Left Atrial Strain in Multisystem Inflammatory Syndrome in Children and Associations with Systemic Inflammation and Cardiac Injury.

Multisystem inflammatory syndrome in children (MIS-C) commonly involves cardiac injury with both systolic and diastolic dysfunction. Left atrial strain (LAS) detects subclinical diastolic dysfunction in adults but is infrequently used in children. We evaluated LAS in MIS-C and the associations with systemic inflammation and cardiac injury. In this retrospective cohort study, LAS parameters [reservoir (LAS-r), conduit (LAS-cd), and contractile (LAS-ct)] obtained from admission echocardiograms of MIS-C patients were compared to healthy controls and between MIS-C patients with and without cardiac injury (BNP > 500 pg/ml or troponin-I > 0.04 ng/ml). Correlation and logistic regression analyses were performed to assess LAS associations with admission inflammatory and cardiac biomarkers. Reliability testing was performed. We identified 118 patients with MIS-C and 20 healthy controls. Median LAS parameters were reduced in MIS-C patients compared to controls (LAS-r: 31.8 vs. 43.1%, p < 0.001; LAS-cd: - 28.8 vs. - 34.5%, p = 0.006; LAS-ct: - 5.2 vs. - 9.3%, p < 0.001) and reduced in MIS-C patients with cardiac injury (n = 59) compared to no injury (n = 59) (LAS-r: 29.6 vs. 35.8%, p = 0.001; LAS-cd: - 26.5 vs. - 30.4%, p = 0.036; LAS-ct: - 4.6 vs. - 9.3%, p = 0.008). A discrete LAS-ct peak was absent in 65 (55%) MIS-C patients but present in all controls (p < 0.001). Procalcitonin correlated strongly with averaged E/e' (r = 0.55, p = 0.001). Moderate correlations were found for ESR and LAS-ct (r = - 0.41, p = 0.007) as well as BNP and LAS-r (r = - 0.39, p < 0.001) and LAS-ct (r = 0.31, p = 0.023). Troponin-I had only weak correlations. Intra-rater reliability was good for all LAS parameters, and inter-rater reliability was good to excellent for LAS-r, and fair for LAS-cd and LAS-ct. LAS analysis, particularly the absence of a LAS-ct peak, was reproducible and may be superior to conventional echocardiographic parameters for detecting diastolic dysfunction in MIS-C. No strain parameters on admission were independently associated with cardiac injury.

Baseline Echocardiography and Laboratory Findings in MIS-C and Associations with Clinical Illness Severity.

Children with COVID-associated multisystem inflammatory syndrome (MIS-C) may develop severe disease. We explored the association of admission echocardiographic and laboratory parameters with MIS-C disease severity. This retrospective, single center study of consecutive MIS-C patients (4/2020-12/2021) excluded those with preexisting cardiomyopathy, congenital heart disease, or prior cardiotoxic therapy. Our hypothesis was that worse admission echocardiographic and laboratory parameters were associated with more severe disease based on vasoactive medication use. Univariable and multivariable logistic regression models assessed the association between vasoactive medication use and baseline variables. Of 118 MIS-C patients, median age was 7.8 years (IQR 4.6, 11.8), 48% received vasoactive medication. Higher admission brain natriuretic peptide [OR 1.07 (95% CI 1.02,1.14), p = 0.019], C-reactive protein [OR 1.08 (1.03,1.14), p = 0.002], troponin [OR 1.05 (1.02,1.1), p = 0.015]; lower left ventricular ejection fraction [LVEF, OR 0.96 (0.92,1), p = 0.042], and worse left atrial reservoir strain [OR 0.96 (0.92,1), p = 0.04] were associated with vasoactive medication use. Only higher CRP [OR 1.07 (1.01, 1.11), p = 0.034] and lower LVEF [0.91 (0.84,0.98), p = 0.015] remained independently significant. Among those with normal admission LVEF (78%, 92/118), 43% received vasoactive medication and only higher BNP [OR 1.09 (1.02,1.19), p = 0.021 per 100 pg/mL] and higher CRP [OR 1.07 (1.02,1.14), p = 0.013] were associated with use of vasoactive medication. Nearly half of all children admitted for MIS-C subsequently received vasoactive medication, including those admitted with a normal LVEF. Similarly, admission strain parameters were not discriminatory. Laboratory markers of systemic inflammation and cardiac injury may better predict early MIS-C disease severity.

