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Objective: To assess the association between primary and staged repair of neonatal symptomatic tetralogy of Fallot (sTOF) and neurodevelopmental outcomes in preschool through school-age children., Study Design: Multicenter cohort (n = 9 sites) study of patients with sTOF who underwent neonatal intervention between 2005 and 2017. The neurodevelopmental outcomes measures included caregivers' ratings of executive function with the Behavior Rating Inventory of Executive Function, and psychosocial functioning with the Behavior Assessment System for Children - third Edition (BASC-3). Results were compared with normative data and by treatment strategy (primary repair vs staged repair). A parent survey assessed history of disabilities and access to services related to neurodevelopment., Results: Although the majority of patients (median age 8.3 years, IQR 5.7-11.2) had median Behavior Rating Inventory of Executive Function and BASC-3 scores within the normal range, a proportion had clinically elevated (abnormal) scores, especially in the school-age patient subgroup (Behavior Rating Inventory of Executive Function 24%-30% and BASC 20%-37%). There were no statistically significant differences based on treatment strategy for either the Behavior Rating Inventory of Executive Function or BASC-3. However, lower birth weight, genetic syndrome, and medical complexity were significantly associated with worse executive function, and lower maternal education was associated in school-age children with lower executive and psychosocial functioning. Ongoing disabilities were relatively common (learning disability 35%, speech delay 33%, developmental delay 31%), although up to 50% of children were not receiving educational or developmental services., Conclusions: Elevated executive and psychosocial concerns are present in the patient population with sTOF. Although initial treatment strategy appears unrelated to neurodevelopmental outcomes, lower birth weight, genetic syndrome, and medical complexity and lower maternal education are risk factors. Early recognition of neurodevelopmental concerns can facilitate access to appropriate neurodevelopmental services in this high-risk group., Competing Interests: Declaration of Competing Interest Dr Jeffrey Zampi is a consultant for Medtronic, Inc and WL Gore and Associates. He also serves on the Data Safety Monitoring Board for Encore Medical. Dr Athar Qureshi is a consultant for Medtronic, Inc, B Braun, and WL Gore and Associates. Dr Bryan Goldstein is a consultant for Medtronic, Inc, WL Gore and Associates, PECA labs, and Mezzion Pharma. This study was funded in part by the generous support from the member institutions of the CCRC as well as the Kennedy Hammill Pediatric Cardiac Research Fund, The Liam Sexton Foundation, A Heart Like Ava, and a research grant from Project Heart. The study sponsors did not have any role in the study design; the collection, analysis, and interpretation of data; the writing of the report; or the decision to submit the manuscript for publication., (Copyright © 2024 Elsevier Inc. All rights reserved.)

To evaluate the association between initial management strategy of neonatal symptomatic Tetralogy of Fallot (sTOF) and later health-related quality of life (HRQOL) outcomes. We performed a multicenter, cross-sectional evaluation of a previously assembled cohort of infants with sTOF who underwent initial intervention at ≤ 30 days of age, between 2005 and 2017. Eligible patients' parents/guardians completed an age-appropriate Pediatric Quality of Life Inventory, a Pediatric Quality of Life Inventory Cardiac Module Heart Disease Symptoms Scale, and a parental survey. The association between treatment strategy and HRQOL was evaluated, and the entire sTOF cohort was compared to published values for the healthy pediatric population and to children with complex congenital heart disease and other chronic illness. The study cohort included 143 sTOF subjects, of which 59 underwent a primary repair, and 84 had a staged repair approach. There was no association between initial management strategy and lower HRQOL. For the entire cohort, in general, individual domain scores decreased as age sequentially increased. Across domain measurements, mean scores for the sTOF cohort were significantly lower than the healthy pediatric population and comparable to those with other forms of complex CHD and other chronic health conditions. The presence of a genetic syndrome was significantly associated with a poor HRQOL (p = 0.003). Initial treatment strategy for sTOF was not associated with differences in late HRQOL outcomes, though the overall HRQOL in this sTOF cohort was significantly lower than the general population, and comparable to others with chronic illness., (© 2024. The Author(s).)

