Introduction: Giant sinus of Valsalva aneurysms (SVA) represent a rare cardiovascular pathology that may remain asymptomatic for an extended period. However, they are associated with a high risk of life-threatening complications, including compression of adjacent structures and aneurysm rupture. Case presentation: We report a clinical case of a 71-year-old female patient with a long-standing history of arterial hypertension and cardiac arrhythmias, in which echocardiography revealed aneurysmal dilatation of the right coronary sinus. Cardiac computed tomography (CT) confirmed the presence of a giant aneurysm of the right sinus of Valsalva measuring 70 × 51 × 49 mm, compressing the outflow tracts of both ventricles (right—up to 7 mm, left—up to 8 mm) and the left inferior pulmonary vein (up to 3 mm), which clinically manifested as dyspnoea, lower-extremity oedema, and rhythm disturbances. The patient successfully underwent complex reconstructive surgery, including aortic root replacement and valve repair. Despite the technical success of the operation, the patient died from pneumonia three months postoperatively. Discussion: This observation underscores the critical role of imaging modalities (echocardiography and CT) in verifying this pathology. The use of multimodal imaging facilitated both a timely diagnosis and a detailed three-dimensional evaluation of the aneurysm’s relationship with adjacent structures. This information, in turn, guided personalised surgical planning. Conclusions: This case highlights the necessity of considering giant SVA in the differential diagnostic workup of patients who present with unexplained symptoms of heart failure.

Background: Myocardial disease is an important component of the wide field of cardiovascular disease. However, the phenomenon of multiple myocardial diseases in a single patient remains understudied. Aim: To investigate the prevalence and impact of myocarditis in patients with genetic cardiomyopathies and to evaluate the outcomes of myocarditis treatment in the context of cardiomyopathies. Methods: A total of 342 patients with primary cardiomyopathies were enrolled. The study cohort included 125 patients with left ventricular non-compaction (LVNC), 100 with primary myocardial hypertrophy syndrome, 70 with arrhythmogenic right ventricular cardiomyopathy (ARVC), 60 with dilated cardiomyopathy (DCM), and 30 with restrictive cardiomyopathy (RCM). The diagnosis of myocarditis was based on data from myocardial morphological examination or a non-invasive diagnostic algorithm consisting of an analysis of clinical presentation, anti-cardiac antibody (Ab) titres, and cardiac MRI. Results: The prevalence of myocarditis was 74.3% in ARVC, 56.7% in DCM, 54.4% in LVNC, 37.5% in RCM, and 30.9% in HCM. Myocarditis had a primary viral or secondary autoimmune nature and manifested with the onset or worsening of chronic heart failure (CHF) and arrhythmias. Treatment of myocarditis in cardiomyopathies has been shown to stabilise or improve patient condition and reduce the risk of adverse outcomes. Conclusions: In cardiomyopathies, the genetic basis and inflammation are components of a single continuum, which forms a complex phenotype. In genetic cardiomyopathies, myocarditis should be actively diagnosed and treated as it is an important therapeutic target.

Aim: to study the level of immunoglobulin FLC in patients with myocarditis in comparison with non-inflammatory heart diseases, and FLC’s correlation with the severity of CHF. Methods: Ninety-nine patients (41 women, 59.6 ± 14.6 y.o.) were included in the study: 50 patients with myocarditis [confirmed by myocardial biopsy (n = 20) and/or cardiac MRI]; 49 patients with non-inflammatory heart disease. CHF was diagnosed in 66% and 65% of patients, respectively. The levels of FLC were determined using the ‘Cloneus S-FLC-K TIA Kit’ and ‘Cloneus S-FLC-L TIA Kit’ reagents. Results: Elevated FLC levels were found in 56% of patients with myocarditis and in 67% of comparison group patients (p > 0.05). Mean FLC kappa levels were 13.4 [11.7; 16.7] and 16.0 [11.3; 23.7] mg/L, FLC lambda 22.7 [16.7; 32.4] and 24.7 [18.1; 39.1] mg/L, FLC kappa/lambda ratio 0.62 [0.50; 0.73] and 0.65 [0.56; 0.76] in myocarditis and comparison groups, respectively; there were no significant differences between groups. Both groups showed correlations of FLC levels with levels of CRP and leukocytes, as well as with glomerular filtration rate, CHF NYHA class, and left ventricular ejection fraction. Only in patients with myocarditis did we observe a significant correlation between both kappa and lambda FLC and NT-proBNP (r = 0.528, p = 0.004, and r = 0.756, p < 0.001) and high-sensitivity troponin (r = 0.829, p = 0.042) levels. Conclusions: Increased FLC level may be considered an important pathogenetic link reflecting both specific mechanisms of myocarditis and severity of CHF. The determination of FLC can be used as an additional diagnostic marker, as well as one predictor of the decompensated course of myocarditis.

