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OBJECTIVE: To test the hypothesis that neonates with symptomatic tetralogy of Fallot (TOF) and absent ductus arteriosus (ADA) have worse clinical outcomes compared with those with a ductus arteriosus (DA), and that this difference is driven by those born with ADA and with critically deficient pulmonary blood flow (CDPBF). STUDY DESIGN: We performed a retrospective, multicenter cohort study of neonates who underwent intervention for symptomatic TOF comparing death and reintervention between subjects with and without a DA identified on fetal echocardiogram or on echocardiogram performed in the first postnatal day. Exclusion criteria were as follows: inability to define DA status, collaterals supplying pulmonary blood flow, atrioventricular septal defect, and absent pulmonary valve. We defined CDPBF as undergoing a procedure to augment pulmonary blood flow on the date of birth or extracorporeal membrane oxygenation prior to such a procedure. RESULTS: The study cohort included 519 patients, among whom 11% had ADA. Patients with ADA were more likely to have a genetic syndrome and had smaller branch pulmonary artery size. In analyses adjusting for center, interventional treatment strategy, genetic syndrome, and minimum branch pulmonary artery size, ADA was associated with higher mortality risk (adjusted hazard ratio of 2.37 (95% CI: 1.07,5.27; P = .034). Seven patients had CDPBF (1.3% of the entire cohort and 12% of patients with ADA). CONCLUSIONS: A minority of symptomatic TOF neonates have ADA, which is associated with higher adjusted mortality risk compared with those with a DA. CDPBF appears to be a rare but important entity in this population.

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BACKGROUND: The delivery of adult primary care (APC) shifted from predominately in-person to modes of virtual care during the COVID-19 pandemic. It is unclear how these shifts impacted the likelihood of APC use during the pandemic, or how patient characteristics may be associated with the use of virtual care. METHODS: A retrospective cohort study using person-month level datasets from 3 geographically disparate integrated health care systems was conducted for the observation period of January 1, 2020, through June 30, 2021. We estimated a 2-stage model, first adjusting for patient-level sociodemographic, clinical, and cost-sharing factors, using generalized estimating equations with a logit distribution, along with a second-stage multinomial generalized estimating equations model that included an inverse propensity score treatment weight to adjust for the likelihood of APC use. Factors associated with APC use and virtual care use were separately assessed for the 3 sites. RESULTS: Included in the first-stage models were datasets with total person-months of 7,055,549, 11,014,430, and 4,176,934, respectively. Older age, female sex, greater comorbidity, and Black race and Hispanic ethnicity were associated with higher likelihood of any APC use in any month; measures of greater patient cost-sharing were associated with a lower likelihood. Conditional on APC use, older age, and adults identifying as Black, Asian, or Hispanic were less likely to use virtual care. CONCLUSIONS: As the transition in health care continues to evolve, our findings suggest that to ensure vulnerable patient groups receive high quality health care, outreach interventions to reduce barriers to virtual care use may be warranted.

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OBJECTIVE: To evaluate early growth following primary or staged repair of neonatal symptomatic tetralogy of Fallot (sTOF). STUDY DESIGN: We performed a retrospective, multicenter cohort study of consecutive infants with sTOF who underwent initial intervention at age ≤30 days, from 2005 to 2017. Management strategies were either primary repair or staged repair (ie, initial palliation followed by complete repair). The primary outcome was change in weight-for-age z-score (ΔWAZ) from the initial intervention to age 6 ± 2 months. Secondary outcomes included method and mode of feeding, feeding-related medications, and feeding-related readmissions. Propensity score adjustment was used to account for baseline differences between groups. A secondary analysis was performed comparing patients stratified by the presence of adequate growth (6-month ΔWAZ > -0.5) or inadequate growth (6-month ΔWAZ ≤ -0.5), independent of treatment strategy. RESULTS: The study cohort included 143 primary repair subjects and 240 staged repair subjects. Prematurity was more common in the staged repair group. After adjustment, median ΔWAZ did not differ between treatment groups over the first 6 months of life (primary: -0.43 [IQR, -1.17 to 0.50]; staged: -0.31 [IQR, -1.31 to 0.71]; P = .55). For the entire cohort, ΔWAZ was negative (-0.36; IQR, -1.21 to 0.63). There were no between-group differences in the secondary outcomes. Secondary analysis revealed that the subjects with adequate growth were more likely to be orally fed at initial hospital discharge (P = .04). CONCLUSIONS: In neonates with sTOF, growth trajectory over the first 6 months of life was substandard, irrespective of treatment strategy. Those patients with adequate growth were more likely to be discharged from the index procedure on oral feeds.

