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Background: Understanding of the hemostatic and complement alterations associated with cardiopulmonary bypass (CPB) in pediatric patients and the impact of these alterations on outcome is limited., Objectives: The present study prospectively characterized these alterations and their association with postoperative outcomes in pediatric CPB., Patients/methods: All patients <21 years undergoing CPB at the authors' institution between 2020 and 2021 that weighed >3 kg, were >36 weeks gestational age, and had no known prothrombotic or hemorrhagic disorders were eligible. Blood samples were analyzed for multiple hemostatic and complement biomarkers pre-, intra-, and 24 hours post-CPB. Biomarker levels were compared to clinical outcomes, including chest tube output (CTO)., Results: Fifty consecutive patients were enrolled. CPB resulted in multiple significant alterations in hemostatic and complement components. Lower platelet counts (<80 x 10 9 platelets/L) at CPB termination were associated with increased postoperative CTO (p=0.003). Lower factor VIII levels (<60 IU/dl) at the end of CPB were associated with a longer hospital stay (p<0.001) and increased postoperative CTO (p<0.001). Patients undergoing staged single ventricle reconstruction were more likely to have lower platelet counts at CPB termination (p=0.009) and higher CTO postoperatively (p=0.001) than patients undergoing other types of surgical repair. These differences were not due to different preoperative platelet counts, increased incidences of circulatory arrest, or longer CPB times., Conclusions: These data suggest that intraoperative alterations in hemostatic system components may predict postoperative outcomes in pediatric CPB. Further study is needed to determine if interventions targeting platelets or factor VIII could improve outcomes in pediatric CPB., (Copyright © 2024 International Society on Thrombosis and Haemostasis. Published by Elsevier Inc. All rights reserved.)

Care and outcomes for the more than 40,000 patients undergoing pediatric and congenital heart surgery in the United States annually are known to vary widely. While consensus recommendations have been published across numerous fields as one mechanism to promote a high level of care delivery across centers, it has been more than two decades since the last pediatric heart surgery recommendations were published in the United States. More recent guidance is lacking, and collaborative efforts involving the many disciplines engaged in caring for these children have not been undertaken to date. The present initiative brings together professional societies spanning numerous care domains and congenital cardiac surgeons, pediatric cardiologists, nursing, and other healthcare professionals from diverse programs around the country to develop consensus recommendations for United States centers. The focus of this initial work is on pediatric heart surgery, and it is recommended that future efforts focus in detail on the adult congenital population. We describe the background, rationale, and methodology related to this collaborative effort, and recommendations put forth for Essential Care Centers (essential services necessary for any program), and Comprehensive Care Centers (services to optimize comprehensive and high-complexity care), encompassing structure, process, and outcome metrics across 14 domains., (Copyright © 2023 [Author/Employing Institution]. Published by Elsevier Inc. All rights reserved.)

Background: Studies examining the volume-outcome relationship in congenital heart surgery (CHS) are more than a decade old. Since then, mortality has declined, and case-mix adjustment has evolved. We determined the current relationship between hospital CHS volume and outcomes., Methods: Patients aged ≤18 years undergoing index operations in The Society of Thoracic Surgeons-Congenital Heart Surgery Database (2017-2020) were included. Associations between annual hospital volume and case-mix-adjusted operative mortality, major complications, failure to rescue (FTR), and postoperative length of stay (PLOS) were assessed using Bayesian hierarchical models, overall, by The Society of Thoracic Surgeons-European Association for Cardio-Thoracic Surgery (STAT) category, and for the Norwood procedure., Results: Across 101 centers (76,714 index operations), median annual volume was 144 operations/y. Operative mortality was 2.7%. Lower-volume hospitals had higher mortality, with an apparent transition zone at ∼190 operations/y (95% credible interval [CrI], 115-450 operations/y), below which a