Background. A direct clinical and morphological comparison of non-COVID myocarditis than was diagnosed before the SARS-CoV-2 pandemic and post-COVID myocarditis has not been performed so far. Purpose: to compare morphological activity, toll-like receptors expression and response to the immunosuppressive therapy in patients with non-COVID and post-COVID myocarditis. Metodhs. Seventy-seven patients (52 male and 25 female, 48.7±11.7 y.o.) with biopsy proven myocarditis, NYHA class 2 or more heart failure, ejection fraction (EF) <45% were included. A history of myocardial infarction, verified cardiomyopathies, systemic autoimmune diseases, viral DNA in the myocardium, except parvovirus B19, were exclusion criteria. The right ventricular endomyocardial biopsy with hematoxylin and eosin, Van Gieson stains, PCR for virus (herpes viruses, parvovirus B19, adeno-, enteroviruses and SARS-CoV-2), immunohistochemical study for CD3, CD45, CD68, CD20, nucleocapsid / spike proteins of SARS-CoV-2 and in addition toll-like receptors (TLR) type 4 and 9 expression (in 38 patients) were performed. The steroids (methylprednisolone 24-40 mg per day), azathioprine, mycophenolate mofetil were prescribed. The mean follow-up was 16 [6; 30] month. Results. In 45 patients, myocarditis was diagnosed before the SARS-CoV-2 pandemic (giant-cell in one case and lymphocytic in the others). Other 32 patients had post-COVID myocarditis positive for RNA or/and proteins of SARS-CoV-2 (giant-cell in one case, eosinophilic in three cases and lymphocytic in the others). There were no differences in age, NYHA class, CRP and anti-heart antibodies level, echocardiographic parameters (mean EF 30.2±7.8 vs 28.7±6.7%), parvovirus B19 positivity (22 vs 34%), doses of methylprednisolone (24-40 mg/day) and death / transplantation rate (11.1 vs 9.4%). Differences between non-COVID vs post-COVID myocarditis focused on higher CD3, CD 45*, TLR 4 (4+ vs 6+), TLR 9 (0 vs 2+) expression and better response to therapy [10% or more increase in EF in 53 vs 86%* of patients, mean EF (43.9±12.3 vs 49.8±7.6%*) by the end of follow-up], *p<0.05. Conclusion. Post-COVID myocarditis is characterised by different morphological types, higher morphological activity, tendency to higher TLR expression and the best response to the immunosupressive therapy in comparison with non-COVID myocarditis.

