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Background. Interstitial lung disease (ILD) is the common internal organ manifestation of idiopathic inflammatory myopathies (IIM) that can severely affect the course and prognosis of the disease. Rituximab (RTX) has been used to treat IIM, including variants with ILD. Objectives. To describe the course of disease in IIM patients with ILD, treated with RTX in long-term follow-up. Methods. Our prospective study included 35 pts with IIM fulfilling Bohan and Peter criteria and having ILD. The mean age was 51.8+/-11.9 years, female-26 pts (74%);24 (68.5%) with antisynthetase syndrome, 5 (14.3%) dermatomyositis (DM), 5 (14.3%) with a-Pm/Scl overlap myositis and 1 (2,9%) with a-SRP necrotizing myopathy were included. 25 (71,4% ) patients had nonspecific interstitial pneumonia, 9 (25,7%) organizing pneumonia (OP) and 1 (2,9%) OP, transformed to diffuse alveolar damage. All pts had the standard examination including manual muscle testing (MMT), creatinkinase (CK) anti-Jo-1 antibodies (anti-Jo-1) assay;forced vital capacity (FVC) and carbon monoxide diffusion capacity (DLCO) evaluation as well as high-resolution computed tomography (HRCT) scanning of the chest were performed at baseline, and 36 and more months. The median disease duration was 3.2 [0.16-18] years, 21 (60%) of pts were positive for a-Jo-1 antibody. All pts received prednisolone at a mean dose of 24.3+/-13 mg/day, immunosupressants at inclusion received 25 (71%) pts: cyclophosphamide 18 , mycophenolate mofetil 6 and comdination 1;Rituximab (RTX) was administered in case of severe course of disease and intolerance or inadequate response to GC and other immunosuppressive drugs. Results. The mean follow-up period after the first infusion of RTX was 47.2+/-11.9 months. Pts received 1-11 courses of RTX . The cumulative mean dose of RTX was 4.6 +/-2.5g. MMT 8 increased from 135.8+/-13.5 to 148.75+/-3.5 (p=0.000001). CK level decreased DELTACK - 762 u/l(median 340;25th% 9;75th% 821). anti-Jo-1 decreased from 173.4+/-37 to 96.5+/-79 u/ml (p=0.00002), FVC increased from 82+/-22.6 to 96,9+/-22% (p=0.00011). DLCO increased from 51.4+/-15.2 to 60+/-77.8% (p=0.0001). The mean prednisone dose was reduced from 24.3+/-13 to 5.7+/-2.4 mg/day. 3 pts died: ILD progression was the cause of death in 1 case, 1 bacterial pneumonia and COVID19 pneumonia. Conclusions. The results of this study confirm the positive effect of RTX in IIM patients with ILD (increase of muscle strength and improve lung function, decrease in anti-Jo-1 levels) and also its good steroid-sparing effect. RTX could be considered as an effective drug for the complex therapy of IIM patients with ILD when standard therapy is ineffective or impossible.
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SESSION TITLE: Treatment Debates in Critical Care SESSION TYPE: Rapid Fire Original Inv PRESENTED ON: 10/17/2022 12:15 pm - 1:15 pm PURPOSE: Interstitial lung disease (ILD) is the common internal organ manifestation of idiopathic inflammatory myopathies (IIM) that can severely affect the course and prognosis of the disease. Rituximab (RTX) has been used to treat IIM, including variants with ILD. OBJECTIVES:To describe the course of disease in IIM patients with ILD, treated with RTX in long-term follow-up. METHODS: Our prospective study included 35 pts with IIM fulfilling Bohan and Peter criteria and having ILD. The mean age was 51.8±11.9 years, female-26 pts (74%);24 (68.5%) with antisynthetase syndrome, 5 (14.3%) dermatomyositis (DM), 5 (14.3%) with a-Pm/Scl overlap myositis and 1 (2,9%) with a-SRP necrotizing myopathy were included. 