A regional multidisciplinary team approach from the general paediatric perspective, in a tertiary centre for suspected PIMS-TS

BackgroundAlder Hey Children’s Hospital (AHCH) in Northwest England provides regional specialist services and local general paediatric care. Paediatric multisystem inflammatory syndrome temporally associated with Covid-19 (PIMS-TS) is a new disease entity requiring paediatricians from District General Hospitals (DGHs) to seek advice from a number of specialists. During the first/second peak of the pandemic, patients with suspected PIMS-TS were transferred to AHCH under general paediatricians with subspecialist input.To optimise patient care, during the 3rd peak of the pandemic (January 2021) an efficient virtual multidisciplinary team (MDT) consisting of rheumatology, cardiology, infectious disease and general paediatrics, was created to facilitate discussion of potential PIMS-TS cases. The MDT aimed to coordinate management of patients requiring specialist input, reduce unnecessary transfers whilst ensuring appropriate case management and utilisation of resources.Daily (including weekends) virtual meetings were held to discuss all active referrals including the patient’s lead clinician, with the option of discussion on consecutive days until clinical improvement.ObjectivesTo analyse the cohort discussed at the MDT (January-March 2021), including demographics, outcomes, and need for transfer to AHCH.MethodsAn online referral form using Microsoft SharePoint was distributed to regional DGHs for completion prior to the MDT. Discussion was documented on a database and outcome uploaded to AHCH patient records. These records were reviewed for this study.Results35 patients were referred over the 6 week study period. 14 (44%) were female. 21 referrals came from DGHs (66%), the remainder were internal. Age range was 4 months to 17 years;9.3% <1year, 25% 2–5 years, 40.6% 6–11 years and 25% 12–17 years. At time of discussion, six had a positive covid-19 PCR test and 13 had a confirmed positive household/family contact. PIMS-TS was diagnosed in 7(20%) patients, three of whom were referred from DGHs. One required transfer to AHCH for inotropic support and one for echocardiogram. Two additional transfers to AHCH for surgical opinions were subsequently referred to the MDT. Of the remaining 25 patients (18 from DGHs), four were treated locally for Kawasaki’s disease. 19 of 21 (90.5%) DGH referrals to the MDT (majority without PIMS-TS) avoided unnecessary transfer to AHCH for assessment.ConclusionsThe daily virtual MDT allowed efficient discussion with exchange of expertise and a collaborative approach from several specialties for suspected PIMS-TS cases across the region. It also enabled continuity across multiple discussions about individual cases. It provided the opportunity to discuss differentials in a new disease entity, empower DGH clinicians to start early treatment of PIMS-TS and recruit where appropriate to the RECOVERY trial. Unnecessary transfers were avoided in 90.5% of external cases. General paediatricians are key valued team members as the spectrum of disease and possible differential is wide. This MDT approach promoted the role of the general paediatrician in caring for these patients. Early treatment with a lower threshold to react and escalate has improved patient care. The use of better IT infrastructure helped bridge the gap of care delivery by geography. Feedback from DGH participants in the MDT was positive.

Cardiac pathology and outcomes vary between Kawasaki disease and PIMS-TS.

Overlapping clinical features promoted the discussion of whether Kawasaki disease (KD) and PIMS-TS share pathophysiological features and disease outcomes. Medical records from English patients with KD (2015-02/20, N = 27) and PIMS-TS (02/2020-21, N = 34) were accessed to extract information. Children with PIMS-TS were older and more frequently of minority ethnicity background. They patients more commonly exhibited cytopenias and hyperferritinemia, which associated with diffuse cardiac involvement and functional impairment. In some PIMS-TS cases, cardiac pathology developed late, but outcomes were more favorable. In both, KD and PIMS-TS, baseline coronary diameter was a predictor of outcomes. PIMS-TS treatment more frequently included respiratory and cardiovascular support, and corticosteroids with IVIG. Cardiac involvement in PIMS-TS may be the result of a cytokine storm. Though more severe and diffuse when compared to KD, cardiac involvement of PIMS-TS has a more favorable prognosis, which may, after recovery, mitigate the need for long-term follow up.

Successful Management of a COVID-19 Positive Infant With Transposition of the Great Arteries.