The aim of this study was to evaluate the clinical course and outcomes of post-COVID myocarditis in patients with cardiomyopathies (CMP). This case series includes 10 patients with different CMPs who had COVID-19 (seven men; 48.4 ± 11.4 yr.): left ventricular non-compaction (n = 2), arrhythmogenic right ventricular CMP in combination with a heterozygous form of hemochromatosis (n = 1, HFE), restrictive CMP (n = 1, MyBPC3), laminopathy (n = 1, LMNA), dilated cardiomyopathy (n = 1, MYH7 + MyBPC3), Danon’s disease (n = 1, LAMP2) and AL cardiac amyloidosis (n = 3). Myocardial morphological examination with immunohistochemical staining and PCR for SARS-CoV-2 and cardiotropic viruses was performed in six patients, while cardiac MRI and anti-cardiac antibody titres were evaluated in all patients. Post-COVID lymphocytic myocarditis was confirmed morphologically in six patients (with LVNC, RCM, ARCV, Danon’s disease, and AL amyloidosis). Spike and nucleocapsid coronavirus proteins were detected in cell infiltrates, endothelium and cardiomyocytes in all biopsies; SARS-CoV-2 RNA was found in five out of six. In four patients, the diagnosis of myocarditis was based on MRI, high titres of anti-cardiac antibodies and clinical data. The mean time from COVID-19 to the diagnosis of myocarditis was 7 (5; 10.5) months. Myocarditis manifested with the onset/increase of arrhythmias and heart failure. Immunosuppressive therapy with corticosteroids was administered to six patients and led to an increase in ejection fraction and improvement of heart failure symptoms in five of them. CMPs are a favourable background for the development of post-COVID myocarditis. The onset or deterioration of heart failure and/or arrhythmias in patients with CMPs after COVID-19 requires the exclusion of myocarditis and, if present, the administration of immunosuppressive therapy.

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

Aim To study the concentration of immunoglobulin free light chains (FLCs) in patients with myocarditis in comparison with non-inflammatory heart diseases, their relationship with inflammatory markers and the severity of chronic heart failure (CHF). Material and methods This study included 77 patients (31 women, mean age 54.1 +/- 13.3 years): 41 patients with myocarditis verified by myocardial biopsy (n=18) or using a noninvasive diagnostic algorithm, 31 patients with noninflammatory CHF (comparison group), and 5 patients with monoclonal gammopathy identified during the study (4 of them were diagnosed with AL amyloidosis with heart damage). In the myocarditis group, CHF was diagnosed in 29 patients, mean stage IIA, functional class (FC) 2-3, with a mean left ventricular ejection fraction 43 %. In the comparison group, patients had predominantly IIA stage, FC 2-3 CHF without systolic dysfunction. The blood concentration of kappa and lambda FLC types was measured with Cloneus S-FLC-K TIA Kit and Cloneus S-FLC-L TIA Kit. Concentrations were considered normal at FLC-kappa 4.84-14.20 mg / l, FLC-lambda 7.03-22.50 mg / l, and the FLCkappa /lambda ratio 0.426-1.050. Results Increased FLC concentrations were found in 58 % of patients with myocarditis and in 77 % of patients in the comparison group. The FLC-lambda concentration was significantly higher in the comparison group; there were no significant differences between the groups in FLC-kappa and their ratio. The closest significant correlations in both groups and the entire cohort were noted between FLCs of either type and CHF, as well as the requirement for loop diuretics (correlation coefficients, 0.60-0.90), independent on the severity of systolic dysfunction. Myocarditis patients also showed correlations of FLCs with the titer of antibodies to cardiomyocyte nuclear antigens, levels of C-reactive protein, leukocytes, neutrophils, erythrocyte sedimentation rate, and the concentration of N-terminal fragment of brain natriuretic peptide. In a subgroup of 10 myocarditis patients who were treated with immunosuppressants, FLCs of both types were significantly lower than in the comparison group; only with the persistence of severe CHF was an increase in FLCs noted. Conclusion An increased FLC concentration can be considered as an important pathogenesis component that reflects both the specific mechanisms of myocarditis and the severity of CHF. In the absence of a statistically significant increase in general inflammatory markers in the blood of myocarditis patients, the measurement of FLCs can be used as an additional diagnostic marker and predictor of the decompensated variant of the course of myocarditis. However, the diagnostic and prognostic significance of FLC concentration in patients without CHF requires a further study.