25 (71,4% ) patients had nonspecific interstitial pneumonia, 9 (25,7%) organizing pneumonia (OP) and 1 (2,9%) OP, transformed to diffuse alveolar damage. All pts had the standard examination including manual muscle testing (MMT), creatinkinase (CK) anti-Jo-1 antibodies (anti-Jo-1) assay;forced vital capacity (FVC) and carbon monoxide diffusion capacity (DLCO) evaluation as well as high-resolution computed tomography (HRCT) scanning of the chest were performed at baseline, and 36 and more months. The median disease duration was 3.2 [0.16-18] years, 21 (60%) of pts were positive for a-Jо-1 antibody. All pts received prednisolone at a mean dose of 24.3±13 mg/day, immunosupressants at inclusion received 25 (71%) pts: cyclophosphamide 18, mycophenolate mofetil 6 and comdination 1;Rituximab (RTX) was administered in case of severe course of disease and intolerance or inadequate response to GC and other immunosuppressive drugs. RESULTS: The mean follow-up period after the first infusion of RTX was 47.2±11.9 months. Pts received 1-11 courses of RTX. The cumulative mean dose of RTX was 4.6 ±2.5g. MMT 8 increased from 135.8±13.5 to 148.75±3.5 (p=0.000001). CK level decreased ΔCK – 762 u/l(median 340;25th% 9;75th% 821). anti-Jo-1 decreased from 173.4±37 to 96.5±79 u/ml (p=0.00002), FVC increased from 82±22.6 to 96,9±22% (p=0.00011). DLCO increased from 51.4±15.2 to 60±77.8% (p=0.0001). The mean prednisone dose was reduced from 24.3±13 to 5.7±2.4 mg/day. 3 pts died: ILD progression was the cause of death in 1 case, 1 bacterial pneumonia and COVID19 pneumonia. CONCLUSIONS: The results of this study confirm the positive effect of RTX in IIM patients with ILD (increase of muscle strength and improve lung function, decrease in anti-Jo-1 levels) and also its good steroid-sparing effect. CLINICAL IMPLICATIONS: RTX could be considered as an effective drug for the complex therapy of IIM patients with ILD when standard therapy is ineffective or impossible DISCLOSURES: No relevant relationships by Lidia Ananyeva No relevant relationships by Maria Aristova No relevant relationships by Oxana Desinova No relevant relationships by Liudmila Garzanova No relevant relationships by Anna Khelkovskaya-Sergeeva No relevant relationships by Olga Koneva No relevant relationships by Dmitry Kulikovsky
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Objective: NA Background: Here we report a patient with COVID-19 associated inflammatory myopathy, presenting with facial, bulbar and proximal limb weakness. A 58-year-old woman presented with cough, dyspnea, and myalgia. Vital signs and her physical exam was unremarkable. Initial PCR testing for SARS-CoV-2 was negative and the patient was discharged home. She returned three weeks later with more severe dyspnea, cough, dysarthria, dysphagia, odynophagia and severe generalized weakness with inability to ambulate. She had no sensory symptoms or bowel or bladder dysfunction. Physical examination was significant for tachycardia and oxygen saturation of 88% on room air. She had bilateral ptosis, facial weakness, hypernasal dysarthria and profound symmetric proximal limb weakness. Reflexes were symmetrically diminished. Repeated SARS-CoV-2 PCR was positive. MRI of the entire neuroaxis showed no central or peripheral nervous system involvement, but demonstrated diffuse muscle edema and enhancement, with a region of myonecrosis Motor nerve conduction studies were unremarkable, needle electromyography revealed sparse fibrillation potentials;On admission, CK was elevated to 700 U/L. Anti-Sjögren's-syndrome-related antigen and anti-small ubiquitinlike modifier-1 activating enzyme antibodies were both strongly positive and Ku antibody was weakly positive. Muscle biopsy showed perivascular inflammatory infiltration with endomysial extension, regenerating fibers and upregulation of HLA Class ABC expression on non-necrotic fibers. Our presumptive diagnosis was COVID-19 associated myositis and a five-day course of 1000 mg intravenous methylprednisolone was administered. Over two weeks, her CK levels normalized and she recovered the ability to raise her arms and legs from the bed and showed slow improvement in bulbar function. Design/Methods: NA Results: Viral infection is a well-known cause of myositis. The severe immune activation known to occur in COVID-19 patients likely plays a major pathophysiologic role. The finding of multiple serologic autoimmune antibodies is intriguing suggesting an epiphenomenon rather than activation or unmasking of a specific immune response directed to the muscles. Conclusions: NA.
