Abstract
Initial reports on COVID-19 described children as largely spared from severe manifestations, with only 2–6% of children requiring intensive care treatment. However, since mid-April 2020, clusters of pediatric cases of severe systemic hyperinflammation and shock epidemiologically linked with COVID-19 have been reported. This condition was named as SARS-Cov-2-associated multisystem inflammatory syndrome in children and showed similarities to Kawasaki disease. Here, we present a narrative review of cases reported in literature and we discuss the clinical acute and follow-up management of these patients. Patients with SARS-Cov-2-associated multisystem inflammatory syndrome frequently presented with persistent fever, gastrointestinal symptoms, polymorphic rash, conjunctivitis, and mucosal changes. Elevated inflammatory markers and evidence of cytokine storm were frequently observed. A subset of these patients also presented with hypotension and shock (20–100%) from either acute myocardial dysfunction or systemic hyperinflammation/vasodilation. Coronary artery dilation or aneurysms have been described in 6–24%, and arrhythmias in 7–60%. Cardiac support, immunomodulation, and anticoagulation are the key aspects for the management of the acute phase. Long-term structured follow-up of these patients is required due to the unclear prognosis and risk of progression of cardiac manifestations.Conclusion: Multisystem inflammatory syndrome is a novel syndrome related to SARS-CoV-2 infection. Evidence is still scarce but rapidly emerging in the literature. Cardiac manifestations are frequent, including myocardial and coronary involvement, and need to be carefully identified and monitored over time.What is Known:• Multisystem inflammatory syndrome in children (MIS-C) has been described associated with SARS-CoV-2.What is New:• Patients with MIS-C often present with fever, gastrointestinal symptoms, and shock.• Cardiac involvement is found in a high proportion of these patients, including ventricular dysfunction, coronary artery dilation or aneurysm, and arrhythmias.• Management is based on expert consensus and includes cardiac support, immunomodulatory agents, and anticoagulation.• Long-term follow-up is required due to the unclear prognosis and risk of progression of cardiac manifestation.
Highlights
Initial reports during the early phase of the COVID-19 pandemic indicated that children were relatively spared from severe manifestations, with 2–6% of children presenting with severe illness [1–3]
Multisystem inflammatory syndrome is a novel syndrome related to SARS-CoV-2 infection
Cardiac involvement is found in a high proportion of these patients, including ventricular dysfunction, coronary artery dilation or aneurysm, and arrhythmias
Summary
MIS-C is a novel syndrome related to SARS-CoV-2 infection characterized by fever, signs of inflammation, and organ dysfunction. Evidence is still scarce but rapidly emerging from literature. Myocardial involvement, due to either acute myocarditis or secondary hyperinflammation, is frequent in children with MIS-C. Immunomodulation, and antiplatelet/anticoagulation treatments are part of the management of acute MIS-C. Follow-up of MIS-C patients is essential to better understand the evolution and prognosis of this disease. Future studies are needed to define evidence-based management of this novel condition. Authors’ contributions F.S. and A.D conceptualized the review, systematically reviewed the literature, evaluated articles for eligibility, extracted relevant data, interpreted the results, and drafted the manuscript. K.F., J.N., M.B.S., and C.J.V. critically revised the first draft of the manuscript and contributed with important intellectual content. All the authors approved the final version of the manuscript to be published and agree to be accountable for all aspects of the work.
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