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Introduction: Neonates with tetralogy of Fallot and pulmonary atresia (TOF/PA) with pulmonary blood flow supplied by the ductus arteriosus require early intervention. This may be accomplished by: initial palliation (IP) followed by complete repair (CR) or initial primary repair (PR). The optimal approach for patients with TOF/PA has not been established. Methods: Neonates with TOF/PA who underwent IP or PR from 2005-17 were retrospectively reviewed from the Congenital Catheterization Research Collaborative. The primary outcome was mortality. Outcomes were compared as IP vs PR and IP+CR vs PR. Secondary outcomes included hospital and procedural complications and are listed in table 1. Propensity scoring was used to adjust for baseline differences between strategies. Results: Of 282 neonates with TOF/PA, 106 underwent PR and 176 underwent IP (144 surgical, 32 transcatheter). Prior to initial intervention, IP patients had higher rates of mechanical ventilation (83.9% vs 72.2%, p=0.023) and DiGeorge syndrome (14.77% vs 4.72%, p=0.009). Mortality was greater in the IP cohort (HR 2.7, 95% CI 1.02 - 7.1, p = 0.046), with no mortality in the PR cohort after 6 months post-repair. After adjustment, differences in survival were no longer significant (HR 1.4, 95% CI 0.7 - 3.1, p=0.39). Both cohorts had similar mechanical ventilation duration and inotrope use as well as procedural and hospital complications. Intensive care and hospital length of stay, cardiac bypass (CPB) and anesthesia time favored PR when compared to IP+CR (p= Conclusions: In neonates with TOF/PA, the IP approach is more often utilized in higher-risk patients. Accounting for this difference, IP and PR strategies have similar adjusted survival rates. Perioperative morbidities and lower risk for reintervention generally favor PR.

Introduction: Symptomatic neonates with tetralogy of Fallot (sTOF) and hypoplastic branch pulmonary arteries (hPA) are at high risk. Management strategies include staged repair (SR) - initial palliation (IP) followed by later complete repair (CR) - or primary repair (PR). A balanced comparison of these approaches is needed in the sTOF neonate with hPA. Methods: Consecutive neonates with sTOF and hPA - defined as one hPA z-score Results: The cohort comprised 188 neonates including 121 SR (IP: 29 transcatheter; 54 surgery) and 67 PR patients. Presence of pulmonary atresia, lower gestational age and birthweight were more common in the SR cohort. Median right and left hPA z-scores were -2.19 (IQR -2.65, -1.92) and -2.33 (-2.84, -1.99), respectively. There were significant differences in treatment strategy across centers (p Conclusions: In this multicenter comparison of SR or PR for management of neonates with sTOF and hPA, after PSA, no difference in mortality was found. Neonatal morbidities largely favored the SR group, but cumulative morbidities and reintervention favored the PR group. Further study is needed to determine late impact of these early advantages to SR.

Neonates with tetralogy of Fallot and symptomatic cyanosis (sTOF) require early intervention.This study sought to perform a balanced multicenter comparison of staged repair (SR) (initial palliation [IP] and subsequent complete repair [CR]) versus primary repair (PR) treatment strategies.Consecutive neonates with sTOF who underwent IP or PR at ≤30 days of age from 2005 to 2017 were retrospectively reviewed from the Congenital Cardiac Research Collaborative. The primary outcome was death. Secondary outcomes included component (IP, CR, PR) and cumulative (SR): hospital and intensive care unit lengths of stay; durations of cardiopulmonary bypass, anesthesia, ventilation, and inotrope use; and complication and reintervention rates. Outcomes were compared using propensity score adjustment.The cohort consisted of 342 patients who underwent SR (IP: surgical, n = 256; transcatheter, n = 86) and 230 patients who underwent PR. Pre-procedural ventilation, prematurity, DiGeorge syndrome, and pulmonary atresia were more common in the SR group (p ≤0.01). The observed risk of death was not different between the groups (10.2% vs 7.4%; p = 0.25) at median 4.3 years. After adjustment, the hazard of death remained similar between groups (hazard ratio: 0.82; 95% confidence interval: 0.49 to 1.38; p = 0.456), but it favored SR during early follow-up (4 months; p = 0.041). Secondary outcomes favored the SR group in component analysis, whereas they largely favored PR in cumulative analysis. Reintervention risk was higher in the SR group (p = 0.002).In this multicenter comparison of SR or PR for management of neonates with sTOF, adjusted for patient-related factors, early mortality and neonatal morbidity were lower in the SR group, but cumulative morbidity and reinterventions favored the PR group, findings suggesting potential benefits to each strategy.