sustained uptick in the estimated odds of death occurred. Odds of death compared with a 450 operations/y reference were 50 operations/y (odds ratio [OR], 1.84; 95% CrI, 1.41-2.37), 100 operations/y (OR, 1.37; 95% CrI, 1.08-1.71), 200 operations/y (OR, 0.92; 95% CrI, 0.1-1.18), 300 operations/y (OR, 0.89; 95% CrI, 0.76-1.04). The volume-outcome effect was more apparent for STAT 4 to 5 than STAT 1 to 3 operations. In the overall cohort, PLOS and complications were similar across hospital volumes, whereas FTR rates were higher at lower-volume hospitals. Lower-volume hospitals had worse outcomes after the Norwood procedure, most notably mortality and FTR., Conclusions: Hospital volume is associated with mortality and FTR after CHS. The relationship is strongest for high-risk operations. These data can inform ongoing initiatives to improve CHS care., (Copyright © 2023 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Objective: The hemoglobin threshold for a decision to transfuse red blood cells in univentricular patients with parallel circulation is unclear. A pediatric expertise initiative put forth a "weak recommendation" for avoiding reflexive transfusion beyond a hemoglobin of 9 g/dL. We have created a mathematical model to assess the impact of hemoglobin thresholds in patients with parallel circulation., Methods: A univentricular circulation was mathematically modeled. We examined the impact on oxygen extraction ratios and systemic and venous oxygen saturations by varying hemoglobin levels, pulmonary to systemic blood flow ratios, and total cardiac output., Results: Applying a total cardiac index of 6 L/m 2 /min, oxygen consumption of 150 mL/min/m 2 , and a Q p /Q s ∼ 1, we found a hemoglobin level of 9 g/dL would lead to severe arterial (arterial oxygen saturation <70%) and venous (systemic venous oxygen saturation <40%) hypoxemia. To operate above the critical oxygen economy boundary (systemic venous oxygen saturation ∼40%) and maintain arterial oxygen saturation >70% would require either increasing the cardiac index to ∼ 9 L/m 2 /min or increasing the hemoglobin to greater than 13 g/dL. Further, we found a greater improvement in arterial and venous saturation arises when hemoglobin is augmented from levels below 12 g/dL., Conclusions: Based on our model, a hemoglobin level of 9 g/dL would require a constricted set of features to sustain arterial saturations >70% and systemic venous saturations >40% and would risk unfavorable oxygen economy with elevations in oxygen consumption. Further prospective clinical studies are needed to delineate the impact of restrictive transfusion practices in univentricular circulation., (Copyright © 2022 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.)

Previous studies suggested that contact pathway factors drive thrombosis in mechanical circulation. We used a rabbit model of veno-arterial extracorporeal circulation (VA-ECMO) to evaluate the role of factors XI and XII in ECMO-associated thrombosis and organ damage. Factors XI and XII (FXI, FXII) were depleted using established antisense oligonucleotides before placement on a blood-primed VA-ECMO circuit. Decreasing FXII or FXI to < 5% of baseline activity significantly prolonged ECMO circuit lifespan, limited the development of coagulopathy, and prevented fibrinogen consumption. Histological analysis suggested that FXII depletion mitigated interstitial pulmonary edema and hemorrhage whereas heparin and FXI depletion did not. Neither FXI nor FXII depletion was associated with significant hemorrhage in other organs. In vitro analysis showed that membrane oxygenator fibers (MOFs) alone are capable of driving significant thrombin generation in a FXII- and FXI-dependent manner. MOFs also augment thrombin generation triggered by low (1 pM) or high (5 pM) tissue factor concentrations. However, only FXI elimination completely prevented the increase in thrombin generation driven by MOFs, suggesting MOFs augment thrombin-mediated FXI activation. Together, these results suggest that therapies targeting FXII or FXI limit thromboembolic complications associated with ECMO. Further studies are needed to determine the contexts wherein targeting FXI and FXII, either alone or in combination, would be most beneficial in ECMO. Moreover, studies are also needed to determine the potential mechanisms coupling FXII to end-organ damage in ECMO., (© 2023 by The American Society of Hematology. Licensed under Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International (CC BY-NC-ND 4.0), permitting only noncommercial, nonderivative use with attribution. All other rights reserved.)

