Paediatric Inflammatory Multisystem Syndrome: Temporally Associated with SARS-CoV-2 (PIMS-TS): Cardiac Features, Management and Short-Term Outcomes at a UK Tertiary Paediatric Hospital

Abstract

Children were relatively spared during COVID-19 pandemic. However, the recently reported hyperinflammatory syndrome with overlapping features of Kawasaki disease and toxic shock syndrome-"Paediatric Inflammatory Multisystem Syndrometemporally associated with SARS-CoV-2" (PIMS-TS) has caused concern. We describe cardiac findings and short-term outcomes in children with PIMS-TS at a tertiary children's hospital. Single-center observational study of children with PIMS-TS from 10th April to 9th May 2020. Data on ECG and echocardiogram were retrospectively analyzed along with demographics, clinical features and blood parameters. Fifteen children with median age of 8.8 (IQR 6.4-11.2) years were included, all were from African/Afro-Caribbean, South Asian, Mixed or other minority ethnic groups. All showed raised inflammatory/cardiac markers (CRP, ferritin, Troponin I, CK and pro-BNP). Transient valve regurgitation was present in 10 patients (67%). Left Ventricular ejection fraction was reduced in 12 (80%), fractional shortening in 8 (53%) with resolution in all but 2. Fourteen (93%) had coronary artery abnormalities, with normalization in 6. ECG abnormalities were present in 9 (60%) which normalized in 6 by discharge. Ten (67%) needed inotropes and/or vasopressors. None needed extracorporeal life support. Improvement in cardiac biochemical markers was closely followed by improvement in ECG/echocardiogram. All patients were discharged alive and twelve (80%) have been reviewed since. Our entire cohort with PIMS-TS had cardiac involvement and this degree of involvement is significantly more than other published series and emphasizes the need for specialist cardiac review. We believe that our multidisciplinary team approach was crucial for the good short-term outcomes.

A dermatologic perspective on multisystem inflammatory syndrome in children

“…Because MIS-C is thought to be an immune-mediated secondary response to the virus, COVID PCRs are usually negative at the time of the illness, and antibodies are positive in the majority of cases. 11 , [14] , [15] , [16] , [17] , [18] , [19] , [20] , [21] Because diagnostic criteria for this condition are generally very broad, there is a significant challenge in identifying which patients falling under these diagnostic criteria are ‘true’ MIS-C. 22 (See Fig. 1 , Fig.…”

“…COVID PCR positivity ranged from 13% – 50%, while antibody positivity ranged from 75%–100%. [10] , [11] , [12] , [14] , [15] , [16] , [17] , [18] , [19] , [20] , [21] , 23 , 24 …”

“…Multiple larger case series published in the recent months [10] , [11] , [12] , [14] , [15] , [16] , [17] , [18] , [19] , [20] , [21] , 23 , 24 focus on characterizing and understanding MIS-C. Each case series has identified children who met criteria for MIS-C and described the cutaneous findings with which they presented. The following descriptive terminology was used to describe the skin findings: “conjunctivitis,” “eruption,” “red/cracked lips,” lips/oral cavity changes,” “cheilitis,” “extremity changes,” or “hand/feet edema”.…”

“…19 Looking at all the thirteen-case series presented in Table 3 , the percentage of children diagnosed with MIS-C who developed mucocutaneous findings included: conjunctivitis 27% – 93%, oral mucosal changes 25% – 87%, eruption 47% - 81%, and hand/feed erythema and edema 27% - 68%. Table 4 summarizes the top mucocutaneous manifestations of children with MIS-C. [10] , [11] , [12] , [14] , [15] , [16] , [17] , [18] , [19] , [20] , [21] , 23 , 24 The skin findings associated with MIS-C tend to be more common in younger children and decrease with age. 87% of children between 0–5 years old had mucocutaneous findings, compared to only 61.5% of those 13–20 years old.…”

Pediatric Inflammatory Multisystem Syndrome: Statement by the Pediatric Section of the European Society for Emergency Medicine and European Academy of Pediatrics

“…Recommendations for the intermediate and long-term follow-up of children with PIMS-TS after hospitals discharge should be guided by the tertiary specialty teams. Special consideration should be given to the need of performing post-discharge echocardiograms to exclude late cardiac complications ( 15 ).…”