Review of Cardiac Involvement in Multisystem Inflammatory Syndrome in Children

Alsaied, Tarek; Tremoulet, Adriana H.; Burns, Jane C.; Saïdi, A.; Dionne, Audrey; Lang, Sean M.; Newburger, Jane W.; Ferranti, Sarah D. de; Friedman, Kevin G.

doi:10.1161/circulationaha.120.049836

Cited by 241 publications

(324 citation statements)

References 43 publications

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“…However, human genetic factors may also underlie the differences in incidence between MIS-C and KD in Western versus Asian countries. Interestingly, KD studies predating COVID-19 suggested differences between ethnic groups for Kawasaki shock syndrome, a condition that affects ∼7% of KD patients (Alsaied et al, 2021). KD shock syndrome has a higher incidence in Western countries than in Asia (Li et al, 2019;Toubiana et al, 2020b), consistent with the observed geographic distribution of MIS-C.…”

Section: Introductionmentioning

confidence: 73%

“…Depending on the definition of myocarditis and the inclusion criteria used (cardiovascular or KD-like illness), cardiac dysfunction has been reported to affect 20-100% of patients (Abrams et al, 2020;Dufort et al, 2020;Friedman et al, 2020;Matsubara et al, 2020;Theocharis et al, 2020). Recent cardiac magnetic resonance imaging data have shown these patients to have a myocardial edema, rather than the necrosis often observed in common viral myocarditis (Alsaied et al, 2021;Blondiaux et al, 2020). All these symptoms may reflect local vasculitis and inflammation of the affected organ, and may account for the rapid, syndrome-resolving response to corticosteroid treatment (Belhadjer et al, 2020a;Ouldali et al, 2021).…”

Section: Comparison Of the Clinical Features Of Mis-c And Kdmentioning

confidence: 99%

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SARS-CoV-2–related MIS-C: A key to the viral and genetic causes of Kawasaki disease?

Sancho-Shimizu

Brodin

Cobat

et al. 2021

Journal of Experimental Medicine

106

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Multisystem inflammatory syndrome in children (MIS-C) emerged in April 2020 in communities with high COVID-19 rates. This new condition is heterogenous but resembles Kawasaki disease (KD), a well-known but poorly understood and clinically heterogenous pediatric inflammatory condition for which weak associations have been found with a myriad of viral illnesses. Epidemiological data clearly indicate that SARS-CoV-2 is the trigger for MIS-C, which typically occurs about 1 mo after infection. These findings support the hypothesis of viral triggers for the various forms of classic KD. We further suggest that rare inborn errors of immunity (IEIs) altering the immune response to SARS-CoV-2 may underlie the pathogenesis of MIS-C in some children. The discovery of monogenic IEIs underlying MIS-C would shed light on its pathogenesis, paving the way for a new genetic approach to classic KD, revisited as a heterogeneous collection of IEIs to viruses.

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Section: Introductionmentioning

confidence: 73%

Section: Comparison Of the Clinical Features Of Mis-c And Kdmentioning

confidence: 99%

SARS-CoV-2–related MIS-C: A key to the viral and genetic causes of Kawasaki disease?

Sancho-Shimizu

Brodin

Cobat

et al. 2021

Journal of Experimental Medicine

106

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“…Patients may present with high fever, lymphadenopathy, pleomorphic rash, conjunctivitis, mucosal pathology changes, and coronary artery dilation. Severe cases may present in cardiovascular shock, encephalitis, and multiple organ failure [8,[14][15][16]. However, obvious differences have also been found between the two syndromes in clinical presentations, diagnosis, and treatment.…”

Section: Introductionmentioning

confidence: 99%

Similarities and differences between multiple inflammatory syndrome in children associated with COVID-19 and Kawasaki disease: clinical presentations, diagnosis, and treatment

