Faculty Bibliography

Myxomas are rare tumors in neonates and tend to have a different presentation compared to adults. We present an infant with a left atrial myxoma presenting with episodic tachycardia who underwent successful surgical excision. In addition, we performed a review of the literature, identifying 17 cases of neonatal myxomas. Unlike adults, neonatal myxomas are more common in males and occur more often on the right side of the heart. Constitutional symptoms such as fever or embolism are rare among neonates. Most patients have favorable outcomes following surgical excision, refuting earlier claims that neonatal myxomas are associated with poor outcomes.

IMPORTANCE/UNASSIGNED:There is limited evidence for therapeutic options for pediatric COVID-19 outside of multisystem inflammatory syndrome in children (MIS-C). OBJECTIVE/UNASSIGNED:To determine whether the use of steroids within 2 days of admission for non-MIS-C COVID-19 in children is associated with hospital length of stay (LOS). The secondary objective was to determine their association with intensive care unit (ICU) LOS, inflammation, and fever defervescence. DESIGN, SETTING, AND PARTICIPANTS/UNASSIGNED:This cohort study analyzed data retrospectively for children (<18 years) who required hospitalization for non-MIS-C COVID-19. Data from March 2020 through September 2021 were provided by 58 hospitals in 7 countries who participate in the Society of Critical Care Medicine Discovery Viral Infection and Respiratory Illness Universal Study (VIRUS) COVID-19 registry. EXPOSURE/UNASSIGNED:Administration of steroids within 2 days of admission. MAIN OUTCOMES AND MEASURES/UNASSIGNED:Length of stay in the hospital and ICU. Adjustment for confounders was done by mixed linear regression and propensity score matching. RESULTS/UNASSIGNED:A total of 1163 patients met inclusion criteria and had a median (IQR) age of 7 years (0.9-14.3). Almost half of all patients (601/1163, 51.7%) were male, 33.8% (392/1163) were non-Hispanic White, and 27.9% (324/1163) were Hispanic. Of the study population, 184 patients (15.8%) received steroids within 2 days of admission, and 979 (84.2%) did not receive steroids within the first 2 days. Among 1163 patients, 658 (56.5%) required respiratory support during hospitalization. Overall, patients in the steroids group were older and had greater severity of illness, and a larger proportion required respiratory and vasoactive support. On multivariable linear regression, after controlling for treatment with remdesivir within 2 days, country, race and ethnicity, obesity and comorbidity, number of abnormal inflammatory mediators, age, bacterial or viral coinfection, and disease severity according to ICU admission within first 2 days or World Health Organization ordinal scale of 4 or higher on admission, with a random intercept for the site, early steroid treatment was not significantly associated with hospital LOS (exponentiated coefficient, 0.94; 95% CI, 0.81-1.09; P = .42). Separate analyses for patients with an LOS of 2 days or longer (n = 729), those receiving respiratory support at admission (n = 286), and propensity score-matched patients also showed no significant association between steroids and LOS. Early steroid treatment was not associated with ICU LOS, fever defervescence by day 3, or normalization of inflammatory mediators. CONCLUSIONS AND RELEVANCE/UNASSIGNED:Steroid treatment within 2 days of hospital admission in a heterogeneous cohort of pediatric patients hospitalized for COVID-19 without MIS-C did not have a statistically significant association with hospital LOS.

BACKGROUND:Describe the incidence and associated outcomes of gastrointestinal (GI) manifestations of acute coronavirus disease 2019 (COVID-19) and multisystem inflammatory syndrome in hospitalized children (MIS-C). METHODS:Retrospective review of the Viral Infection and Respiratory Illness Universal Study registry, a prospective observational, multicenter international cohort study of hospitalized children with acute COVID-19 or MIS-C from March 2020 to November 2020. The primary outcome measure was critical COVID-19 illness. Multivariable models were performed to assess for associations of GI involvement with the primary composite outcome in the entire cohort and a subpopulation of patients with MIS-C. Secondary outcomes included prolonged hospital length of stay defined as being >75th percentile and mortality. RESULTS:Of the 789 patients, GI involvement was present in 500 (63.3%). Critical illness occurred in 392 (49.6%), and 18 (2.3%) died. Those with GI involvement were older (median age of 8 yr), and 18.2% had an underlying GI comorbidity. GI symptoms and liver derangements were more common among patients with MIS-C. In the adjusted multivariable models, acute COVID-19 was no associated with the primary or secondary outcomes. Similarly, despite the preponderance of GI involvement in patients with MIS-C, it was also not associated with the primary or secondary outcomes. CONCLUSIONS:GI involvement is common in hospitalized children with acute COVID-19 and MIS-C. GI involvement is not associated with critical illness, hospital length of stay or mortality in acute COVID-19 or MIS-C.