Background: The supported Ross is used to mitigate the neoaortic root dilation that has been described with the unsupported Ross. There is limited literature assessing the efficacy of the supported Ross in young patients. In this study, the fate of the neoaortic root was compared in the supported and unsupported Ross procedure in adolescent patients., Methods: A retrospective review was performed of patients who underwent the Ross procedure between 1996 and 2019. An analysis was conducted of patients aged 10 to 18 years who underwent the supported and unsupported Ross operation, without a Konno enlargement, to assess for longitudinal echocardiographic changes. Given differences in follow-up time, both regression analysis and Mann-Whitney nonparametric tests were used to correct for time from discharge to most recent follow-up., Results: The median follow-up time for supported and unsupported Ross patients without a Konno enlargement was 2.90 years (0.21-13.03 years) and 12.13 years (2.63-19.47 years), respectively. Unsupported Ross patients experienced a higher rate of change per year in the aortic annulus (P = .003 and P = .014) and aortic sinus (P = .002 and P = .002) diameters, respectively. There was no significant difference in the rate of change of end-diastolic left ventricular internal diameter (P = .703 and P = .92) and aortic insufficiency (P = .687 and P = .215) between the supported and unsupported Ross patients., Conclusions: Progressive dilation of the neoaortic root in unsupported Ross patients is significantly mitigated with the supported Ross with excellent stability. The supported Ross is safe and effective and may play an increasing role in the management of children with aortic disease., (Copyright © 2023 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Aims: Hypoplastic left heart syndrome (HLHS) survival relies on surgical reconstruction of the right ventricle (RV) to provide systemic circulation. This substantially increases the RV load, wall stress, maladaptive remodelling, and dysfunction, which in turn increases the risk of death or transplantation., Methods and Results: We conducted a phase 1 open-label multicentre trial to assess the safety and feasibility of Lomecel-B as an adjunct to second-stage HLHS surgical palliation. Lomecel-B, an investigational cell therapy consisting of allogeneic medicinal signalling cells (MSCs), was delivered via intramyocardial injections. The primary endpoint was safety, and measures of RV function for potential efficacy were obtained. Ten patients were treated. None experienced major adverse cardiac events. All were alive and transplant-free at 1-year post-treatment, and experienced growth comparable to healthy historical data. Cardiac magnetic resonance imaging (CMR) suggested improved tricuspid regurgitant fraction (TR RF) via qualitative rater assessment, and via significant quantitative improvements from baseline at 6 and 12 months post-treatment ( P < 0.05). Global longitudinal strain (GLS) and RV ejection fraction (EF) showed no declines. To understand potential mechanisms of action, circulating exosomes from intramyocardially transplanted MSCs were examined. Computational modelling identified 54 MSC-specific exosome ribonucleic acids (RNAs) corresponding to changes in TR RF, including miR-215-3p, miR-374b-3p, and RNAs related to cell metabolism and MAPK signalling., Conclusion: Intramyocardially delivered Lomecel-B appears safe in HLHS patients and may favourably affect RV performance. Circulating exosomes of transplanted MSC-specific provide novel insight into bioactivity. Conduct of a controlled phase trial is warranted and is underway.Trial registration number NCT03525418., (© The Author(s) 2023. Published by Oxford University Press on behalf of the European Society of Cardiology.)

Objective: Prematurity, low birth weight, genetic syndromes, extracardiac conditions, and secondary cardiac lesions are considered high-risk conditions associated with mortality after stage 1 palliation. We report the impact of these conditions on outcomes from a prospective multicenter improvement collaborative., Methods: The National Pediatric Cardiology Quality Improvement Collaborative Phase II registry was queried. Comorbid conditions were categorized and quantified to determine the cumulative burden of high-risk diagnoses on survival to the first birthday. Logistic regression was applied to evaluate factors associated with mortality., Results: Of the 1421 participants, 40% (575) had at least 1 high-risk condition. The aggregate high-risk group had lower survival to the first birthday compared with standard risk (76.2% vs 88.1%, P < .001). Presence of a single high-risk diagnosis was not associated with reduced survival to the first birthday (odds ratio, 0.71; confidence interval, 0.49-1.02, P = .066). Incremental increases in high-risk diagnoses were associated with reduced survival to first birthday (odds ratio, 0.23; confidence interval, 0.15-0.36, P < .001) for 2 and 0.17 (confidence interval, 0.10-0.30, P < .001) for 3 to 5 high-risk diagnoses. Additional analysis that included prestage 1 palliation characteristics and stage 1 palliation perioperative variables identified multiple high-risk diagnoses, poststage 1 palliation extracorporeal membrane oxygenation support (odds ratio, 0.14; confidence interval, 0.10-0.22, P < .001), and cardiac reoperation (odds ratio, 0.66; confidence interval, 0.45-0.98, P = .037) to be associated with reduced survival odds to the first birthday., Conclusions: The presence of 1 high-risk diagnostic category was not associated with decreased survival at 1 year. Cumulative diagnoses across multiple high-risk diagnostic categories were associated with decreased odds of survival. Further patient accrual is needed to evaluate the impact of specific comorbid conditions within the broader high-risk categories., (Copyright © 2022 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.)

