the second-third degree. The patient also had hypertrophic cardiomyopathy, moderate pressure gradient on the pulmonary artery valve and pericardial effusion. Later he had two cardiac tamponades (serous punctuate). From the age of 42 years breathlessness, right ventricular wall thickness, right heart pressure and pulmonary artery pressure aggravated. The patient has undergone ballon plastic surgery of the pulmonary artery valve. At the age of 43 computed tomography detected massive tumor of the heart and mediastinum first diagnosed as sarcoma but later specified as lymphoma. Endobronchial biopsy specimens contained the tissue of diffuse large B-cell lymphoma, the liver and lymph nodes were also involved. After the first course of effective polychemotherapy (CHOP) the patient died of mesenterial thrombosis. Diagnostic difficulties and problems of treatment policy are discussed.

Constrictive polyserositis (pleuritis, pericarditis) is a syndrome within the underlying disease (tuberculosis, periodic disease, rheumatoid arthritis, systemic lupus erythematosus, asbestos, silicosis, uremia, some genetic diseases), a complication due to chest surgery or radiation or drug therapy, is occasionally idiopathic (fibrosing mediastinitis). There are frequently great difficulties in making its nosological diagnosis. The paper describes a patient in whom the onset of disease was exudative pleurisy with the signs of constriction, arthralgias; pleural punctures provided serous exudates with 80% lymphocytes. A year later there was ascitis and shin and foot edemas, which concurrent with hepatomegaly and cholestasis was regarded as cryptogenic liver cirrhosis. The signs of constrictive pericarditis were further revealed. The disease was complicated by the development of pulmonary artery thromboembolism (PATE) (which required the use of warfarin) and hemorrhagic vasculitis. Therapy with metipred in combination with isoniazid yielded a slight effect. The diagnoses of tuberculosis, liver cirrhosis, and autoimmune hepatitis, systemic vasculitis were consecutively rejected; the diagnosis of rheumatoid polyarthritis with systemic manifestations was made, by taking into account persistent arthalgias with the minimum signs of arthritis, noticeably increased C-reactive protein, rheumatoid factor, and cyclic citrullinated peptide antibodies (CCPA); plasmapheresis, therapy with metipred and methotrexate, and subtotal pericardectomy were performed. Constrictive polyserositis concurrent with PATE, hemorrhagic vasculitis (probably, drug-induced one), and hepatic lesion has been first described in a CCPA-positive patient with rheumatoid arthritis in the presence of moderate true arthritis (during steroid therapy).

The V. N. Vinogradov Faculty Therapy Clinic of the Imperial Moscow University (IMU) (then Moscow State University - I. M. Sechenov Moscow Medical Academy) was opened under this name in 1846 simultaneously with the Surgery Clinic on Rozhdestvenka Street. The paper shows the continuity of the idea for bedside clinical teaching, whose need was clearly realized just by S. G. Zybelin and which was first done in practice by his follower and the first Director of the Clinical Institute, IMU, F. G. Politkovsky, as well as that of specific forms of implementing of this idea. It is shown that just early internal medicine teaching provided the principles that were stated by M. Ya. Mudrov and that subsequently formed the basis of a course taught at the Department of Faculty Clinic. The original teaching of a theoretical course without a clinic one at the Department of Particular Pathology and Therapy, which was stipulated by the 1835 Charter, gradually lost its importance as clinical teaching was formed. The succession of the Clinical Institute and the Faculty Clinic was also shown in the staff: the director of the newly-opened Clinic and Therapy Department Professor was A. I. Over, M. Ya. Mudrov's favorite disciple and the last Director of the Clinical Institute; the adjuvant teacher at the Therapeutic Department of the Clinical Institute was K. Ya. Mlodzeyevsky. This all permits the V. N. Vinogradov Faculty Therapy Clinic to be regarded a competent descendant of the first clinical institutes. In conclusion, there is a clinical analysis of division of an internal medicine course into faculty and hospital courses at the present stage and prospects for teaching therapy are discussed.