Discussion and review of literature:
The 2019 novel coronavirus disease is caused by the SARS-CoV-2. The virus replicates in symptomatic and asymptomatic people and has resulted in more than 323,000 deaths worldwide as of May 20th, 2020 (https://coronavirus.jhu.edu/map.html). Disease presentation varies from nonspecific symptoms (fever, cough, sore throat, malaise, abdominal pain, diarrhea, vomiting)1,7 to pneumonia and acute respiratory distress syndrome (ARDS).2Cardiovascular involvement is increasingly recognized in critically ill adult patients; the spectrum varies from myocarditis to acute heart failure/cardiomyopathy, and may increase the risk of acute myocardial infarction.4 In adults indirect cardiac injury related to a hyperinflammatory process and cytokine storm has been noted.8
On April 27th 2020, the Paediatric Intensive Care Society released a statement describing a rise in critically ill pediatric patients presenting with severe multisystem inflammatory disease related to COVID-19 infection. A recently published report,7 together with numerous anecdotal reports from Spain, France, the United Kingdom and the Unites states have described patients presenting with COVID-19 related hyperinflammatory shock syndrome. These patients had a range of clinical presentations including classic Kawasaki disease, atypical Kawasaki disease and toxic shock syndrome, with and without cardiac dysfunction. The mechanism of the cardiac injury is unclear but may be associated with a dramatic inflammatory response and cytokine storm.7,8 The clinical presentations reported are very similar to our patient, with unrelenting fever, severe gastrointestinal symptoms, hypotension, tachycardia, and minimal to no respiratory involvement. Elevated markers of cardiac injury have also been commonly reported in these patients pointing towards a more global cardiac involvement as part of the hyperinflammatory shock syndrome.7 None of these reports in children however describe concomitant focal myocarditis. Our case presented with focal electrocardiographic abnormalities (T-wave inversion in inferior leads) as well as regional wall motion abnormalities on echocardiography prompting further investigation for other potential mechanisms of cardiac involvement, such as inflammation.
Inflammation of cardiac muscle, or myocarditis, may be due to direct virus-related injury to cardiomyocytes or as a consequence of indirect injury from systemic inflammatory response syndrome or ARDS-induced hypoxemia.9 Currently, CMRI is the non-invasive gold standard to detect myocardial dysfunction, edema, and fibrosis, and to diagnose myocarditis.10 Since its emergence in December 2019, there have been few reported cases of COVID-19 related myocarditis in adults6, but none in children. Due to focal electrocardiographic abnormalities, regional dysfunction on echocardiography, and reports of varying cardiovascular involvement in adults with COVID-19, we obtained a CMRI to further delineate the mechanism of cardiovascular involvement in our patient. CMRI confirmed a diagnosis of focal myocarditis (suggested by T2 hyper-intensity and mid-wall LGE) apart from a previously well-described phenomenon of global cardiac dysfunction related to hyperinflammatory syndrome. In addition, CMRI provided other useful information in terms of diagnosing RV dysfunction (not well visualized by Lumify), and ruling out acute coronary syndrome, reported in adult patients.6 In contrast to coronary involvement in children with multisystem inflammatory disease related to COVID-19 infection,7our patient had no coronary involvement, but rather cardiac dysfunction and features consistent with focal myocarditis.