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Due to its expression by macrophages, galectin-3 is among the most recently studied biomarkers. It is likely involved in the inflammatory process that leads to remodeling and eventually fibrosis of organs such as the heart, brain, and kidneys. Coronavirus disease 19 (COVID-19) infection causes excessive inflammatory reactions in the whole body, playing a role in the development of fibrosis due to the activation of the galectin-3-macrophage-fibroblast axis. Heart failure or cardiac dysfunction occurred not only due to pro-inflammatory activation but also due to the overactivation of sympathetic nerves and failure of the respiratory system. The latter increases. the possibility of direct infection or necrosis of the heart due to the heart-lung interaction observed in our pilot study. Forty-five intensive care unit (ICU) patients were recruited consecu-tively in this study to be observed their galectin-3 and troponin I levels. This pilot study demonstrates the correlation between galectin-3 as a proinflammatory biomark-er and troponin I as a definitive biomarker for direct heart injury and highlights its potential use in COVID-19 patients. With the assessment of appropriate biomarkers such as cardiac fibro-sis markers, possible worsening of cardiac conditions in COVID-19 patients treated in the ICU can be detected in its early stages.
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Clinical Information Patient Initials or Identifier Number: CE 16/04/1941 Relevant Clinical History and Physical Exam: An 80 years of age lady without any previous disease were conducted to our emergency department due to dyspnoea lasting several hours. At arrival in our emergency department, the patient was still dyspnoeic. Her ECG demonstrated diffuse ischemic changes without certain site definite ischemia. Her chest X-ray showed thickening of the interlobular septa, peri-bronchial cuffing, thickening of the fissures, increased vascular marking, bilateral pleural effusions, cardiomegaly and aortic calcifications. [Formula presented] Relevant Test Results Prior to Catheterization: Her laboratory data revealed increase in myocardial necrosis markers as her TnI-HS was 3450 ng/ml and relatively normal values of other parameters. At echocardiography flash we found severe aortic valve calcification causing stenosis with peak gradient 48 mmHg, mean gradient 28 mmHg and diffuse segmental hypokinesis of left ventricle with global systolic function about 30%. The DAPT was started, and the patient was planned for coronary angiography within 24 hours of admission. [Formula presented] [Formula presented] Relevant Catheterization Findings: Coronary angiography performed through the right radial artery shoed ostial and mid RCA stenosis, severe calcific distal left main (Medina 1.1.1.), mid LAD and mid Lcx stenosis (Fig. 3). At the time of COVID any transfer to any surgical center was extremely difficult so after discussion with the patient and the family we fixed the RCA with one in the mid segment and one ostial DES. Then after aortic valvuloplasty (Valver 20 mm) for Impella 2,5 placement in the left ventricle was done. [Formula presented] [Formula presented] [Formula presented] Interventional Management Procedural Step: Through right radial artery access, the right coronary artery was fixed with stenting. Then aortic balloon valvuloplasty (Valve 20 mm). The Impella device was advanced and after crossing the dilated aortic valve the tip was placed in the left ventricle. Sequential predilatations of LM-LAD and LM-LCx with semi-compliant, non-compliant and scoring balloons were performed. For persisting of unacceptable for stenting result we continued the preparation of LM bifurcation with predilatation using intravascular lithotripsy (IVL) with Shockwave balloon on LM-LAD and LM-CX. Then we implanted one bifurcation dedicated stent Bioss Lim on the axis LM-LCx. After predilatation of mid LAD we placed one drug eluting stent from LM to LAD proximal through the Bioss stent (short culotte stenting). The procedure was ended with drug coated balloon on LAD mid and with drug coated balloon inflation on LCx mid. Then, Impella was removed, and vascular closure was achieved with Manta closing device. In the ICCU, the patient complained of intensive pain in the left lumbar and iliac region, nausea and severe hypotension (80/40 mmHg blood pressure). Contralateral injection demonstrated common femoral artery injury with large amount of blood passage in the pelvic cavity. A self-expandable covered stent 8 x 60 mm was introduced and placed at the site of artery rupture. The control angiography evidenced complete closure of the artery wall with no blood passage. [Formula presented] [Formula presented] [Formula presented] Conclusions: In time of pandemic restrictions, decision of treatment must be done using available in-hospital facilities. The presence of aortic valve stenosis and multi-vessel disease and low ejection fraction requires contemporary preparation of aortic valve for haemodynamic support during coronary angioplasty. Vessel preparation can be achieved with new devices as intravascular lithotripsy (IVL) to reduce the risk of complication. DCB are valid alternative to DES particularly in small vessels with long atherosclerotic disease. Vascular access site dramatic complications in the experienced hands and well-organized catheterization laboratory can be managed within the cath lab percutaneously.