A six-week-old infant presented in extremis and was diagnosed with dextro-transposition of the great arteries, intact ventricular septum, features of left ventricular deconditioning, and abnormal coronary arteries. Treatment with prostaglandin E1 and balloon atrial septostomy was insufficient, necessitating extracorporeal membrane oxygenation (ECMO). Severe acute respiratory syndrome coronavirus-2 was detected. The arterial switch operation was delayed by eight days because of COVID-19. Although stable on ECMO, the infant was treated with remdesivir. Extracorporeal membrane oxygenation was not required postoperatively with chest closure on day 2 and extubation on day 5.

Acute Cardiovascular Manifestations in 286 Children With Multisystem Inflammatory Syndrome Associated With COVID-19 Infection in Europe.

BACKGROUND: The aim of the study was to document cardiovascular clinical findings, cardiac imaging, and laboratory markers in children presenting with the novel multisystem inflammatory syndrome associated with coronavirus disease 2019 (COVID-19) infection. METHODS: This real-time internet-based survey has been endorsed by the Association for European Paediatric and Congenital Cardiologists Working Groups for Cardiac Imaging and Cardiovascular Intensive Care. Children 0 to 18 years of age admitted to a hospital between February 1 and June 6, 2020, with a diagnosis of an inflammatory syndrome and acute cardiovascular complications were included. RESULTS: A total of 286 children from 55 centers in 17 European countries were included. The median age was 8.4 years (interquartile range, 3.8-12.4 years) and 67% were boys. The most common cardiovascular complications were shock, cardiac arrhythmias, pericardial effusion, and coronary artery dilatation. Reduced left ventricular ejection fraction was present in over half of the patients, and a vast majority of children had raised cardiac troponin when checked. The biochemical markers of inflammation were raised in most patients on admission: elevated C-reactive protein, serum ferritin, procalcitonin, N-terminal pro B-type natriuretic peptide, interleukin-6 level, and D-dimers. There was a statistically significant correlation between degree of elevation in cardiac and biochemical parameters and the need for intensive care support (P<0.05). Polymerase chain reaction for severe acute respiratory syndrome coronavirus 2 was positive in 33.6%, whereas immunoglobulin M and immunoglobulin G antibodies were positive in 15.7% cases and immunoglobulin G in 43.6% cases, respectively, when checked. One child in the study cohort died. CONCLUSIONS: Cardiac involvement is common in children with multisystem inflammatory syndrome associated with the Covid-19 pandemic. The majority of children have significantly raised levels of N-terminal pro B-type natriuretic peptide, ferritin, D-dimers, and cardiac troponin in addition to high C-reactive protein and procalcitonin levels. In comparison with adults with COVID-19, mortality in children with multisystem inflammatory syndrome associated with COVID-19 is uncommon despite multisystem involvement, very elevated inflammatory markers, and the need for intensive care support.

Presentation, Treatment Response and Short-Term Outcomes in Paediatric Multisystem Inflammatory Syndrome Temporally Associated with SARS-CoV-2 (PIMS-TS).

The novel Severe Acute Respiratory Syndrome Coronavirus 2 (SARS-CoV-2) is the pathogen responsible for Coronavirus Disease 2019 (COVID-19). Whilst most children and young people develop mild symptoms, recent reports suggest a novel paediatric inflammatory multisystem syndrome temporally associated with SARS-CoV-2 (PIMS-TS). Case definition and classification are preliminary, treatment is empiric and disease-associated outcomes are unclear. Here, we report 29 patients with PIMS-TS who were diagnosed, admitted and treated in the English North West between March and June 2020. Consistent with patterns observed internationally, cases peaked approximately 4 weeks after the initial surge of COVID-19-like symptoms in the UK population. Clinical symptoms included fever (100%), skin rashes (72%), cardiovascular involvement (86%), conjunctivitis (62%) and respiratory involvement (21%). Some patients had clinical features partially resembling Kawasaki disease (KD), toxic shock syndrome and cytokine storm syndrome. Male gender (69%), black, Asian and other minority ethnicities (BAME, 59%) were over-represented. Immune modulating treatment was used in all, including intravenous immunoglobulin (IVIG), corticosteroids and cytokine blockers. Notably, 32% of patients treated with IVIG alone went into remission. The rest required additional treatment, usually corticosteroids, with the exception of two patients who were treated with TNF inhibition and IL-1 blockade, respectively. Another patient received IL-1 inhibition as primary therapy, with associated rapid and sustained remission. Randomized and prospective studies are needed to investigate efficacy and safety of treatment, especially as resources of IVIG may be depleted secondary to high demand during future waves of COVID-19.