Inflammatory Heart Disease in Multisystem Inflammatory Syndrome

Abstract

Purposeof the review: In this review article, we aim to provide an overview of the pathophysiology, the clinical features, the therapeutic management and prognosis of patients affected by Multisystemic inflammatory syndrome (MIS) with cardiac involvement, focusing on myocarditis and pericarditis.

Recent findings: MIS is a multiorgan hyperinflammatory condition due to a cytokine storm following (within 4-12 weeks) SARS-CoV-2 (Severe Acute Respiratory Syndrome Coronavirus 2) infection. First described in children, it also affects young adults without comorbidities, predominantly males with highly heterogeneous clinical manifestations, including cardiac involvement. Pericardial and myocardial involvement are prevalent among patients affected by MIS leading to different clinical manifestations including myocarditis with arrhythmias, acute heart failure and cardiogenic shock that significantly affect the patient's prognosis. The heterogeneity of its clinical features and the significant overlap with other hyperinflammatory diseases make the diagnosis particularly challenging. Moreover, the evidence on the efficacy of pharmacological treatments targeting the hyperinflammatory response is scarce, as well as data on long-term prognosis.

Keywords: Heart failure; Inflammation; Myocarditis; Pericarditis; Severe Acute Respiratory Syndrome Coronavirus 2.

Fig. 1

Myocardial and Pericardial Manifestations of Multisystemic Inflammatory Syndrome with Cardiac Involvement. The figure illustrates the clinical manifestations, laboratory and imaging (both at echocardiography and cardiovascular magnetic resonance) findings of Multisystem Inflammatory Syndrome (MIS) with myocardial and pericardial involvement. The figure summarizes the main pathophysiological mechanisms leading to the cytokine storm underlying the cardiac manifestations of this syndrome. Figure created with BioRender.com. Abbrev: AV: atrio-ventricular; ECG: electrocardiogram; CMR: cardiovascular magnetic resonance; CRP: C-reactive protein; echo: echocardiogram; ESR: erythrocyte sedimentation rate; IL: interleukin; LGE: late gadolinium enhancement; LV: left ventricular; NLRP3 inflammasome: pyrin domain-containing-3 inflammasome; NT-proBNP: N- terminal pro-b-type natriuretic peptide; RMWA: regional wall motion abnormality; SARS-CoV-2: severe acute respiratory syndrome coronavirus 2; STIR: short-tau inversion recovery; VES:; VT: ventricular tachycardia; VF: ventricular fibrillation