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.

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.

We present the case of a 4-month-old, former 23-week premature baby who underwent patent ductus arteriosus device closure in the cardiac catheterisation lab with an Amplatzer Piccoloâ„¢ device at 12 weeks of life. This was complicated by late migration of the device into the aorta resulting in severe obstruction and requiring surgical intervention.

Intrapericardial teratomas are rare, predominantly benign tumors that warrant surgical resection in the neonatal period because of their potential detrimental effects on the cardiorespiratory system. Surgical resection can be a challenge when the tumor encases and obscures a coronary artery. Adherence to certain operative principles is necessary to achieve successful outcomes.

We report a case of a 17-year-old healthy male presenting with multisystem hyperinflammatory shock temporally associated with COVID-19. Cardiac involvement was suspected based on evidence of significant cardiac injury (elevated cardiac biomarkers, electrocardiographic and echocardiographic abnormalities). Cardiac magnetic resonance imaging was performed demonstrating global biventricular systolic dysfunction, as well as a small area of T2 hyperintensity and mid-wall late gadolinium enhancement. This case discusses the varied cardiac involvement in pediatric patients with COVID-19 infection and highlights that cardiac injury is not just limited to hyperinflammatory syndrome related global dysfunction but a more focal myocarditis can also be seen.

The Center for Disease Control recommends prompt removal of nonessential central venous catheters (CVCs) to reduce the risk for central line-associated bloodstream infections. Safety checklists have been trialed to reduce nonessential CVC days, but pediatric studies are lacking. Our specific aim was to detect >10% reduction in mean CVC duration after implementation of a safety checklist addressing CVCs in our unit.

PURPOSE/OBJECTIVE:Drug reaction with eosinophilia and systemic symptoms (DRESS) is associated with antiepileptic drug use and is a rare but life-threatening side effect. We present a case of phenobarbital-induced DRESS in a patient who subsequently required phenobarbital and was successfully desensitized. SUMMARY/CONCLUSIONS:A 5-year-old male presented with medically refractory status epilepticus (SE). He had been trialed on several antiepileptic medications without achieving burst suppression. Burst suppression was achieved with a pentobarbital infusion, and thus, phenobarbital was initiated as the pentobarbital was weaned. After five days of phenobarbital, the patient developed signs and symptoms concerning for DRESS; a punch biopsy confirmed the drug reaction. Two months later, he again developed SE unresponsive to antiepileptic infusions. Burst suppression was achieved with pentobarbital, and it was decided to transition the patient to phenobarbital. Due to concerns of phenobarbital-induced DRESS, the patient underwent a phenobarbital desensitization consisting of 6 doses sequentially administered in 10-fold increasing concentrations before achieving therapeutic dosing. Three days later, the patient achieved therapeutic phenobarbital levels, was weaned off of pentobarbital, and remained seizure-free without recurrence of DRESS. CONCLUSIONS:Graded desensitization may be an option to minimize recurrence of DRESS in patients where avoidance of the offending agent is not possible.