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Background: We aimed to study the clinical characteristics, myocardial injury, and longitudinal outcomes of COVID-19 vaccine-associated myocarditis (C-VAM). Methods: In this longitudinal retrospective observational cohort multicenter study across 38 hospitals in the United States, 333 patients with C-VAM were compared with 100 patients with multisystem inflammatory syndrome in children (MIS-C). We included patients ≤30 years of age with a clinical diagnosis of acute myocarditis after COVID-19 vaccination based on clinical presentation, abnormal biomarkers and/or cardiovascular imaging findings. Demographics, past medical history, hospital course, biochemistry results, cardiovascular imaging, and follow-up information from April 2021 to November 2022 were collected. The primary outcome was presence of myocardial injury as evidenced by late gadolinium enhancement (LGE) on cardiac magnetic resonance (CMR) imaging. Findings: Patients with C-VAM were predominantly white (67%) adolescent males (91%, 15.7 ± 2.8 years). Their initial clinical course was more likely to be mild (80% vs. 23%, p < 0.001) and cardiac dysfunction was less common (17% vs. 68%, p < 0.0001), compared to MIS-C. In contrast, LGE on CMR was more prevalent in C-VAM (82% vs. 16%, p < 0.001). The probability of LGE was higher in males (OR 3.28 [95% CI: 0.99, 10.6, p = 0.052]), in older patients (>15 years, OR 2.74 [95% CI: 1.28, 5.83, p = 0.009]) and when C-VAM occurred after the first or second dose as compared to the third dose of mRNA vaccine. Mid-term clinical outcomes of C-VAM at a median follow-up of 178 days (IQR 114-285 days) were reassuring. No cardiac deaths or heart transplantations were reported until the time of submission of this report. LGE persisted in 60% of the patients at follow up. Interpretation: Myocardial injury at initial presentation and its persistence at follow up, despite a mild initial course and favorable mid-term clinical outcome, warrants continued clinical surveillance and long-term studies in affected patients with C-VAM. Funding: The U.S. Food and Drug Administration.

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OBJECTIVES: To characterize the patient population with severe bronchopulmonary dysplasia (BPD) requiring tracheostomy in a large tertiary level 4 neonatal intensive care unit (NICU) and to identify potential targets for improvement in the delivery of high-quality healthcare. METHODS: An IRB-exempt but IRB-registered retrospective review of medical records. Study inclusion criteria: patients treated for severe BPD with tracheostomy under 2 years of age in our tertiary referral center NICU. Control group criteria: 4-year aggregate NICU patient demographics. Basic demographics, maternal history, clinical data points, and outcomes variables were collected. RESULTS: There was a statistically significant difference between the two groups in only one variable: racial identification (p-value = 0.036). All data points were then analyzed against racial identification, and statistically significant differences appeared in 4 categories: 1) illicit drug use, 2) birth head circumference and length, 3) days to readmission, and 4) child opportunity index scores. There was not a statistically significant difference in any other maternal characteristics or medical comorbidities, NICU length of stay, age at tracheostomy, or decannulation status. CONCLUSION: The incidence of our tracheostomy in infants with severe BPD was significantly higher (p = 0.036) in the subjects whose families identified as racially African American or Black, a marked contrast to our general NICU population and our overall tracheostomy population. The timing of the first readmission to the hospital was shorter for Caucasian or White infants compared to African American or Black infants. COI demonstrated statistically significantly poorer resources for African American or Black infants compared to White infants with tracheostomy. All other perinatal and outcome measurements did not differ significantly between the two racial groups. This suggests that this racial disparity is present and needs further investigation to better assess its impact on risk and outcomes as we develop pathways for high-quality healthcare delivery.

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Bronchopulmonary Dysplasia (BPD) is a pulmonary disease affecting newborns, commonly those with prematurity or low birth weight. Its pathogenesis involves underdevelopment of lung tissue with subsequent limitations in ventilation and oxygenation, resulting in impaired postnatal alveolarization. Despite advances in care with improved survival, BPD remains a prevalent comorbidity of prematurity. In severe cases, management may involve mechanical ventilation via tracheostomy. BPD's demand for multidisciplinary care compounds the challenges in management of this condition. Here, we review existing literature: the history of disease, criteria for diagnosis, pathogenesis, and modes of treatment with a focus on the severe subtype: that which is associated with pulmonary hypertension (PAH) for which tracheostomy is often required to facilitate long-term mechanical ventilation. We review the current recommendations for tracheostomy and decannulation.

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INTRODUCTION: Although medication toxicity is uncommon in neonates, there are several medications used in this population that pose a risk. Phenytoin has an increased risk of toxicity given its narrow therapeutic window and variations in drug elimination. CASE REPORT: We describe the case of a 3-day-old male infant who developed cardiovascular collapse secondary to severe phenytoin toxicity (max phenytoin level 86 µg/mL) and was placed on extracorporeal membrane oxygenation support (ECMO). Several ancillary treatments were utilized in an attempt to decrease serum phenytoin concentrations and limit toxicity including albumin boluses, phenobarbital administration, intravenous lipid infusion, and folic acid supplementation. DISCUSSION: Although uncommon, drug toxicity should be considered in patients with acute changes who are exposed to medications with potential toxicity. With elevated levels of phenytoin, the half-life can be prolonged resulting in longer exposure to elevated levels of the drug as seen in our patient. This case report highlights the importance of ECMO utilization for cardiac support in neonates with medication toxicity and other potential ancillary treatments to decrease serum phenytoin concentrations.

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Pompe disease is a rare genetic disorder resulting from a deficiency of the acid α-glucosidase enzyme. Although arrhythmias occur in these patients undergoing general anesthesia, they have not received sufficient emphasis in pediatric cardiology. We report a case of an infant with Pompe disease who experienced ventricular fibrillation during induction of anesthesia.

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Long QT syndrome is a rare disorder that can manifest as syncope, Torsades de Pointes, or sudden cardiac death. We report a newborn with asymptomatic bradycardia, 2:1 atrioventricular block, long QT syndrome, and episodes of Torsades de Pointes. The patient was managed with mexiletine and propranolol and continued to have episodes of Torsades de Pointes, so she underwent epicardial pacemaker implantation. No further episodes of Torsades de Pointes were noted prior to discharge.

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BACKGROUND: The presumptive prenatal diagnosis of tuberous sclerosis (TSC) previously depended upon fetal imaging. Cloning of the two TSC genes (TSC1 and TSC2) now enables precise molecular diagnosis by gene sequencing. We used this approach for the prenatal diagnosis of a fetus showing multiple intracardiac tumors. METHODS: DNA extracted from cultivated amniotic fluid cells underwent sequencing of all coding regions and exon-intron boundaries of the TSC1 and TSC2 genes. RESULTS: A mutation (R611Q) was found in exon 16 of the TSC2 gene. Thus far, neither clinically unaffected parents has provided blood samples for mutation analysis. CONCLUSION: For the first time, mutation analysis of a TSC gene enabled a precise prenatal diagnosis.

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