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OBJECTIVE: To evaluate long-term transplant-free survival and causes of death in the trisomy 21 (T21) population after surgery for congenital heart disease (CHD) in comparison with patients who are euploidic. STUDY DESIGN: This is a retrospective cohort study from the Pediatric Cardiac Care Consortium, enriched with prospectively collected data from the National Death Index and the Organ Procurement and Transplantation Network for patients with sufficient direct identifiers. Kaplan-Meier survival plots were generated and multivariable Cox proportional hazards models were used to examine risk factors for mortality between patients with T21 and 1:1 matched patients with comparable CHD who are euploidic. RESULTS: A long-term survival analysis was completed for 3376 patients with T21 (75 155 person-years) who met inclusion criteria. The 30-year survival rate for patients with T21 ranged from 92.1% for ventricular septal defect to 65.3% for complex common atrioventricular canal. Of these, 2185 patients with T21 were successfully matched with a patient who was euploidic. After a median follow-up of 22.86 years (IQR, 19.45-27.14 years), 213 deaths occurred in the T21 group (9.7%) compared with 123 (5.6%) in the euploidic comparators. After adjustment for age, sex, era, CHD complexity, and initial palliation, the hazard ratio of CHD-related mortality was 1.34 times higher in patients with T21 (95% CI, 0.92-1.97; P = .127). CONCLUSIONS: CHD-related mortality for patients with T21 after cardiac surgical intervention is comparable with euploidic comparators. Children with T21 require lifelong surveillance for co-occurring conditions associated with their chromosomal abnormality.

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OBJECTIVE: To assess characteristics and outcomes of young children receiving intensive multidisciplinary intervention for chronic food refusal and feeding tube dependence. STUDY DESIGN: We conducted a retrospective study of consecutive patients (birth to age 21 years) admitted to an intensive multidisciplinary intervention program over a 5-year period (June 2014-June 2019). Inclusion criteria required dependence on enteral feeding, inadequate oral intake, and medical stability to permit tube weaning. Treatment combined behavioral intervention and parent training with nutrition therapy, oral-motor therapy, and medical oversight. Data extraction followed a systematic protocol; outcomes included anthropometric measures, changes in oral intake, and percentage of patients fully weaned from tube feeding. RESULTS: Of 229 patients admitted during the 5-year period, 83 met the entry criteria; 81 completed intervention (98%) and provided outcome data (46 males, 35 females; age range, 10-230 months). All patients had complex medical, behavioral, and/or developmental histories with longstanding feeding problems (median duration, 33 months). At discharge, oral intake improved by 70.5%, and 27 patients (33%) completely weaned from tube feeding. Weight gain (mean, 0.39 ± 1 kg) was observed. Treatment gains continued following discharge, with 58 patients (72%) weaned from tube feeding at follow-up. CONCLUSIONS: Our findings support the effectiveness of our intensive multidisciplinary intervention model in promoting oral intake and reducing dependence on tube feeding in young children with chronic food refusal. Further research on the generalizability of this intensive multidisciplinary intervention approach to other specialized treatment settings and/or feeding/eating disorder subtypes is warranted.

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OBJECTIVE: To assess the feasibility and initial efficacy of a structured parent training program for children with autism spectrum disorder and moderate food selectivity. STUDY DESIGN: This 16-week randomized trial compared the Managing Eating Aversions and Limited variety (MEAL) Plan with parent education. MEAL Plan (10 core and 3 booster sessions) provided parents with nutrition education and strategies to structure meals and expand the child's diet. Parent education (10 sessions) provided information about autism without guidance on nutrition, meal structure, or diet. In addition to feasibility outcomes, primary efficacy outcomes included the Clinical Global Impression - Improvement scale and the Brief Autism Mealtime Behaviors Inventory. Grams consumed during a meal observation served as a secondary outcome. RESULTS: There were 38 eligible children (19 per group, 32 males). For MEAL Plan, attrition was <10% and attendance >80%. Therapists achieved >90% fidelity. At week 16, positive response rates on the Clinical Global Impression - Improvement scale were 47.4% for the MEAL Plan and 5.3% for parent education (P < .05). The adjusted mean difference (SE) on Brief Autism Mealtime Behaviors Inventory at week 16 was 7.04 (2.71) points (P = .01) in favor of MEAL Plan. For grams consumed, the adjusted standard mean difference (SE) was 30.76 (6.75), also in favor of MEAL Plan (P = .001). CONCLUSIONS: The MEAL Plan seems to be feasible, and preliminary efficacy results are encouraging. If further study replicates these results, the MEAL Plan could expand treatment options for children with autism spectrum disorder and moderate food selectivity. TRIAL REGISTRATION: Clinicaltrials.gov: NCT02712281.