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Background. COVID 19 is associated with a hypercoagulable state with cytokine storm syndrome and thrombocytopenia leading to complications across various systems. COVID-19 infection, its treatment, resultant immunosuppression, and pre-existing comorbidities have made patients vulnerable to secondary infections Methods. We systematically reviewed COVID-19 cases between Jan to May 2021 for pulmonary and extrapulmonary complications. Patients with recent COVID-19 vaccination and neurological symptoms were also included. Results. Neurological complications: Neurological complications include ischemic and haemorrhagic strokes. Other complications are encephalopathy, encephalitis, Guillain-Barré syndrome, acute hemorrhagic necrotizing encephalopathy. Demyelination and radiculopathies are seen as post vaccination complications. Mucormycosis: Unprecedented high rate of invasive fungal sinusitis in association with COVID -19 is reported from the Indian subcontinent. This has a propensity for intra orbital and intracranial extension. COVID -19 associated coagulopathy: COVID -19 is a pro-inflammatory hypercoagulable state. Pulmonary thromboembolism, deep venous thrombosis and catheter related thrombosis are well documented. Cardiac complications: Cardiac manifestations include Myocardial Injury with non-obstructed coronary arteries (MINOCA), myocarditis, myocardial ischemia, cardiomyopathy. Pulmonary complications and sequelae of COVID -19: Progression of lung injury to ARDS during the initial phase and fibrosis of parenchyma in the recovery phase. Spontaneous pneumomediastinum, pneumatoceles and pneumothorax and secondary infections are identified in our study. COVID- 19 associated gastrointestinal complications: Patients evaluated for renal colic, pancreatitis, cholecystitis showed, ground glass opacities or subpleural bands in typical Covid-19 distribution. COVID-19 may lead of acute kidney and bowel injury due to arterial thrombosis. COVID - 19 associated myonecrosis: Ischemia of the small caliber vessels may result in myonecrosis. Conclusion. Awareness of these unusual manifestations will facilitate an early diagnosis, improve management and help reduce morbidity and mortality.
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Objectives: To have a comprehensive review on effects of novel coronavirus on heart. Methods: review article. Results: The novel coronavirus may affect cardiac tissue via three main mechanisms: 1-direct myocardial injury and myocarditis caused by the virus, 2-hyper-inflammation and immunopathology, and 3-respiratory failure, acute respiratory distress syndrome, and effects of hypoxemia on cardiac tissue. In a large number of patients, all three mechanisms are involved. Hypercoagulability is a mechanism for coronary artery stenosis and acute coronary syndrome or myocardial infarction. Also, blood pressure abnormalities, either hypertension or hypotension are frequent in severely ill patients. A high proportion of critically ill patients develop arrhythmias. Arrhythmias may arise due to hypoxemia, metabolic derangements, systemic inflammation, or myocarditis. In a postmortem study, real-time polymerase chain reaction analyses on heart tissue detected the viral genome in nearly one third of patients. Interleukin-6, serum ferritin, brain natriuretic peptide, and high sensitivity cardiac troponin are among the various biomarkers elevated during the course of the disease. It has been shown that as the disease severity increases and in the 3rd stage of the disease-host response-inflammatory and cardiac markers show elevations. Cardiac involvement ad elevated cardiac biomarkers are prominent features in COVID-19 and associated with a worse prognosis and increased mortality. In a survey in Wuhan, 40% of deaths were attributed to myocardial damage or heart failure, alone or in combination with respiratory failure. In autopsies, mononuclear infiltrates of macrophages and CD4+ T cells have been shown in areas of cardiac necrosis. It has been proposed that acute cardiac involvement is a stronger risk factor for increased mortality than age, diabetes mellitus, chronic pulmonary disease, or even history of cardiovascular disease. Since the virus is new-emerging, we do not know much about its long term consequences. So, its effects on heart in the convalescent and chronic phases are not well-known. Delayed myocarditis, cardiac arrest, hyperlipidemia and pulmonary fibrosis are possible long term consequences of the disease. Conclusion: We have to be aware of cardiac consequences of COVID-19 to manage the disease optimally.