Background: Neonates with tetralogy of Fallot and symptomatic cyanosis (sTOF) require early intervention, utilizing either a staged repair (SR) or primary repair (PR) approach. They are exposed to several sources of low-dose ionizing radiation, which may contribute to increased cancer risk., Objectives: The purpose of this study was to compare cumulative radiation exposure and associated lifetime attributable risk (LAR) of cancer between treatment strategies in sTOF., Methods: Neonates with sTOF who underwent SR or PR from 2012 to 2017 were retrospectively reviewed from the Congenital Cardiac Research Collaborative. Radiation exposure from all radiologic studies prior to 18 months of age was converted to organ-equivalent doses and projected LAR of cancer incidence using the National Cancer Institute dosimetry tools., Results: There were 242 neonates from 8 centers, including patients with 146 SR and 96 PR. Cumulative total effective dose was significantly higher for SR (median 8.3 mSv, IQR: 3.0-17.4 mSv) than PR (2.1 mSv, IQR: 0.8-8.5 mSv; P  < 0.001). Cumulative organ-level doses were significantly higher in SR compared to PR. Regardless of treatment strategy, LARs were higher in females compared to males. Among organs with median exposure >1 mGy in females, the LAR was highest for breast in SR (mean 1.9/1,000 patients). The highest proportion of cancers attributable to radiation exposure was projected for thyroid cancer in females undergoing SR (7.3%)., Conclusions: Cumulative radiation exposure and LARs were higher among those undergoing SR compared to PR. This will be an important factor to consider in determining the preferred neonatal treatment strategy and should substantiate efforts to reduce radiation exposure in this vulnerable population., Competing Interests: Financial support for this research was derived, in part, from the Kennedy Hammill Pediatric Cardiac Research Fund, the Liam Sexton Foundation, and A Heart Like Ava. Dr Goldstein has reported consulting relationships with Medtronic, W.L. Gore & Associates, and Edwards Lifesciences; and is an advisory board member for PECA Labs. Dr Qureshi has reported consulting relationships with W.L. Gore & Associates, Edwards Lifesciences, and Abiomed. Dr Zampi has reported consulting relationships with Medtronic and W.L. Gore & Associates. All other authors have reported that they have no relationships relevant to the contents of this paper to disclose., (© 2024 The Authors.)

Background: There is a significant incidence of pre-Fontan attrition-defined as failure to undergo Fontan completion-after superior cavopulmonary connection. This study investigated the impact of at least moderate ventricular dysfunction (VD) and atrioventricular valve regurgitation (AVVR) on pre-Fontan attrition., Methods: This single-center retrospective cohort study included all infants who underwent Norwood palliation from 2008 to 2020 and subsequently underwent superior cavopulmonary connection. Pre-Fontan attrition was defined as death, listing for heart transplantation before Fontan completion, or unsuitability for Fontan completion. The study's secondary outcome was transplant-free survival., Results: Pre-Fontan attrition occurred in 34 of 267 patients (12.7%). Isolated VD was not associated with attrition. However, patients with isolated AVVR had 5 times the odds of attrition (odds ratio, 5.4; 95% CI 1.8-16.2), and patients with both VD and AVVR had 20 times the odds of attrition (odds ratio, 20.1; 95% CI 7.7-52.8) compared with patients without VD or AVVR. Only patients with both VD and AVVR had significantly worse transplant-free survival compared with patients without VD or AVVR (hazard ratio, 7.7; 95% CI 2.8-21.6)., Conclusions: The additive effect of VD and AVVR is a powerful contributor to pre-Fontan attrition. Future research investigating therapies that can mitigate the degree of AVVR may help improve Fontan completion rates and long-term outcomes., (Copyright © 2023 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)