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To assess MUC5AC as a biomarker for respiratory syncytial virus (RSV) disease severity, we tested nasal aspirates from RSV+ children with mild, moderate, and severe disease. Levels were significantly higher in those in the severe and moderate groups compared to mild group, indicating MUC5AC may be a useful biomarker for RSV disease severity.

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OBJECTIVE: To assess models of care and conduct a meta-analysis of program outcomes for children receiving intensive, multidisciplinary intervention for pediatric feeding disorders. STUDY DESIGN: We searched Medline, PsycINFO, and PubMed databases (2000-2015) in peer-reviewed journals for studies that examined the treatment of children with chronic food refusal receiving intervention at day treatment or inpatient hospital programs. Inclusion criteria required the presentation of quantitative data on food consumption, feeding behavior, and/or growth status before and after intervention. Effect size estimates were calculated based on a meta-analysis of proportions. RESULTS: The systematic search yielded 11 studies involving 593 patients. Nine articles presented outcomes based on retrospective (nonrandomized) chart reviews; 2 studies involved randomized controlled trials. All samples involved children with complex medical and/or developmental histories who displayed persistent feeding concerns requiring formula supplementation. Behavioral intervention and tube weaning represented the most common treatment approaches. Core disciplines overseeing care included psychology, nutrition, medicine, and speech-language pathology/occupational therapy. The overall effect size for percentage of patients successfully weaned from tube feeding was 71% (95% CI 54%-83%). Treatment gains endured following discharge, with 80% of patients (95% CI 66%-89%) weaned from tube feeding at last follow-up. Treatment also was associated with increased oral intake, improved mealtime behaviors, and reduced parenting stress. CONCLUSIONS: Results indicate intensive, multidisciplinary treatment holds benefits for children with severe feeding difficulties. Future research must address key methodological limitations to the extant literature, including improved measurement, more comprehensive case definitions, and standardization/examination of treatment approach.

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OBJECTIVES: To identify the clinical presentation of children and adolescents affected by 1 of 4 cardiac conditions predisposing to sudden cardiac arrest: hypertrophic cardiomyopathy, long QT syndrome (LQTS), catecholaminergic polymorphic ventricular tachycardia (CPVT), and anomalous origin of the left coronary artery from the right sinus of Valsalva (ALCA-R). STUDY DESIGN: This was a retrospective review of newly diagnosed pediatric patients with hypertrophic cardiomyopathy, LQTS, CPVT, and ALCA-R referred for cardiac evaluation at 6 US centers from 2008 to 2014. RESULTS: A total of 450 patients (257 male/193 female; median age 10.1 years [3.6-13.8 years, 25th-75th percentiles]) were enrolled. Patient age was ≤13 years for 70.4% of the cohort (n = 317). Sudden cardiac arrest was the initial presentation in 7%; others were referred on the basis of abnormal or suspicious family history, personal symptoms, or physical findings. Patients with LQTS and hypertrophic cardiomyopathy were referred most commonly because of family history concerns. ALCA-R was most likely to have abnormal signs or symptoms (eg, exercise chest pain, syncope, or sudden cardiac arrest). Patients with CPVT had a high incidence of syncope and the greatest incidence of sudden cardiac arrest (45%); 77% exhibited exercise syncope or sudden cardiac arrest. This study demonstrated that suspicious or known family history plays a role in identification of many patients ultimately affected by 1 of the 3 genetic disorders (hypertrophic cardiomyopathy, LQTS, CPVT). CONCLUSION: Important patient and family history and physical examination findings may allow medical providers to identify many pediatric patients affected by 4 cardiac disorders predisposing to sudden cardiac arrest.

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