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Case Report According to the Center for Disease Control, Severe Acute Respiratory Syndrome Coronavirus 2 (COVID- 19) may present with a wide range of symptoms. Among those, fever, cough, and shortness of breath are commonly present. While COVID-19 associated myalgias is reported as a symptom, we present a case of COVID-19 related rhabdomyolysis. This is a previously healthy 13-year-old obese male who arrived to an outside hospital in respiratory distress after a 1- week history of nasal congestion, productive cough, shortness of breath, emesis and diarrhea associated with malaise and muscle weakness sensation. On presentation he was hypoxic (O2 sat 87%), tachypneic and had increased work of breathing for which was initially treated with 2 liters per minute of oxygen by nasal cannula and fluid resuscitation. The patient had a rapid COVID antigen test that was negative, a chest xray that showed bilateral infiltrates (worse on the right), normal complete blood count and normal electrolytes. However, he was found to have an elevated serum creatinine (1.2), an elevated creatinine kinase level (1264 U/L) and serum myoglobin (97.2 ng/ml) leading to him being diagnosed with acute kidney injury secondary to viral induced rhabdomyolysis and pneumonia. He was treated with antibiotics (Azithromycin and Ceftriaxone) and aggressive fluid resuscitation and transferred to our hospital for further care. At our facility, antibiotics were discontinued after a COVID-19 PCR testing resulted positive. Aggressive fluid resuscitation was continued to treat rhabdomyolysis and he received a course of Remdesivir and Decadron to treat COVID-19 infection. The patient then recovered and was discharged home on the 5th day. Rhabdomyolysis is a life-threatening condition that occurs when muscle necrosis results in the release of its intracellular contents into circulation. In the pediatric population, viral myositis has been reported as a leading cause. It is commonly diagnosed by elevation of serum creatine kinase (CK) and presence of myoglobinuria. Clinically, patients with rhabdomyolysis may be asymptomatic or present with severe disease characterized by myalgias, massive CK enzyme elevations, severe electrolyte imbalances and acute kidney injury. While rhabdomyolysis has previously been reported as a rare complication of COVID-19 infection, there are fewer reports of rhabdomyolysis secondary to COVID-19 in the pediatric population. This case illustrates the importance of suspecting this life threating condition in patients with COVID-19 infection complaining of myalgia or muscle weakness to avoid the severe complications. Of note, while we did not test specifically for the Delta variant, given the time frame of the patient's presentation, we may consider the possibility of COVID-19 Delta variant related rhabdomyolysis.
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Introduction Cases of rhabdomyolysis causing myoglobinuria in post-COVID-19 patients have been seen occasionally, and exact mechanisms behind this seem multi-factorial. Some patients have severe myoglobinuria with highly elevated creatinine phosphokinase levels requiring urgent hemodialysis to keep creatinine and blood urea nitrogen levels under control and protect the kidneys from long-term damage. Case presentation We present a case of a 24-year-old man with autism who was admitted to the hospital for COVID-19 viral pneumonia and discharged without major complications. After 3 weeks, he came to the ER with a decreased mental status and asterixis, and labs indicated creatinine had increased from baseline 0.7 mg/dl to 2.9 mg/dl and eventually increased to 6.4 mg/dl despite IV hydration. Creatinine phosphokinase was ordered, and it was 289,500 mcg/L. The patient likely suffered acute tubular necrosis secondary to rhabdomyolysis. Urgent hemodialysis was initiated, and the patient showed clinical improvement after one week and was taken off dialysis in 2 weeks. During an outpatient Nephrology clinic visit, the creatinine level was close to baseline level at 0.9 mg/dl, and the patient was asymptomatic. Discussion Different viruses have been described to cause myositis and rhabdomyolysis. The list is long but not limited to influenza A and B, coxsackie, Epstein-Barr, herpes simplex, parainfluenza, adenovirus, cytomegalovirus, measles, varicellazoster, human immunodeficiency, and dengue. In addition, reports about myoglobinuria post-COVID-19 infection have been emerging. The mechanism is unclear, but one theory suggests muscular necrosis from the direct viral invasion of myocytes, and another one suggests a toxic effect on myocytes by the host response, i.e., cytokine release and other immunological factors. Hence, early clinical recognition of this entity can be lifesaving in some cases.
