One of the rare manifestations of COVID-19 is pediatric inflammatory multisystem syndrome, most often in publications it is found out how pediatric inflammatory multisystem syndrome in children (РIMS). The progression of the inflammatory response in РIMS may be a consequence of the development of secondary hemophagocytic syndrome, a significant part of which is refractory fever, an increase in ferritin, AST and ALT, cytopenia, an increase in interleukin 6, severe hepatic and neurological dysfunction. The case with this group of representatives of other multiple organ failure manifestations in the detection of acute distress syndrome and acute heart failure, in the detection of the development of myocarditis. A feature of the described clinical detection is the dynamic observation of a severe pediatric multisystem inflammatory syndrome associated with COVID-19, complicated by myocarditis with acute heart failure, left ventricular dysfunction and secondary hemophagocytic syndrome, in an 11-year-old girl with underlying obesity. The child is taking triple therapy with glucocorticosteroids in combination with intravenous immunoglobulin and cyclosporine A, with an expected effect on the ongoing treatment. Possible features of observation are noted: 1) a very early start of РIMS during the acute phase of СOVID-19; 2) transient dyslipidemia at the onset of РIMS with an increase in cholesterol, mainly due to the low-density lipoprotein fraction, and spontaneous regression without lipid-lowering drugs; 3) MRI signs of myocarditis in a month after the onset of the disease, despite the normalization of echocardiography and pro-BNP.
The pathogenetic basis for Pediatric Multisystem Inflammatory Syndrome (PMIS) associated with SARS-CoV-2 is systemic vasculitis resulting from hyperproduction of pro-inflammatory cytokines associated with dysregulation of the immune response. Clinical manifestations of PMIS include fever with features of Kawasaki Syndrome (KS) and multi-organ dysfunction coupled with frequent unusual heart involvement, ranging from mild with minimal ECG changes or elevated troponin to fulminant myocarditis or Takotsubo syndrome (TTS). The purpose of the research was to study the characteristics of cardiovascular disorders in Kawasaki-like multisystem inflammatory syndrome associated with COVID-19 in children observed in the East Siberian area of Russia. A single-center retrospective cohort study was carried out on the basis of the Irkutsk Oblast Regional Children's Clinical Hospital (Irkutsk, Russia): 36 patients with PMIS associated with COVID-19 in Nov. 18, 2020 - Dec. 31, 2021, including 21 boys (M/F ratio 1.4:1) at the average age of 7.5 years old (1.5 months to 17 years old). The diagnostic signs of KS were observed in 34 (94%) children, including the complete set of diagnostic signs in 21 (58%). Refractory fever and a sharp increase in inflammatory biomarkers were detected in all 36 (100%) children; multiorgan dysfunction in 31 (86%); thrombocytopenia in 24 (67%); gastrointestinal syndrome in 25 (69%); cerebral dysfunction in 24 (66)%; various cardiovascular disorders in 30 (83%), including: dilatation of the left ventricle (LV) and a decrease in myocardial contractility in 3 (8%); moderate coronary dilatation in 5 (14%); thickening of the posterior LV walls in diastole in 5 (13%); thickening of the interventricular septum in diastole in 6 (16%). According to the MRI results performed in 15 children the signs of myocardial damage were found in 6 (40%). LV dilatation and decreased myocardial contractility were noted in 3 (8%) children, clinically apparent thrombotic complications in 3 (8%) children as well, and severe neurological deficit in one patient (3%). An increase in the level of D-dimer was observed in 26 (72%) patients, thrombocytopenia in 24 (67%), a statistically significant correlation between thrombocytopenia and myocardial damage was found (R=-0.506, p=0.001). The treatment measures were given as follows: inotropic support (dobutrex/dobutamine) was received by 8 (22%), artificial lung ventilation - by 8 (22%), intravenous human immunoglobulin 1.5-2.0 g/kg/course - by 28 (77%), dexamethasone - by 24 (67%), tocilizumab - by 2 (5%) patients. All 36 (100%) patients have survived. Conclusion: the Kawasaki-like multisystem inflammatory syndrome associated with COVID-19 was accompanied by the cardiovascular disorders (pericarditis, arrhythmias, abnormal ECG changes, elevated troponin and/or natriuretic peptide, LV thickening on echocardiography, coronary dilatation) in most patients, although LV dilatation and decreased myocardial contractility were observed in a few cases only. A negative correlation between thrombocytopenia and myocardial damage was found, which may indicate the involvement of thrombotic microvascular inflammation in the genesis of myocardial disorders.
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