Zhang

2021

World J Pediatr

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Background Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection has been spreading rapidly around the world, while "multisystem inflammatory syndrome in children" (MIS-C) is a new type of syndrome that has now been reported in many countries. Similar and different characteristics between KD and MIS-C have been reported in a variety of literature. We aimed to focus on reviewing clinical presentations, diagnosis, and treatment of KD and MIS-C. Methods We searched articles in the electronic databases, including the Cochrane Library database, EMBASE, and MED-LINE with the keywords "multiple inflammatory syndrome" and/or "COVID-19" and/or "Kawasaki disease" and "children". Results Main presentations of MIS-C and KD include fever, rashes, mucous membrane involvement, conjunctivitis, hands and feet erythema/edema, and cervical lymphadenopathy. However, compared with the highest incidence of KD among some Asian countries, MIS-C is common among Black and Hispanic children. MIS-C is common in older children and teenagers, whereas classic KD is common in children under five years of age. Gastrointestinal symptoms, shock, and coagulopathy are common in MIS-C patients but are not common in classic KD. Cardiac manifestations are more common than KD, including myocarditis with cardiac dysfunction and coronary artery dilation or aneurysms. Severe cases in MIS-C present with vasodilated or cardiogenic shock that requires fluid resuscitation, muscular support, and even mechanical ventilation and extracorporeal membrane oxygenation (ECMO), whereas KD rarely presents with these manifestations and requires these treatments. Increased serum ferritin, leukopenia, lymphopenia and thrombocytopenia are common in MIS-C. However, thrombocytosis is a characteristic feature of KD. Intravenous immunoglobulin (IVIG) and moderate-high dose aspirin are still a standard recommended treatment for KD. In addition to the above-mentioned medications, steroids and biological drugs are frequently used in patients with MIS-C. Most of the children with KD have a good prognosis; however, the longterm clinical outcomes of MIS-C are not clear. Conclusions The overall presentation and treatment of MIS-C appear to overlap with KD. However, there are still great differences between the syndromes, and it is controversial to say whether MIS-C is a new entity or is a "severe type" of KD.

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“…Due to a high prevalence of intensive care needs, directly associated to the elevation of myocardial and in ammatory markers, a prompt recognition and treatment of MIS-C patient is mandatory (9). Up to now, immunomodulant treatment seems effective to recover from cardiac damage, but no studies evaluated long-term cardiovascular sequalae (2,(10)(11)(12).…”

Section: Introductionmentioning

confidence: 99%

Simil-appendicitis presentation may precede cardiac involvement in MIS-C patients

Trevisan¹,

Amaddeo²,

Taddio³

et al. 2021

Preprint

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BackgroundMultisystem inflammatory syndrome in children (MIS-C) is a new clinical entity characterized by a systemic hyperinflammation triggered by SARS-CoV-2 infection in children and adolescents. This condition could potentially involve all organs with main complications concerning cardiovascular system. Despite up to 90% of patients complain gastrointestinal symptoms (nausea, vomit and diarrhea), a presentation mimicking acute appendicitis has rarely been reported, and can be the presenting feature of the disease, potentially leading to misdiagnosis and delayed treatment.Case presentationA 15-year-old boy presented to the Emergency Department for a two-day history of fever, vomiting and mild abdominal pain. One month before, the patient complained ageusia and anosmia while his mother tested positive for Sars-CoV2 nasopharyngeal swab. At admission, laboratory tests showed leukocytosis with lymphopenia and elevation of inflammatory markers, while cardiac enzymes, electrocardiogram and echocardiography were unremarkable. An abdominal ultrasound displayed a thickening of terminal ileus and cecum with ascites. Because of the worsening abdominal pain and a physical examination suggestive of acute appendicitis, a laparoscopy was performed but no surgical condition was found. After surgery, fever and generalized malaise persisted, so a cardiac evaluation was repeated, showing a relevant increase in inflammatory markers and cardiac enzymes. Electrocardiogram demonstrated a QTc prolongation with mild decrease in left ventricular ejection fraction at echocardiogram. A MIS-C was diagnosed and intravenous immunoglobulin along with a steroid treatment started. After 36 hours, the patient presented a complete clinical recovery with fever cessation. Cardiac anomalies normalized in three weeks.ConclusionMIS-C has been defined as a systemic inflammation, involving at least two organs, after a previous SARS-CoV2 infection in children and adolescents. Physicians should be aware that while gastrointestinal manifestations are common, a pseudo appendicitis presentation may also occur, leading to misdiagnosis and delayed treatment. This report suggests that in patients with symptoms suggestive of an acute appendicitis, the presence of lymphopenia, hypoalbuminemia and ultrasound images of terminal ileus inflammation, should raise the suspect for MIS-C even without initial overt signs of cardiac involvement.

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Review of Cardiac Involvement in Multisystem Inflammatory Syndrome in Children

Cited by 241 publications

References 43 publications

SARS-CoV-2–related MIS-C: A key to the viral and genetic causes of Kawasaki disease?

SARS-CoV-2–related MIS-C: A key to the viral and genetic causes of Kawasaki disease?

Similarities and differences between multiple inflammatory syndrome in children associated with COVID-19 and Kawasaki disease: clinical presentations, diagnosis, and treatment

Simil-appendicitis presentation may precede cardiac involvement in MIS-C patients

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