Importance/UNASSIGNED:Patients with language barriers have a higher risk of experiencing hospital safety events. This study hypothesized that language barriers would be associated with poorer perceptions of hospital safety climate relating to communication openness. Objective/UNASSIGNED:To examine disparities in reported hospital safety climate by language proficiency in a cohort of hospitalized children and their families. Design, Setting, and Participants/UNASSIGNED:This cohort study conducted from April 29, 2019, through March 1, 2020, included pediatric patients and parents or caregivers of hospitalized children at general and subspecialty units at 21 US hospitals. Randomly selected Arabic-, Chinese-, English-, and Spanish-speaking hospitalized patients and families were approached before hospital discharge and were included in the analysis if they provided both language proficiency and health literacy data. Participants self-rated language proficiency via surveys. Limited English proficiency was defined as an answer of anything other than "very well" to the question "how well do you speak English?" Main Outcomes and Measures/UNASSIGNED:Primary outcomes were top-box (top most; eg, strongly agree) 5-point Likert scale ratings for 3 Children's Hospital Safety Climate Questionnaire communication openness items: (1) freely speaking up if you see something that may negatively affect care (top-box response: strongly agree), (2) questioning decisions or actions of health care providers (top-box response: strongly agree), and (3) being afraid to ask questions when something does not seem right (top-box response: strongly disagree [reverse-coded item]). Covariates included health literacy and sociodemographic characteristics. Logistic regression was used with generalized estimating equations to control for clustering by site to model associations between openness items and language proficiency, adjusting for health literacy and sociodemographic characteristics. Results/UNASSIGNED:Of 813 patients, parents, and caregivers who were approached to participate in the study, 608 completed surveys (74.8% response rate). A total of 87.7% (533 of 608) of participants (434 [82.0%] female individuals) completed language proficiency and health literacy items and were included in the analyses; of these, 14.1% (75) had limited English proficiency. Participants with limited English proficiency had lower odds of freely speaking up if they see something that may negatively affect care (adjusted odds ratio [aOR], 0.26; 95% CI, 0.15-0.43), questioning decisions or actions of health care providers (aOR, 0.19; 95% CI, 0.09-0.41), and being unafraid to ask questions when something does not seem right (aOR, 0.44; 95% CI, 0.27-0.71). Individuals with limited health literacy (aOR, 0.66; 95% CI, 0.48-0.91) and a lower level of educational attainment (aOR, 0.59; 95% CI, 0.36-0.95) were also less likely to question decisions or actions. Conclusions and Relevance/UNASSIGNED:This cohort study found that limited English proficiency was associated with lower odds of speaking up, questioning decisions or actions of providers, and being unafraid to ask questions when something does not seem right. This disparity may contribute to higher hospital safety risk for patients with limited English proficiency. Dedicated efforts to improve communication with patients and families with limited English proficiency are necessary to improve hospital safety and reduce disparities.

Tricuspid atresia with an absent pulmonary valve is a rare congenital cardiac defect. Although extensive pathological reviews have been published in the past, there are only a handful of cases that have been successfully palliated to the stage of Fontan. We hereby describe the successful management of one such case and review the surgical strategies described in the literature.

BACKGROUND:It is unclear how acute coronavirus disease 2019 (COVID-19)-directed therapies are used in children with life-threatening COVID-19 in US hospitals. We described characteristics of children hospitalized in the intensive care unit or step-down unit (ICU/SDU) who received COVID-19-directed therapies and the specific therapies administered. METHODS:Between March 15, 2020 and December 27, 2020, children <18 years of age in the ICU/SDU with acute COVID-19 at 48 pediatric hospitals in the United States were identified. Demographics, laboratory values, and clinical course were compared in children who did and did not receive COVID-19-directed therapies. Trends in COVID-19-directed therapies over time were evaluated. RESULTS:Of 424 children in the ICU/SDU, 235 (55%) received COVID-19-directed therapies. Children who received COVID-19-directed therapies were older than those who did not receive COVID-19-directed therapies (13.3 [5.6-16.2] vs 9.8 [0.65-15.9] years), more had underlying medical conditions (188 [80%] vs 104 [55%]; difference = 25% [95% CI: 16% to 34%]), more received respiratory support (206 [88%] vs 71 [38%]; difference = 50% [95% CI: 34% to 56%]), and more died (8 [3.4%] vs 0). Of the 235 children receiving COVID-19-directed therapies, 172 (73%) received systemic steroids and 150 (64%) received remdesivir, with rising remdesivir use over the study period (14% in March/April to 57% November/December). CONCLUSION/CONCLUSIONS:Despite the lack of pediatric data evaluating treatments for COVID-19 in critically ill children, more than half of children requiring intensive or high acuity care received COVID-19-directed therapies.