Proposed competencies for the performance of cardiovascular computed tomography in pediatric and adult congenital heart disease.

Cardiovascular computed tomography (CCT) is rated appropriate by published guidelines for the initial evaluation and follow up of congenital heart disease (CHD) and is an essential modality in cardiac imaging programs for patients of all ages. However, no recommended core competencies exist to guide CCT in CHD imaging training pathways, curricula development, or establishment of a more formal educational platform. To fill this gap, a group of experienced congenital cardiac imagers, intentionally inclusive of adult and pediatric cardiologists and radiologists, was formed to propose core competencies fundamental to the expert-level performance of CCT in pediatric acquired and congenital heart disease and adult CHD. The 2020 SCCT Guideline for Training Cardiology and Radiology Trainees as Independent Practitioners (Level II) and Advanced Practitioners (Level III) in Cardiovascular Computed Tomography (1) for adult imaging were used as a framework to define pediatric and CHD-specific competencies. Established competencies will be immediately relevant for advanced cardiac imaging fellowships in both cardiology and radiology training pathways. Proposed future steps include radiology and cardiology society collaboration to establish provider certification levels, training case-volume recommendations, and continuing medical education (CME) requirements for expert-level performance of CCT in pediatric and adult CHD.

Left atrial strain in multisystem inflammatory syndrome in children (MIS-C) and associations with systemic inflammation and cardiac injury.

Background: Multisystem inflammatory syndrome in children (MIS-C) commonly involves cardiac injury with both systolic and diastolic dysfunction. Left atrial strain (LAS) detects subclinical diastolic dysfunction in adults but is infrequently used in children. We evaluated LAS in MIS-C and the associations with systemic inflammation and cardiac injury. Methods: In this retrospective cohort study, conventional parameters and LAS (reservoir [LAS-r], conduit [LAS-cd], and contractile [LAS-ct]) obtained from admission echocardiograms of MIS-C patients were compared to healthy controls and between MIS-C patients with and without cardiac injury (BNP >500 pg/ml or troponin-I >0.04 ng/ml). Correlation and logistic regression analyses were performed to assess LAS associations with admission inflammatory and cardiac biomarkers. Reliability testing was performed. Results: Median LAS components were reduced in MIS-C patients (n=118) compared to controls (n=20) (LAS-r: 31.8 vs. 43.1%, p<0.001; LAS-cd: -28.8 vs. -34.5%, p=0.006; LAS-ct: -5.2 vs. -9.3%, p<0.001) and reduced in MIS-C patients with cardiac injury (n=59) compared to no injury (n=59) (LAS-r: 29.6 vs. 35.8%, p=0.001; LAS-cd: -26.5 vs. -30.4%, p=0.036; LAS-ct: -4.6 vs. -9.3%, p=0.008). An LAS-ct peak was absent in 65 (55%) MIS-C patients but present in all controls (p<0.001). Procalcitonin had strong correlation with averaged E/e' (r=0.55, p=0.001); ESR had moderate correlation with LAS-ct (r=-0.41, p=0.007); BNP had moderate correlation with LAS-r (r=-0.39, p<0.001) and LAS-ct (r=0.31, p=0.023), and troponin-I had only weak correlations. No strain indices were independently associated with cardiac injury on regression analysis. Intra-rater reliability was good for all LAS components; and inter-rater reliability was good to excellent for LAS-r, and fair for LAS-cd and LAS-ct. Conclusions: LAS analysis, particularly the absence of a LAS-ct peak, was reproducible and may be superior to conventional echocardiographic parameters for detecting diastolic dysfunction in MIS-C. No strain parameters on admission were independently associated with cardiac injury.

Identifying Patients With Kawasaki Disease Safe for Early Discharge: Development of a Risk Prediction Model at a US Children's Hospital.