The state of the heart after a coronavirus disease 2019 (COVID-19, coronavirus disease 2019) is very diverse and in some cases requires a complex differential diagnosis. We described a 35-year-old smoking male patient who, by the end of the first month after COVID-19, developed left ventricular dysfunction with decrease in ejection fraction to 25-30% and persistent left bundle branch block (LBBB). Echocardiography also detected local contractility disorders. A significant increase in blood anticardiac antibody titers was noted. Myocardial biopsy revealed active lymphocytic myocarditis, coronavirus ribonucleic acid, while coronary angiography revealed extensive stenosis of the anterior interventricular artery. On the first day after balloon angioplasty and stenting, the disappearance of LBBB block was noted with the appearance of deep negative T waves in the precordial leads, which did not allow ruling out a previous myocardial infarction. As a result of treatment of heart failure and steroid therapy, the structural and functional cardiac parameters and the electrocardiography were completely normalized by the end of the second year of treatment. Only a transient LBBB remained at high loads. Cardiac computed tomography after 2 years showed no delayed contrast agent accumulation in the myocardium, and coronary stenosis up to 30%.The mechanisms of complex myocardial and coronary damage (including the role of coronaritis in the atherosclerosis progression) after COVID-19 are discussed.

BACKGROUND AND AIMS:Contemporary multicentre data on clinical and diagnostic spectrum and outcome in myocarditis are limited. Study aims were to describe baseline features, 1-year follow-up, and baseline predictors of outcome in clinically suspected or biopsy-proven myocarditis (2013 European Society of Cardiology criteria) in adult and paediatric patients from the EURObservational Research Programme Cardiomyopathy and Myocarditis Long-Term Registry. METHODS:Five hundred eighty-one (68.0% male) patients, 493 adults, median age 38 (27-52) years, and 88 children, aged 8 (3-13) years, were divided into 3 groups: Group 1 (n = 233), clinically suspected myocarditis with abnormal cardiac magnetic resonance; Group 2 (n = 222), biopsy-proven myocarditis; and Group 3 (n = 126) clinically suspected myocarditis with normal or inconclusive or no cardiac magnetic resonance. Baseline features were analysed overall, in adults vs. children, and among groups. One-year outcome events included death/heart transplantation, ventricular assist device (VAD) or implantable cardioverter defibrillator (ICD) implantation, and hospitalization for cardiac causes. RESULTS:Endomyocardial biopsy, mainly right ventricular, had a similarly low complication rate in children and adults (4.7% vs. 4.9%, P = NS), with no procedure-related death. A classical myocarditis pattern on cardiac magnetic resonance was found in 31.3% of children and in 57.9% of adults with biopsy-proven myocarditis (P < .001). At 1-year follow-up, 11/410 patients (2.7%) died, 7 (1.7%) received a heart transplant, 3 underwent VAD (0.7%), and 16 (3.9%) underwent ICD implantation. Independent predictors at diagnosis of death or heart transplantation or hospitalization or VAD implantation or ICD implantation at 1-year follow-up were lower left ventricular ejection fraction and the need for immunosuppressants for new myocarditis diagnosis refractory to non-aetiology-driven therapy. CONCLUSIONS:Endomyocardial biopsy was safe, and cardiac magnetic resonance using Lake Louise criteria was less sensitive, particularly in children. Virus-negative lymphocytic myocarditis was predominant both in children and adults, and use of immunosuppressive treatments was low. Lower left ventricular ejection fraction and the need for immunosuppressants at diagnosis were independent predictors of unfavourable outcome events at 1 year.