To compare management strategies for neonates2.5 kg with tetralogy of Fallot and symptomatic cyanosis who either undergo staged repair (SR) (initial palliation followed by later complete repair) or primary repair (PR).Consecutive neonates with tetralogy of Fallot and symptomatic cyanosis weighing2.5 kg at initial intervention and between 2005 and 2017 were retrospectively reviewed from the Congenital Cardiac Research Collaborative. Primary outcome was mortality and secondary outcomes included component (eg, initial palliation, complete repair, or primary repair) and cumulative (SR: initial palliation followed by later complete repair) hospital and intensive care unit lengths of stay, durations of ventilation, inotrope use, cardiopulmonary bypass time, procedural complications, and reintervention. Outcomes were compared with propensity score adjustments with PR as the reference group.The cohort included 76 SR (initial palliation: 53 surgical and 23 transcatheter) and 44 PR patients. The observed risk of overall mortality was similar between SR and PR groups (15.8% vs 18.2%: P = .735). The adjusted hazard of mortality remained similar between groups overall (hazard ratio, 0.59; 95% confidence interval, 0.26-1.36; P = .214), as well as during short-term (4 months: hazard ratio, 0.37; 95% confidence interval, 0.13-1.09; P = .071) and midterm (4 months: hazard ratio, 1.32; 95% confidence interval, 0.30-5.79; P = .717) follow-up. Reintervention in the first 18 months was common in both groups (53.2% vs 48.4%; hazard ratio, 1.69; 95% confidence interval, 0.96-2.28; P = .072). Adjusted procedural complications and neonatal morbidity burden were overall lower in the SR group. Cumulative secondary outcome burdens largely favored the PR group.In this study comparing SR and PR treatment strategies for neonates with tetralogy of Fallot and symptomatic cyanosis and weight2.5 kg, mortality and reintervention burden was high and independent of treatment strategy. Other potential advantages were observed with each approach.

Supplemental Digital Content is available in the text., Introduction: Inpatient rounding serves numerous roles. The American Academy of Pediatrics recommends a family-centered bedside model. Residents identified physical examination teaching during and satisfaction with rounds as areas for improvement. A resident group developed a project utilizing quality improvement (QI) methodology to address these concerns. We aimed to increase the frequency of bedside physical examination teaching most or every day on a single inpatient unit by 20% over 1 year, with secondary goals to increase the percentage of interns spending one hour or more at bedside per day by 10% and intern satisfaction by 15%, without impacting rounding duration. Methods: We developed an organizational structure to complete a long-term resident-led project. Interventions included daily bedside examination teaching on rounds, afternoon examinations, goal communication, topic recording, and a teaching “tip sheet.” Using an institutional QI framework, we utilized iterative plan-do-study-act cycles to implement interventions and surveys to assess outcomes, with rounding efficiency as a balancing measure. Results: The survey response rate was 57%. Bedside teaching frequency increased from a mean of 10% to 61%, perceived time at the bedside increased from 37% to 59%, and rounding satisfaction improved from a rating of 6.7/10 to 7.4/10. Efficiency was not impacted. Conclusions: We improved inpatient rounds bedside physical examination teaching and satisfaction without sacrificing efficiency. This project demonstrates the feasibility and success of a resident-driven education initiative to successfully motivate fellow residents and colleagues across disciplines to enact change. The organizational structure may serve as a model for resident-led QI projects across institutions.

Neonates with tetralogy of Fallot and symptomatic cyanosis (sTOF) may undergo palliation to delay complete repair (CR) with a systemic to pulmonary artery connection (SPC) or right ventricular outflow tract intervention (RVOTI). Comparison of these palliative strategies is lacking. Consecutive

Located contiguously on the long arm of the second chromosome are gene paralogs encoding the immunoglobulin-family co-activation receptors CD28 and cytotoxic T-lymphocyte-associated protein 4 (CTLA4). CD28 and CTLA4 share the same B7 ligands yet each provides opposing proliferative signals to T cells. Herein, we describe for the first time two unrelated subjects with coexisting CD28 and CTLA4 haploinsufficiency due to heterozygous microdeletions of chromosome 2q. Although their clinical phenotype, multi-organ inflammatory disease, is superficially similar to that of CTLA4 haploinsufficient autoimmune lymphoproliferative syndrome type V (ALPS5) patients, we demonstrate our subjects' underlying immunopathology to be distinct. Unlike ALPS5 T cells which hyperproliferate to T-cell receptor-mediated activation and infiltrate organs, T cells from our subjects are hypoproliferative and do not. Instead of T cell infiltrates, biopsies of affected subject tissues demonstrated infiltrates of lineage negative lymphoid cells. This histologic feature correlated with significant increases in circulating type 3 innate lymphoid cells (ILC3s) and ILC3 cytokines, interleukin 22, and interleukin-17A. CTLA4-Ig monotherapy, which we trialed in one subject, was remarkably effective in controlling inflammatory diseases, normalizing ILC3 frequencies, and reducing ILC3 cytokine concentrations.