OBJECTIVES: To develop a model to predict risk of intravenous immunoglobulin (IVIg) nonresponse in patients with Kawasaki disease (KD) to assist in early discharge decision-making. METHODS: Retrospective cohort study of 430 patients 0 to 18 years old discharged from a US children's hospital January 1, 2010, through July 31, 2017 with a diagnosis of KD. IVIg nonresponse was defined as at least 1 of the following: temperature ≥38.0°C between 36 hours and 7 days after initial IVIg dose, receipt of a second IVIg dose after a temperature ≥38.0°C at least 20 hours after initial IVIg dose, or readmission within 7 days with administration of a second IVIg dose. Backward stepwise logistic regression was used to select a predictive model. RESULTS: IVIg nonresponse occurred in 19% (81 of 430) of patients. We identified a multivariate model (which included white blood cell count, hemoglobin level, platelet count, aspartate aminotransferase level, sodium level, albumin level, temperature within 6 hours of first IVIg dose, and incomplete KD) with good predictive ability (optimism-adjusted concordance index: 0.700) for IVIg nonresponse. Stratifying into 2 groups by a predictive probability cutoff of 0.10, we identified 26% of patients at low risk for IVIg nonresponse, with a sensitivity and specificity of 90% and 30%, respectively, and a negative predictive value of 93%. CONCLUSIONS: We developed a model with good predictive value for identifying risk of IVIg nonresponse in patients with KD at a US children's hospital. Patients at lower risk may be considered for early discharge by using shared decision-making. Our model may be used to inform implementation of electronic health record tools and future risk prediction research.

The First Cardiac Operation Using Cardiopulmonary Bypass.

The first open heart operation ever performed was by Clarence Dennis on April 5, 1951, at the University of Minnesota. For several years he had worked developing a heart-lung machine. The operation was on a young girl considered to have an ostium secundum atrial septal defect, but at the procedure a large ostium primum was identified instead. It could not be repaired, and the child died. The anatomic details of this heart have not been previously presented. For historical purposes, the clinical history, intraoperative course, and pathologic details will be discussed and shown.

Controlled evaluation of the effects of hyperbaric oxygen therapy on the behavior of 16 children with autism spectrum disorders.

Hyperbaric oxygen therapy (HBOT) has been used to treat individuals with autism. However, few studies of its effectiveness have been completed. The current study examined the effects of 40 HBOT sessions at 24% oxygen at 1.3 ATA on 11 topographies of directly observed behavior. Five replications of multiple baselines were completed across a total of 16 participants with autism spectrum disorders. No consistent effects were observed across any group or within any individual participant, demonstrating that HBOT was not an effective treatment for the participants in this study. This study represents the first relatively large-scale controlled study evaluating the effects of HBOT at the level of the individual participant, on a wide array of behaviors.

Low serum myeloperoxidase in autistic children with gastrointestinal disease.

AIM: To assess serum myeloperoxidase (MPO) levels in autistic children with severe gastrointestinal (GI) disease and to test the hypothesis that there is an association between serum MPO concentration and inflammatory GI disease, including antineutrophil cytoplasmic antibodies (ANCA), previously seen in a subgroup of autistic children. SUBJECTS AND METHODS: Serum from 40 autistic children with chronic digestive disease (most with ileo-colonic lymphoid nodular hyperplasia (LNH) and inflammation of the colorectum, small bowel and/or stomach), and 48 controls (12 age-matched autistic children with no GI disease, 20 age-matched children without autism or GI disease, and 16 nonautistic individuals with no family history of autism) were tested using enzyme-linked immunosorbent assays designed to quantitate serum MPO levels. MPO serum concentration of autistic children with GI disease was compared to GI disease severity (including LNH and erythema) and presence of ANCA. RESULTS: We found that a significant number of autistic children with chronic digestive disease had low serum levels of MPO. However, there was no significant relationship between these levels and severity of GI disease, including the presence of ANCA. DISCUSSION: These results suggest a relationship between low MPO levels and GI disease seen in a subpopulation of autism spectrum disorders individuals. MPO concentration may therefore be a useful biomarker for GI disease in this group of autistic children.