Fever of unknown origin (FUO), despite the wide diagnostic potential of modern medicine, remains a difficult problem for clinicians. Often, making a correct diagnosis requires a comprehensive examination of the patient, as well as the joint work of doctors from different specialties.A 57-year-old male patient was hospitalized due to an episode of loss of consciousness, unmotivated weight loss and daily evening temperature rises to 38-39о C, accompanied by chills and increased sweating. Previously, he was examined by a general practitioner, a cardiologist, and repeatedly by a hematologist. There was no evidence of an infectious disease, arrhythmias, multiple myeloma, lymphoproliferative disorders. Monoclonal gammopathy of undetermined significance (MGUS) was diagnosed. Blood tests revealed neutrophilia (9800 cells/ μl) and moderate anemia. The patient was hospitalized to determine the FUO cause. Heart auscultation revealed a systolic murmur in the mitral valve, which was previously absent. Echocardiography revealed a mobile masses on the mitral valve, severe mitral regurgitation, and therefore infective endocarditis was diagnosed. Empirical antibiotic therapy with ceftriaxone and levofloxacin was administered. Further blood culture revealed growth of a HACEK representative Aggregatibacter actinomycetemcomitans, sensitive to both drugs. Examination established the odontogenic nature of endocarditis. During treatment, stable normothermia and significant blood count improvement were achieved. However, fluoroquinolone therapy led to tendinopathy. Due to persistent grade 3 mitral regurgitation, the patient underwent routine mitral valve replacement. Further follow-up revealed satisfactory condition of the patient.A feature of this case is the atypical course of infective endocarditis, occurring under a hematological mask, which made its diagnosis difficult. When examining a FUO patient, infective endocarditis should be included in the range of possible causes, taking into account not only the typical clinical picture, but also the numerous masks of this disease.

The emergence of effective therapy for AL amyloidosis with cardiac involvement raises the challenge of a comprehensive assessment of the risk of SCD and its prevention. This problem should be approached individually and the possibility of ICD implantation should be considered in patients with ventricular arrhythmias: frequent extrasystoles and unstable ventricular tachycardia. With the development of rhythm and conduction disturbances in patients with amyloidosis, one should keep in mind the possibility of myocarditis, and in patients who have suffered a new coronavirus infection, its possible role in the induction of both post-Covid myocarditis and amyloidosis itself.

Aim. To study the mechanisms and clinical variants of subacute and chronic myocarditis after SARS-CoV-2 infection using morphological and virological studies and to develop approaches to its treatment.Material and methods. The study included 89 patients with documented coronavirus disease 2019 (COVID-19). The diagnosis of post-COVID myocarditis was established based on myocardial biopsy and/or cardiac magnetic resonance imaging (MRI) data in combination with anamnesis, increased titers of anticardiac antibodies, and other criteria. The average time of presentation after infection was 8,0 [4; 17,5] months, while the average follow-up period was 7,0 [6,0; 13,5] months. Electrocardiography, Holter monitoring, echocardiography, cardiac MRI (n=60), determination of anticardiac antibody levels, myocardial biopsy (n=38), and autopsy (n=1) were performed.Results. All patients showed an association between the onset or exacerbation of cardiac symptoms and COVID-19. The clinical variants of post-COVID myocarditis were identified: arrhythmic (n=24) with newly developed arrhythmias with normal myocardial contractility; decompensated (n=65) with newly diagnosed heart failure, including a variant developed with primary (genetic) cardiomyopathies and amyloidosis (n=10). The profile of arrhythmias in the arrhythmic variant varied from potentially life-threatening rhythm and conduction disorders (sustained ventricular tachycardia, grade II-III atrioventricular block) to infrequent supraventricular premature beats. In the decompensated variant, lymphocytic myocarditis was most often morphologically detected, while eosinophilic and giant cell myocarditis, associated with a worse prognosis, were rarer. Simultaneous development of nonbacterial thrombotic endocarditis, infective endocarditis with the development of valvular heart defects was noted. In 10 cases, myocarditis was combined with primary cardiomyopathies, AL amyloidosis. Treatment included the administration of antiarrhythmic, cardiotropic, immunosuppressive therapy (glucocorticoids 16-32 mg/day in 68,5% of patients, hydroxychloroquine 200-400 mg/day in 33,7%). In the arrhythmic type, complete arrhythmia suppression was achieved in 25% of cases, while partial in 58,3%. In one case, a pacemaker was implanted. In patients with a decompensated myocarditis, corticosteroids were effective regardless of the viral genome/proteins in the myocardium.Conclusion. COVID-19 can induce subacute and chronic myocarditis with the development of isolated arrhythmias or severe heart failure.