OBJECTIVES/OBJECTIVE:Survivors of the PICU face long-term morbidities across health domains. In this study, we detail active PICU follow-up programs (PFUPs) and identify perceptions and barriers about development and maintenance of PFUPs. METHODS:A web link to an adaptive survey was distributed through organizational listservs. Descriptive statistics characterized the sample and details of existing PFUPs. Likert responses regarding benefits and barriers were summarized. RESULTS:One hundred eleven respondents represented 60 institutions located in the United States (n = 55), Canada (n = 3), Australia (n = 1), and the United Kingdom (n = 1). Details for 17 active programs were provided. Five programs included broad PICU populations, while the majority were neurocritical care (53%) focused. Despite strong agreement on the need to assess and treat morbidity across multiple health domains, 29% were physician only programs, and considerable variation existed in services provided by programs across settings. More than 80% of all respondents agreed PFUPs provide direct benefits and are essential to advancing knowledge on long-term PICU outcomes. Respondents identified "lack of support" as the most important barrier, particularly funding for providers and staff, and lack of clinical space, though successful programs overcome this challenge using a variety of funding resources. CONCLUSIONS:Few systematic multidisciplinary PFUPs exist despite strong agreement about importance of this care and direct benefit to patients and families. We recommend stakeholders use our description of successful programs as a framework to develop multidisciplinary models to elevate continuity across inpatient and outpatient settings, improve patient care, and foster collaboration to advance knowledge.

Purpose or Case Report: To assess variability in diffusion tensor imaging of the physis and metaphysis (DTI-P/M) of the distal femur between scanners, observers, tractography software, and resolution. Methods & Materials: We prospectively obtained DTI-P/M (20 directions, b values of 0 and 600 sec/mm2) in 11 healthy subjects (5 males, 6 females) ages 10-15 (mean 12.54) and quantified tract volume, tract length, and number of tracts in the distal femoral physis. Each subject was imaged in both GE and Siemens 3T units, and the sequence was performed twice in each session with voxel sizes of 2x2x3mm or 1x1x2mm and gap sizes of 0mm and 0.6 mm (22 studies). We compared interobserver variability (with two observers) using hand-drawn regions of interest in the distal femur physis between two tractography software, Trackvis (FACT algorithm) and DSI Studio (Euler algorithm), both with a 40o angular threshold. For DSI Studio, we set the tracking threshold to 0.1, length range to 0-200mm, and seed termination to 1000000. We compared tract number, length, and volume and fractional anisotropy, between the 22 studies using Spearman's correlation and Bland-Altman (BA) plots.
Result(s): Correlation between Siemens and GE was significant between Siemens with 2x2x3mm voxel size and 0.6mm gap with GE 1x1x2mm voxel size and 0mm gap (rho = 0.93, p<10^- 15). Bland-Altman plots normalizing for voxel size between Siemens and GE showed no bias in inter-scanner variability (bias -5.76, BA limits of agreement (LOA) -24.31 and 12.78), nor was there significant intrascanner variability between consecutive runs of either Siemens (bias 0.565, BA LOA -10.31 and 9.18) or GE (bias 0.309, BA LOA -2.08 and 2.69). The 95% confidence intervals for the inter-scan (Mean=1.109, 95%-CI [- 0.84 3.06]) and intra-scan (Mean=0.3, 95%-CI [-0.31 0.91]) both included zero confirming no significant bias between scanners (p<0.05). Trackvis, the current standard for DTI-P/M, had high interobserver agreement (rho = 0.95) whereas DSI Studio showed poor interobserver agreement (rho = 0.29).
Conclusion(s): DTI-P/M shows high GE and Siemens inter- and intra-scanner reproducibility and low variability. Trackvis has low interobserver variability for tractography generation

BACKGROUND:Previous studies of severe acute respiratory syndrome coronavirus 2 infection in infants have incompletely characterized factors associated with severe illness or focused on infants born to mothers with coronavirus disease 2019 (COVID-19). Here we highlight demographics, clinical characteristics and laboratory values that differ between infants with and without severe acute COVID-19. METHODS:Active surveillance was performed by the Overcoming COVID-19 network to identify children and adolescents with severe acute respiratory syndrome coronavirus 2-related illness hospitalized at 62 sites in 31 states from March 15 to December 27, 2020. We analyzed patients aged >7 days to <1 year hospitalized with symptomatic acute COVID-19. RESULTS:We report 232 infants aged >7 days to <1 year hospitalized with acute symptomatic COVID-19 from 37 US hospitals in our cohort from March 15 to December 27, 2020. Among 630 cases of severe COVID-19 in patients aged >7 days to <18 years, 128 (20.3%) were infants. In infants with severe illness from the entire study period, the median age was 2 months, 66% were from racial and ethnic minority groups, 66% were previously healthy, 73% had respiratory complications, 13% received mechanical ventilation and <1% died. CONCLUSIONS:Infants accounted for over a fifth of children aged <18 years hospitalized for severe acute COVID-19, commonly manifesting with respiratory symptoms and complications. Although most infants hospitalized with COVID-19 did not suffer significant complications, longer term outcomes remain unclear. Notably, 75% of infants with severe disease were <6 months of age in this cohort study period, which predated maternal COVID-19 vaccination, underscoring the importance of maternal vaccination for COVID-19 in protecting the mother and infant.