Background: Overall one-year non-mortality outcomes for surgically palliated hypoplastic left heart syndrome (HLHS) patients remain understudied. Using the metric Days Alive and Outside of Hospital (DAOH), the present study sought to characterize expectations for surgically palliated patients' first year of life., Methods: The Pediatric Health Information System database was used to identify by ICD-10 code all HLHS patients who underwent surgical palliation (Norwood/hybrid and/or heart transplantation [HTx]) during their index neonatal admission and were successfully discharged alive (n  =  2227) and for whom one-year DAOH could be calculated. DAOH quartiles were used to group patients for analysis., Results: Median one-year DAOH was 304 (interquartile range [IQR] 250-327), including a median index admission length of stay of 43 days (IQR 28-77). Patients required a median 2 (IQR 1-3) readmissions, each spanning 9 days (IQR 4-20). One-year readmission mortality or hospice discharge occurred in 6% of patients. Patients with lower-quartile DAOH had a median DAOH of 187 (IQR 124-226), whereas upper-quartile DAOH patients had a median DAOH of 335 (IQR 331-340) ( P  < .001). Readmission mortality/hospice-discharge rates were 14% and 1%, respectively ( P  < .01). On multivariable analysis, factors independently associated with lower-quartile DAOH included interstage hospitalization (odds ratio [OR] 44.78 [95% confidence interval [CI] 25.1-80.2]), index-admission HTx (8.73 [4.66-16.3]), preterm birth (1.97 [1.34-2.90]), chromosomal abnormality (1.85 [1.26-2.73]), age >7 days at surgery (1.50 [1.14-1.99]), and non-white race/ethnicity (1.33 [1.01-1.75])., Conclusions: In the current era, surgically palliated HLHS infants spend approximately 10 months alive and outside of the hospital, although outcomes are highly variable. Knowledge of the factors associated with lower DAOH can inform expectations and guide management decisions.

The mechanisms driving the pathologic state created by extracorporeal membrane oxygenation (ECMO) remain poorly defined. We developed the first complete blood-primed murine model of veno-arterial ECMO capable of maintaining oxygenation and perfusion, allowing molecular studies that are unavailable in larger animal models. Fifteen C57BL/6 mice underwent ECMO by cannulating the left common carotid artery and the right external jugular vein. The mean arterial pressure was measured through cannulation of the femoral artery. The blood-primed circuit functioned well. Hemodynamic parameters remained stable and blood gas analyses showed adequate oxygenation of the animals during ECMO over a 1-hour timeframe. A significant increase in plasma-free hemoglobin was observed following ECMO, likely secondary to hemolysis within the miniaturized circuit components. Paralleling clinical data, ECMO resulted in a significant increase in plasma levels of multiple proinflammatory cytokines as well as evidence of early signs of kidney and liver dysfunction. These results demonstrate that this novel, miniature blood-primed ECMO circuit represents a functional murine model of ECMO that will provide unique opportunities for further studies to expand our knowledge of ECMO-related pathologies using the wealth of available genetic, pharmacological, and biochemical murine reagents not available for other species., Competing Interests: Disclosure: The authors have no conflicts of interest to report., (Copyright © ASAIO 2022.)