AIMS:This paper aimed to study the efficacy and safety of mycophenolate mofetil (MM) in combination with corticosteroids in the treatment of lymphocytic myocarditis (LM) when compared to the standard combination of corticosteroids and azathioprine.METHODS:The study included 50 adult patients (47.8 ± 10.8 y.o.) in a NYHA III functional class due to LM who were verified using endomyocardial biopsy. The main group included 29 patients who received MM at 2 g/day. The comparison group comprised 21 patients who received azathioprine at 150 [50; 150] mg/day. Both groups were administered with methylprednisolone. The average follow-up period was 30 [22; 35] months, but no less than 6 months.RESULTS:The groups were comparable in the baseline parameters and standard drug therapy. In both groups, there was a comparable significant increase in the ejection fraction (from 30.6 ± 7.7% to 44.0 ± 9.4% vs. 29.2 ± 7.7% to 46.2 ± 11.8%, p < 0.001), and a decrease in systolic pressure in the pulmonary artery and the dimensions of the left ventricle and atrium. The frequency of death was two (6.9%) and two (9.5%), transplantation was one (3.4%) and one (4.8%) patient and the "death + transplantation" endpoint was three (10.3%) and three (14.3%) without differences between the groups. The presence of the parvovirus B19 in the myocardium in 6/5 patients did not affect the results. The incidence of infectious complications was comparable. The most severe infectious complications were pneumonia and fatal purulent encephalitis (both cases in the azathioprine group), leptospirosis meningitis (in the mycophenolate mofetil group).CONCLUSIONS:In the patients with LM, the combination of corticosteroids with MM at a dose of 2 g/day was at least no less effective than with azathioprine. There was a tendency toward a better tolerance using MM.

Patients with damage of the mitral, aortic and tricuspid valves and systolic myocardial dysfunction associated with previous SARS-CoV-2 infection are described. The diagnosis of acquired defect was established in 4 patients based on medical history, electrocardiography, echocardiography, magnetic resonance imaging of the heart, endomyocardial or intraoperative myocardial biopsy, and in one case, autopsy. The study of the myocardium included H&E, Van Gieson staining, immunohistochemical (IHC) study with antibodies to CD3, CD20, CD45, CD68, to the nucleocapsid and Spike proteins of SARS-CoV-2. Previous valve diseases (prolapse, bicuspid aortic valve) served as a background for the development of the defect in 2 patients. In all cases, IHC studies revealed coronavirus proteins, lymphocytic endocarditis and myocarditis, moderate fibrosis, and signs of connective tissue disorganization. High titers of anticardiac antibodies indicated an autoimmune mechanism for carditis. No signs of infective endocarditis or thromboembolic complications were identified in any case. In patients with an unclear nature of valvular heart defects, a previous new coronavirus infection should be identified and taken into account as a possible etiological factor. The simultaneous development of lymphocytic myocarditis significantly increases the risk of surgical intervention on the valves and requires an integrated approach to treatment.

A 60-year-old male with hypertrophic cardiomyopathy, conduction disorders, post-COVID-19 myopericarditis and heart failure was admitted to the hospital’s cardiology department. Blood tests revealed an increase in CPK activity, troponin T elevation and high titers of anticardiac antibodies. Whole exome sequencing showed the presence of the pathogenic variant NM_213599:c.2272C>T of the ANO5 gene. Results of the skeletal muscle biopsy excluded the diagnosis of systemic amyloidosis. Microscopy of the muscle fragment demonstrated sclerosis of the perimysium, moderate lymphoid infiltration, sclerosis of the microvessels, dystrophic changes and a lack of cross striations in the muscle fibers. Hypertrophy of the LV with a low contractile ability, atrial fibrillation, weakness of the distal skeletal muscles and increased plasma CPK activity and the results of the skeletal muscle biopsy suggested a diagnosis of a late form of distal myopathy (Miyoshi-like distal myopathy, MMD3). Post-COVID-19 myopericarditis, for which genetically modified myocardium could serve as a favorable background, caused heart failure decompensation.

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.