Background: Exposure to supraphysiologic oxygen concentrations, or hyperoxia, during neonatal cardiopulmonary bypass (CPB) has been associated with worse outcomes in single‐center studies. We aimed to describe variation in oxygen exposure during CPB and determine if hyperoxia is associated with worse outcomes in a multicenter cohort of neonates undergoing cardiac surgery. Methods: We conducted a retrospective study of neonates who underwent surgery with CPB between January 2021 and December 2022 at 29 centers. Primary outcomes were operative mortality and major adverse cardiovascular events, which included cardiopulmonary resuscitation, extracorporeal support, stroke, and mortality. Logistic regression assessed the associations between median partial pressure of oxygen in arterial blood (PaO ₂ ) during CPB and outcomes in the entire cohort and subset of patients with single‐ventricle (SV) anatomy. Results: We analyzed 1175 neonates, including 357 with SV anatomy. Median PaO ₂ during CPB was 296 mm Hg (range: 54–800), with significant variation across centers ( P <0.001). There were 54 deaths (5%) and 203 major adverse cardiovascular events (17%). No patients with PaO ₂ 221 mm Hg demonstrated modest mortality prediction (96% sensitivity, 19% specificity). In multivariable analysis, before adjustment for center, PaO ₂ >221 mm Hg was significantly associated with mortality and major adverse cardiovascular events in all patients and patients with SV anatomy. After adjustment for center, associations between hyperoxia and mortality ( P =0.053) or major adverse cardiovascular events ( P =0.08) were not significant in the entire cohort but remained significant in patients with SV anatomy. Conclusions: We observed significant variation in oxygen exposure during neonatal CPB across centers and significant associations between hyperoxia and worse outcomes, particularly in neonates with SV anatomy.

Cystic fibrosis (CF) newborn screening (NBS) aims to improve outcomes through early diagnosis, yet disparities in time to diagnosis remain. This study examines CFTR allele frequencies and variant panel performance among a diverse CF population in Georgia to inform recommendations for updating the NBS algorithm and improving equity. This cross-sectional study includes 969 people with CF (PwCF) from Georgia’s accredited CF centers. CFTR variant frequencies were calculated according to race and ethnicity. Panel performance was evaluated for Georgia’s current Luminex-39 variant test and three expanded panels. Statistical analyses compared detection rates across panels and demographic groups. Georgia’s diverse CF population demonstrates a unique CFTR allelic variability compared to national data. Increasing panel size enhances case identification. A panel including 719 CF-causing variants from the CFTR2 database significantly improves case detection from 93% to 97% (p = 0.002), as well as two-variant detection from 69% to 86% (p < 0.001). Detection of minoritized PwCF also improves with increasing panel size. However, even using the 719-variant panel, detection of non-Hispanic Black PwCF remains significantly lower compared to non-Hispanic White PwCF (case detection: p = 0.003; two-variant detection: p < 0.001). In conclusion, the use of expanded CFTR panels for NBS in Georgia would enhance timely diagnosis and improve equity.

Early diagnosis by newborn screening (NBS) has contributed to improved outcomes in children with cystic fibrosis (CwCF). Georgia’s two-tiered algorithm consists of a fixed immunoreactive trypsinogen (IRT) cut-off followed by a 39-variant CFTR genetic panel. We conducted a retrospective review of CwCF born in Georgia from 2007 to 2022 to evaluate false negative NBS frequency. We characterized CwCF whose diagnosis was delayed beyond 28 days of age despite positive NBS. Six cases were detailed demonstrating the impact of missed and delayed diagnoses. We examined IRT trends from 2018 to 2022 and cut-off approaches. Missed case detection by expanded CFTR variant assays was assessed. Of 390 CwCF born in Georgia, 18 (4.6%) had false negative NBS—6 due to lack of CFTR variant detection and 12 due to low IRT values. Thirty children had delayed diagnosis, with the majority related to sweat testing. Minoritized children made up 19% of the population but 43% of missed and 44% of delayed diagnoses. Black and Hispanic infants had higher odds of missed or delayed diagnosis compared to non-Hispanic White infants (OR = 2.7, p = 0.027 and OR = 6.1, p < 0.001, respectively). Average IRT values varied across kits and were lower in warmer seasons. Expanded CFTR assays would reduce missed cases. Our results informed recommendations for improvement at multiple steps in the NBS process.

Objectives: Burnout is common among pediatric emergency medicine (PEM) physicians, but the relationship between creative problem-solving (CPS) preferences and burnout is not well understood. This study examined associations between CPS mindsets and burnout in PEM. Methods: This cross-sectional study included PEM (attending physicians and fellows) from 3 emergency departments. Measures: Maslach Burnout Inventory–Human Services Survey for Burnout; FourSight for CPS preferences (clarifying, ideating, developing, implementing). The primary outcome was the association between burnout and CPS preferences, and the secondary outcomes were comparisons of burnout prevalence and CPS preferences in PEM compared with published norms. Results: Participation was 100%, with 96.7% providing complete data. Participants: female 58%; median age 41 years; 51% White, 23% Asian, and 83% married. High clarifying scores were associated with greater emotional exhaustion (EE; adjusted β = 0.67; 95% CI, 0.15 to 1.19), with a stronger association among women (adjusted β = 0.76; 95% CI, 0.03 to 1.48). Ideating was associated with higher personal accomplishment (PA; β = 0.31; 95% CI, 0.08 to 0.55). In univariate analysis, female sex (β = 5.03; 95% CI, 0.17 to 9.89) and educational debt (β = 5.46; 95% CI, 0.47 to 10.4) were each associated with higher EE. Compared with health care norms, PEM demonstrated higher PA scores (mean = 5.00; 95% CI, 4.84 to 5.15 vs. 4.30). PEM were more likely to show an implementing mindset (55% vs. 21%, P < 0.001) and less likely to show a Developing mindset (10% vs. 23%, P = 0.007). Conclusions: A clarifying mindset was associated with higher EE, particularly among women, while Ideating was associated with greater PA. CPS self-awareness and targeted training may offer novel strategies to mitigate burnout among PEM.

Background: Pediatric pulmonary vein stenosis (PVS) is a progressive and recurrent condition associated with high morbidity and mortality. Long-term outcomes of individual pulmonary veins remain poorly described. Methods: All pediatric (<18 years) PVS cases diagnosed between September 2005 and January 2020 with a minimum follow-up of 5 years were included. Vein-specific outcomes were assessed using competing-risk survival analysis adjusted for patient death and vein atresia. Veins with recurrent disease maintaining patency for ≥3 years were analyzed longitudinally to characterize reintervention burden as a surrogate for disease trajectory. Results: A total of 244 affected veins in 107 patients were identified. The cumulative incidence of vein loss progressively increased during the first 3 years, followed by plateauing thereafter. Overall, 147 of 244 veins (60%) were lost within 3 years of diagnosis, including 75 veins lost secondary to patient death. Vein attrition was greatest during the first year following diagnosis. Ninety-seven veins remained patent for ≥3 years, including 73 with recurrent disease. Among these veins, the median reintervention rate decreased significantly from 2 (IQR: 1–4) during the first 3 years to 1 per year (IQR: 1–3) thereafter (p < 0.01) while maintaining continued patency. Conclusions: In pediatric PVS, the first 3 years after diagnosis represent a critical period of active disease with high rates of vein attrition. Veins maintaining patency beyond this period demonstrate a reduced reintervention rate, suggesting stabilization of disease progression. Aggressive surveillance and intervention during this therapeutic window may improve long-term vein and patient outcomes.