Background: Infants experience the worst one-year post-heart transplant (HTx) survival of any other pediatric group. Although mechanical ventilatory (MV) requirement at the time of transplant is an established predictor of post-transplant mortality, the impacts of commonly co-utilized support modalities such as total parenteral nutrition (TPN)-dependence and paralytics are understudied. Methods: All infant HTx recipients from 2003 to 2020 in both the United Network for Organ Sharing and Pediatric Health Information System databases were identified (n = 1344) and categorized depending upon support requirement at the time of transplant-none (59%), MV-only (10%), MV + Paralytics (2%), TPN-dependence-only (15%), MV + TPN (10%), and MV + Paralytics + TPN (4%). The primary study aim was to characterize the impact of TPN-dependence and paralytics on one-year post-transplant survival (PTS). Results: Compared to no-support, supported infants were generally at higher risk and more ill at transplant, with greater rates of congenital heart disease, renal and hepatic dysfunctions, and inotrope requirements. Post-transplant hospital outcomes were inferior among supported patients; all support groups experienced longer post-transplant MV, intensive care unit, and hospital lengths of stay (all P  < .05 vs no-support). Upon multivariable analysis, each support modality independently predicted 1-year mortality (MV vs no-MV: 1.54 [1.10-2.14]; MV + Paralytics vs neither: 2.02 [1.25-3.27]; TPN vs no-TPN: 1.53 [1.10-2.13]; P  < .01 for all), whereas no-support was protective (HR 0.66 [95% CI 0.48-0.91]). Conclusions: Infants who require paralytics and/or who are TPN-dependent at the time of HTx experience worse one-year PTS. Such knowledge can assist in risk-stratification, and the identification of patients who would benefit from pretransplant optimization.

Background: Repair of complex congenital heart disease frequently requires use of a patch as an anatomic substitute. The study's aim is to evaluate the use, effectiveness, and safety of using small intestine submucosal extracellular matrix (SIS-ECM) patches in a congenital cardiac surgery program., Methods: This is a single-center, retrospective, cohort study of surgeries using SIS-ECM between 2012 and 2019. The SIS-ECM data were categorized by use and type (four-ply and two-ply). All reinterventions and complications were reviewed by an independent surgeon, a practicing congenital heart surgeon, and a pediatric cardiologist., Results: In all, 408 SIS-ECM patches were used in 309 patients (188 male, 121 female; median age 8.5 months). Use of the patches consisted of 314 arterioplasties (77%), 22 venoplasties (5.4%), 63 intracardiac repairs (15.4%), and 9 valve repairs (2.2%). The most common use was for pulmonary artery repair (n = 181; 44.4%). Median follow-up time was 3.9 years (range, 3 days to 7.4 years). Ten patches (2.5%) required surgical reintervention (2 in the first 30 days and 5 in the first year) and 27 (6.6%) required percutaneous reinterventions (2 in the first 30 days and 22 in the first year). Between four-ply (n = 376) and two-ply (n = 32) SIS-ECM, the rate of surgical (2.1% [n = 8] vs 6.3% [n = 2], P = .18) or percutaneous reinterventions (6.4% [n = 24] vs 9.4% [n = 3], P = .46) was not different. There were no deaths related to the SIS-ECM patch or reports of calcification., Conclusions: The SIS-ECM is a viable patch option that can be used in various cardiac and vascular reconstructive surgeries with low risk of failure and calcification. Long-term, positive outcomes may be maximized by using consistent techniques and understanding the appropriate applications of the patch., (Copyright © 2022 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: Digoxin has been associated with reduced interstage mortality for patients with functional single ventricles with aortic hypoplasia or ductal-dependent systemic circulation. The NEONATE (type of stage 1 palliation operation, postoperative extracorporeal membrane oxygenation, discharge with opiates, no digoxin at discharge, postoperative arch obstruction, moderate to severe tricuspid regurgitation without an oxygen requirement, and extra oxygen required at discharge in patients with moderate to severe tricuspid regurgitation) score can stratify patients by risk of death or transplantation (DTx) on the basis of clinical factors. The study investigators suspected a variable transplant-free survival benefit of digoxin in high-risk vs low-risk patients., Methods: National Pediatric Cardiology Quality Improvement Collaborative patients discharged after stage 1 palliation with complete data were categorized as high- or low-risk on the basis of a modified NEONATE score. The primary outcome of DTx was evaluated. A mixed-effect regression evaluated associations between digoxin prescription and risk factors., Results: A total of 1199 patients were included; 399 (33%) were high risk. Baseline demographics were similar between the cohorts. Blalock-Taussig shunt or a hybrid operation, postoperative extracorporeal membrane oxygenation, opiate prescription, and significant tricuspid regurgitation or arch obstruction were more common in high-risk patients. The odds of DTx were 65% lower in high-risk patients prescribed digoxin compared with patients who were not (P = .001). Digoxin prescription was associated with 60.8% lower DTx in the high-risk cohort (7.8% vs 19.9%; P = .001). There