Acute heart failure in multisystem inflammatory syndrome in children (MIS-C) in the context of global SARS-CoV-2 pandemic


Acute heart failure in multisystem inflammatory syndrome in children (MIS-C) in the context of global SARS-CoV-2 pandemic.

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Belhadjer Z, Méot M, Bajolle F, Khraiche D, Legendre A, Abakka S, Auriau J, Grimaud M, Oualha M, Beghetti M, Wacker J, Ovaert C, Hascoet S, Selegny M, Malekzadeh-Milani S, Maltret A, Bosser G, Giroux N, Bonnemains L, Bordet J, Di Filippo S, Mauran P, Falcon-Eicher S, Thambo JB, Lefort B, Moceri P, Houyel L, Renolleau S, Bonnet D.Circulation. 2020 May 17. doi: 10.1161/CIRCULATIONAHA.120.048360. Online ahead of print.PMID: 32418446

Take Home Points:

  • Multisystem inflammatory syndrome in children (MIS-C) is a new syndrome that is temporally associated with exposure to COVID-19.
  • Multisystem inflammatory syndrome in children shares similarities with atypical Kawasaki disease, but many clinical signs are unique.
  • MIS-C is characterized by patients with recent diagnosis of COVID-19 presenting with fever, laboratory evidence of inflammation, and evidence of clinically severe illness requiring hospitalization, with multisystem (≥2) organ involvement (cardiac, renal, respiratory, hematologic, gastrointestinal, dermatologic, or neurologic) in the absence of other plausible diagnoses.
  • Additional studies are needed to determine the full spectrum of this illness and its long-term effects on cardiac structure and function.

Dr Shaji Menon

Commentary from Dr. Shaji Menon (Salt Lake City, Utah), section editor of Pediatric Cardiology Journal Watch:   This retrospective multicenter study from 12 hospitals in France and 1 hospital in Switzerland presents data for children with acute left ventricular systolic dysfunction or cardiogenic shock and associated multisystem inflammatory syndrome between March 22 and April 30, 2020.  The inclusion criteria were the presence of fever (>38.5°C), cardiogenic shock, or acute left ventricular dysfunction (left ventricular ejection fraction <50%) with inflammatory state (C-reactive protein >100 mg/mL). 35 patients fulfilling the inclusion criteria with febrile

cardiogenic shock or left ventricular dysfunction and inflammatory state were included in the study. All children presented with fever (>38.5°C). Gastrointestinal symptoms including abdominal pain, vomiting, or diarrhea present in majority (80%). Two patients underwent emergency operation for suspected appendicitis that was ultimately diagnosed as mesenteric adenolymphitis.

Although clinical signs mimicking Kawasaki disease were common, including skin rash, cheilitis, cervical adenopathy, and meningism none of the patients met criteria for classic Kawasaki disease (Table 1). Only 6 patients complained of chest pain. The ECG was not specific, with ST-segment elevation in only 1 patient (Table 2). In a large proportion of patients, the hemodynamic presentation at admission to the pediatric ICU was shock with low systemic blood pressure. The median duration between the first clinical symptoms and symptoms of heart failure was 6 days (interquartile range, 4.5–6 days). Ventricular systolic dysfunction with global hypokinesia was common. One patient manifested takotsubo syndrome presentation with akinesis of the apical segment. Segmental hypokinesia and pericardial effusion was seen in 3 patients. Right ventricular systolic function was preserved in all patients. Dilatation of the coronary arteries (Z score >2 adjusted for body temperature) was found in 6 patients (17%), including 5 patients with dilatation of the left main stem and 1 patient with dilatation of the right coronary artery.

The majority of patients received intravenous immunoglobulin (25 of 35 of patients). Twelve patients received intravenous steroids, 3 children received anakinra because of persistent severe inflammatory state and 23 of 35 patients were treated with therapeutic-dose heparin. Complete recovery of left ventricular systolic function was observed in 71% of patients. Five patients had residual mild to moderate left ventricular systolic dysfunction. None had a thrombotic or embolic event. Median ICU stay was 7 days (interquartile range, 3.7–10 days), and median hospital stay was 10 days (interquartile range, 8–14 days). Unlike patients with Kawasaki disease, median age of patients with MIS-C were older (10 years) and left ventricular dysfunction was more common at presentation.

Clinical Signs and Symptoms

Cardiac Signs

Laboratory Findings

Treatment and Responses

Maculopapular rash in a 12-year-old girl.

Maculopapular rash in a 12-year-old girl

sars-cov-2 related multisystem inflammation