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INTRODUCTION: Myositis is a rare, underdiagnosed disease that presents in variable ways. Presentation is usually vague and non-specific. Physicians often attribute symptoms to deconditioning given the lack of objective findings at the first glance. DESCRIPTION: A 56-year-old African American woman who was diagnosed late with antisynthetase syndrome after progressing to hypoxic respiratory failure. The patient initially presented to the hospital complaining of progressive symptoms of shortness of breath and fatigue. She is a lifelong non-smoker and worked as a cook for 30 years with no significant occupational or environmental exposures. Initial vital signs were normal except for low oxygen saturation of 86% requiring 2 L oxygen. Physical exam was positive for trace lower extremity edema with no crackles or wheezing. Initial lab work was normal with negative COVID 19 test. Her CT scan revealed diffuse mild ground glass opacities. Echocardiogram showed grade I diastolic dysfunction. She was treated as heart failure and was started on diuretics without significant improvement. She was discharged on 2 L oxygen and diuretics. Her admissions recurred over the course of few months with progressive worsening of her imaging with diffuse GGO and increasing oxygen requirements for up to 5 L on exertion. Her symptoms of dyspnea progressed to the point where the patient was wheelchair bound and only able to walk a few feet. She was re admitted to the hospital and the pulmonary service was consulted. The patient was suspected of having a form of interstitial lung disease. Workup revealed normal ANA, RF, CCP, and ANCA. Her repeat echocardiogram revealed normal pulmonary artery pressures and normal ejection fraction. Her myositis panel showed positive PL1 antibodies and high CPK levels. A muscle biopsy was performed confirming necrotizing myopathy. The patient was diagnosed with antisynthetase syndrome. She was started on steroids then transitioned to Imuran with improvement in her symptoms. She is currently in pulmonary rehab and progressing as expected. DISCUSSION: This case helps highlight the importance of suspecting myositis as a cause of hypoxic respiratory failure that is otherwise unexplained. Undiagnosed, myositis may progress quickly and can involve the lungs.
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Introduction: Multisystem Inflammatory Syndrome in Children (MISC) is a known severe condition affecting children previously exposed to SARS-CoV-2. Cardiovascular manifestations in MIS-C are quite common and include myocardial dysfunction, coronary artery dilation or aneurysms, arrhythmias, conduction abnormalities, pericarditis and valvulitis. Severe cases can present even with cardiogenic shock. To date, little is known about the very early myocardial abnormalities in pediatric patients with MIS-C. The Speckle Tracking Echocardiography (STE) and cardiac MRI (cMRI) have shown to be potential candidate for identifying regional ventricular dysfunctions in early stages of inflammatory COVID-related conditions [1,2]. Objectives: To describe the early cardiac findings in patients with MIS-C, evaluated by two advanced cardiovascular imaging, STE and cMRI. Methods: Consecutive patients with MIS-C underwent standard transthoracic echocardiography (TTE), speckle-tracking echocardiography (STE) with analysis of left ventricle (LV) global longitudinal strain (GLS) and cardiac MRI (cMRI). Clinical and laboratory data, including markers of systemic inflammation, Troponin I (TnI) and Brain Natriuretic Peptide (BNP) were also collected at onset and during follow up. All patients received intravenous immunoglobulins (IVIGs), intravenous corticosteroids (methylprednisolone) and antiplatelet therapy (aspirin). The use of biological agents (Anakinra) was reserved to patients with severe or critical illness. The need for Intensive Care Unit (ICU) was based on clinical and hemodynamic status at presentation. Results: Twenty-three patients (13M, 10F), mean age 8.1±4years (range 5.4-15.7), all with positive clinical and/or serological evidence of previous SARS-COV2 infection, entered the study. The majority (78.2%) was caucasian. All presented high degree fever, gastrointestinal symptoms and rash. Conjunctivitis and cardiovascular symptoms, as hypotension, thoracic pain or dysrhythmia, were present in 10 (43.5%). Nine children (39.1%) shared Kawasaki Disease-like symptoms. Four patients (17.4%) needed ICU admission and 3 required inotropic support. Short-term survival was 100%. All patients showed an hyperinflammatory state with elevated CRP, ESR, and D-Dimer. Tn- I was abnormal (>34 ng/L) in 15 patients (65.2%), BNP was significantly elevated in 20 (86.9%). Median time to STE evaluation was 8 days and to cMRI was 18 days since fever onset. Mean LVEF and RVEF were respectively 59±10% and 45±7%. Coronary dilation was observed in 6 (26.1%) patients. STE showed reduced mean LVGLS (-17±4.3%). LVEF on cMR was 60±13%, LGE with non-ischemic pattern was evident in 6/16 patients (37.5%) and pericardial effusion in 2 (12.5%). Conclusion: MIS-C can occur in a small but not negligible proportion of children previously affected by COVID-19 and affects the heart in a significant proportion of them. STE and cMRI were shown to be very sensitive tools to evaluate and monitor the early cardiac dysfunctions in patients with MIS-C. The elevation of myocardial necrosis markers, the myocardial injury confirmed by reduced LVGLS and presence of LGE on cMR in about a quarter of the patients support the pathogenetic hypothesis of a post-viral immuno-mediated myocarditis.