was no significant difference in the DTx rate according to digoxin prescription in the low-risk cohort (4.7% vs 5.7%; P = .46). Blalock-Taussig shunt, aortic arch obstruction, and significant tricuspid regurgitation were most strongly associated with deriving a benefit from digoxin., Conclusions: Digoxin use is associated with significant improvement in transplant-free survival in high-risk but not in low-risk interstage patients. A tailored approach to the use of digoxin in interstage patients may be warranted., (Copyright © 2022 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: This study describes the evolving in-hospital management strategies for neonates who are diagnosed with tetralogy of Fallot (ToF)., Methods: The Pediatric Health Information System (PHIS) database was used to identify admitted patients aged 0 to 1 month old with ToF from 2010 through 2019; era 1, 2010 through 2014; and era 2, 2015 through 2019. International Classification of Diseases codes were used to identify related interventions that occurred during this admission but not necessarily as a neonate: full repair, systemic-to-pulmonary shunt, and percutaneous stent in the right ventricular outflow tract and/or patent ductus arteriosus., Results: Among 6021 neonates diagnosed with ToF, 2030 (34%) underwent an intervention: 60% had total repair, 31% systemic-to-pulmonary shunt, and 9% percutaneous stent. In the no-intervention cohort, in-hospital mortality was 9%. In-hospital mortality between repair (6%), shunt (6%), and stent (3%) patients (P = .446) did not differ. Regarding regional practices, no intervention was most frequently used in the Midwest (69% vs 65% average for all other regions [avg], P = .075) while interventions overall were performed most frequently in the West (36% vs 33.5% avg, P = .075). Among the interventions, full repair was most frequent in the Northeast (76% vs 57% avg, P < .001), shunt was most frequent in the Midwest (39% vs 28% avg, P < .001), and stent was most frequent in the South (11% vs 7% avg, P = .083). Between eras 1 and 2, the type of intervention changed: full repair (52% vs 69%, P < .001) and stent (1% vs 16%, P < .001) increased, while shunt decreased (47% vs 15%, P < .001)., Conclusions: Although most neonates admitted with ToF are discharged with no intervention, more than one-third undergo some intervention with a 3% to 6% mortality. The proportion of these patients who undergo an intervention is unchanged during the past decade, but the types of intervention have changed, and significant regional differences exist., (Copyright © 2022 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: The Society of Thoracic Surgeons Congenital Heart Surgery Database (STS-CHSD) provides observed-to-expected (O/E) operative mortality ratios to more than 100 congenital heart centers in North America. We compared the current approach for estimating O/E ratios to approaches incorporating information on diagnosis as moderators of procedures, other unused risk factors, and additional variation in confidence interval construction to characterize center performance., Methods: Bayesian additive regression trees (BART) and lasso models linked operative mortality to diagnosis-procedure categories, procedure-specific risk factors, and syndromes/abnormalities. Bootstrapping accounted for variation in the STS-CHSD (STS bootstrap) and lasso CIs. We compared O/E estimates, interquartile range of CI widths, and concordance of center performance categorizations (worse-than-, as-, or better-than-expected mortality) of the new approaches to the STS-CHSD., Results: In 110 surgical centers including 98,822 surgical operative encounters, there were 2818 (2.85%) operative mortalities (center range, 0.37%-10%). Compared with the STS-CHSD, BART- and lasso-estimated O/E ratios varied more and had narrower confidence intervals (interquartile range of confidence interval: STS-CHSD = 1.11, STS bootstrap = 0.98; lasso = 0.80; BART = 0.96). Concordance of performance categorization with the STS-CHSD ranged from 84% (lasso) to 91% (STS Bootstrap); more than 70% of discordant centers improved categories. Discordant centers had smaller volumes, fewer operative mortalities, and treated more patients with congenital lung abnormalities., Conclusions: Relative to the STS-CHSD, up to 16% of hospitals changed performance categories, most improving performance. Given the significance of quality reports for congenital heart centers, inclusion of additional risk factors and unaddressed variation should be considered., (Copyright © 2022 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: The Society of Thoracic Surgeons (STS) Congenital Heart Surgery Database (CHSD) provides risk-adjusted operative mortality rates to approximately 120 North American congenital heart centers. Optimal case-mix adjustment methods for operative mortality risk prediction in this population remain unclear., Methods: A panel created diagnosis-procedure combinations of encounters in the CHSD. Models for operative mortality using the new diagnosis-procedure categories, procedure-specific risk factors, and syndromes or abnormalities included in the CHSD were estimated using Bayesian additive regression trees and least absolute shrinkage and selector operator (lasso) models. Performance of the new models was compared with the current STS CHSD risk model., Results: Of 98 825 operative encounters (69 063 training; 29 