Case Report: Lymphohistiocytic Myocarditis With Severe Cardiogenic Shock Requiring Mechanical Cardiocirculatory Support in Multisystem Inflammatory Syndrome Following SARS-CoV-2 Infection

Xavier Bemtgen; Karin Klingel; Markus Hufnagel; Ales Janda; Christoph Bode; Dawid L Staudacher; Alexander Supady; Ilona Jandova

doi:10.3389/fcvm.2021.716198

Case Report: Lymphohistiocytic Myocarditis With Severe Cardiogenic Shock Requiring Mechanical Cardiocirculatory Support in Multisystem Inflammatory Syndrome Following SARS-CoV-2 Infection

Front Cardiovasc Med. 2021 Sep 9:8:716198. doi: 10.3389/fcvm.2021.716198. eCollection 2021.

Authors

Xavier Bemtgen^{1

2}, Karin Klingel³, Markus Hufnagel⁴, Ales Janda⁵, Christoph Bode^{1

2}, Dawid L Staudacher^{1

2}, Alexander Supady^{1

2

6}, Ilona Jandova⁷

Affiliations

¹ Department of Cardiology and Angiology I (Heart Center Freiburg - Bad Krozingen), University Medical Center - University of Freiburg, Faculty of Medicine, University of Freiburg, Freiburg, Germany.
² Department of Medicine III (Interdisciplinary Medical Intensive Care), University Medical Center - University of Freiburg, Faculty of Medicine, University of Freiburg, Freiburg, Germany.
³ Cardiopathology, Institute for Pathology and Neuropathology, University Hospital Tübingen, Tübingen, Germany.
⁴ Department of Pediatrics and Adolescent Medicine, University Medical Center, Medical Faculty, University of Freiburg, Freiburg, Germany.
⁵ Department of Pediatrics and Adolescent Medicine, Ulm University Medical Center, Ulm, Germany.
⁶ Heidelberg Institute of Global Health, University of Heidelberg, Heidelberg, Germany.
⁷ Department of Rheumatology and Clinical Immunology, University Medical Center, Faculty of Medicine, University of Freiburg, Freiburg, Germany.

Abstract

Multisystem Inflammatory Syndrome (MIS) is a novel hyperinflammatory syndrome associated with SARS-CoV-2 infection. It predominantly affects children (MIS-C) a few weeks after a usually asymptomatic SARS-CoV-2 infection and is only rarely seen in adults above 21 years (MIS-A). Only scarce data on histological findings in both pediatric and adult patients has been published so far. An 18-year-old male patient was admitted to hospital in a febrile state, which progressed to severe cardiogenic shock and multi-organ failure requiring extracorporeal life support. Myocardial biopsy revealed small vessel-associated immune cell infiltrates. Diagnosis of MIS-C was made after ruling out all potential differential diagnosis. Use of immunosuppressive treatment with steroids, interleukin-1 blockade and high-dose intravenous immunoglobulins resulted in the patient's full recovery. Multisystem Inflammatory Syndrome (MIS) is a new differential diagnosis of cardiac dysfunction in pediatric and adult patients. The lack of myocardial necrosis differentiates the disease from other viral myocarditis and offers an explanation for the fast response to immunomodulatory therapy and the favorable prognosis. The preceding SARS-CoV-2 infection might only have been mildly symptomatic or even asymptomatic.

Keywords: COVID-19; Impella®; MIS-C; Multisystem Inflammatory Syndrome in children; V-A ECMO; myocardial biopsy.

Publication types

Case Reports