762 testing), 2818 (2.85%) STS-defined operative mortalities were observed. Differences in sensitivity, specificity, and true and false positive predicted values were negligible across models. Calibration for mortality predictions at the higher end of risk from the lasso and Bayesian additive regression trees models was better than predictions from the STS CHSD model, likely because of the new models' inclusion of diagnosis-palliative procedure variables affecting <1% of patients overall but accounting for 27% of mortalities. Model discrimination varied across models for high-risk procedures, hospital volume, and hospitals., Conclusions: Overall performance of the new models did not differ meaningfully from the STS CHSD risk model. Adding procedure-specific risk factors and allowing diagnosis to modify predicted risk for palliative operations may augment model performance for very high-risk surgical procedures. Given the importance of risk adjustment in estimating hospital quality, a comparative assessment of surgical program quality evaluations using the different models is warranted., (Copyright © 2022 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Patient-level characteristics associated with survival for single ventricle heart disease following initial staged palliation have been described. However, the impact of peri-operative events on hospital discharge has not been examined. To characterize patient-level characteristics and peri-operative events that were associated with inability to be discharged after Stage 1 palliation (S1P). Analysis of the National Pediatric Cardiology Quality Improvement Collaborative Dataset including patients who underwent a S1P procedure between 2016 and 2019 (Norwood or Hybrid Stage 1 procedure). We examined patient-level characteristics and peri-operative events as possible predictors of inability to discharge after S1P. We constructed multivariate logistic regression models examining post-S1P discharge and in-hospital mortality, adjusting for covariates. 843 patients underwent a S1P and 717 (85%) patients were discharged home or remained inpatient until Stage 2 for social but not medical concerns. Moderate or greater pre-operative atrioventricular valve regurgitation (odds ratio (OR) 4.6, 95% confidence interval (CI) 1.8-12), presence of high-risk pre-operative adverse events (OR 1.5, 95%CI 1.0-2.3), peri-operative events: temporary dialysis (OR 5.4, 95%CI 1.5-18.9), cardiac catheterization or cardiac surgery (OR 2.9, 95%CI 1.8-4.6), sepsis (OR 2.7, 95%CI 1.2-6.2), junctional tachycardia (OR 2.6, 95%CI 1.0-6.3), necrotizing enterocolitis (OR 2.6, 95%CI 1.3-5.2), ECMO (OR 2.5, 95%CI 1.4-4.3), neurological injury (OR 2.1, 95%CI 1.1-4.1), and re-intubation (OR 1.8, 95%CI 1.1-2.9) were associated with inability to discharge after Stage 1. Cardiac anatomical factors, pre-operative adverse events, post-operative re-intubation, post-operative ECMO, infectious complications, and unplanned catheter or surgical re-interventions were associated with inability to discharge after S1P. These findings suggest that quality improvement efforts aimed at reducing these peri-operative events may improve Stage 1 survival and likelihood of discharge., (© 2022. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

Background: Administrative billing data are critical to many initiatives in congenital heart surgery. Mapping algorithms for International Classification of Disease, 10th Revision diagnosis and procedure codes to clinical registry procedure definitions will allow identification of surgical cases and account for patient and procedural factors within administrative data. Our objectives were to develop mapping logic to crosswalk International Classification of Disease, 10th Revision procedure codes to 10 Society of Thoracic Surgeons Congenital Heart Surgery Database benchmark and beta-test the algorithm., Methods: Patients undergoing Society of Thoracic Surgeons Congenital Heart Surgery Database benchmark procedures from 2015 to 2019 were identified and served as the gold standard. Cases were linked on direct identifiers to cases from the Pediatric Health Information System Database. Two independent teams developed International Classification of Disease, 10th Revision-based algorithms for cases capture. Algorithms were compared and iteratively refined to optimize sensitivity and specificity. Operative mortalities for cases identified in the administrative versus registry data were compared., Results: Overall sensitivity was 91% and specificity was 99% for capture of benchmark operations using International Classification of Diseases 10th Revision codes. Sensitivity was more than 90% in identifying 6 of the 10 individual benchmark procedures and more than 98% sensitive in identifying Fontan, Glenn, and arterial switch with ventricular septal defect procedures. Specificity was more than 98% for all benchmark operations. There were no statistical differences in operative mortality between cases identified in the administrative versus the registry data., Conclusions: Novel mapping algorithm for International Classification of Disease, 10th Revision procedure codes enables identification of congenital heart benchmark procedures within administrative billing data. This crosswalk facilitates population-based congenital heart surgical research and quality assessment., (Copyright © 2021 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.)

Introduction: In patients with right ventricular diastolic dysfunction after complete repair of tetralogy of Fallot, some employ the use of beta-blockade. The theoretical benefit of this therapy is felt to be one of the two: 1) reduction in heart rate with subsequent increase in diastolic filling time and stroke volume; 2) halting or reversal of right ventricular remodelling. This study aimed to characterise the use of beta-blockade in paediatric admissions with complete repair of tetralogy of Fallot and characterise the effects of beta-blockade on admission characteristics., Methods: Admissions from 2004 to 2015 in the Pediatric Health Information System database with complete repair of tetralogy of Fallot were identified. Characteristics between admissions with and without beta-blockade were compared by univariate analysis. Next, regression analyses were conducted to determine the independent association of beta-blockade on length of admission, billed charges, cardiac arrest, and inpatient mortality while controlling for demographic variables and comorbidities., Results: A total of 3594 admissions were included in the final analyses. Of these, 371 employed beta-blockade. Admissions with beta-blockade were more likely to have heart failure and tachyarrhythmias. These admissions also tended to be longer by univariate analysis. Regression analyses demonstrated that beta-blockade was independently associated with a 2.8-day increase in length of stay and no statistically significant change in billed charges, cardiac arrest, or inpatient mortality., Conclusions: Beta-blockade after complete repair of tetralogy of Fallot is associated with a longer length of stay but did not statistically significantly impact billed charges, cardiac arrest, or inpatient mortality.

Socioeconomic status (SES) affects a range of health outcomes but has not been extensively explored in the single ventricle population. We investigate the impact of community-level deprivation on morbidity and mortality for infants with single ventricle heart disease in the first year of life. Retrospective cohort analysis of infants enrolled in the National Pediatric Cardiology Improvement Collaborative who underwent staged single ventricle palliation examining mortality and length of stay (LOS) using a community-level deprivation index (DI). 974 patients met inclusion criteria. Overall mortality was 20.5%, with 15.7% of deaths occurring between the first and second palliations. After adjusting for clinical risk factors, the DI was associated with death (log relative hazard [Formula: see text] = 8.92, p = 0.030) and death or transplant (log relative hazard [Formula: see text] = 8.62, p = 0.035) in a non-linear fashion, impacting those near the mean DI. Deprivation was associated with LOS following the first surgical palliation (S1P) (p = 0.031) and overall hospitalization during the first year of life (p = 0.018). For every 0.1 increase in the DI, LOS following S1P increased by 3.35 days (95% confidence interval 0.31-6.38) and total hospitalized days by 5.08 days (95% CI 0.88-9.27). Community deprivation is associated with mortality and LOS for patients with single ventricle congenital heart disease. While patients near the mean DI had a higher hazard of one year mortality compared to those at the extremes of the DI, LOS and DI were linearly associated, demonstrating the complex nature of SES factors., (© 2021. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

Background: Infective endocarditis (IE) is one of the major complications following pulmonary valve replacement (PVR). This analysis hopes to evaluate the incidence, outcomes and possible risk factors of IE associated with trans-catheter and surgical placement of a bovine jugular vein (BJV) graft in the pulmonary position. Methods: In this single-center retrospective study, all records of trans-catheter and surgical PVR from 3/2010 to 12/2019 were reviewed. IE was defined as positive blood cultures, with vegetations seen on echocardiography or sudden increase in peak gradient across the valve or vegetations confirmed at time of valve replacement. Poor dental hygiene:1.dental procedures without S.B.E prophylaxis AND/OR 2.one or more dental cavities, caries, dental abscess. Results: 165 patients had PVR with BJV:107 trans-catheter and 63 surgical. 7%(12/170) of PVRs developed IE(catheter:n = 10, surgery:n = 2) at a median time from valve placement of 38 months. The incidence of IE in the catheter group:3-per-100patient-years and in surgical group:1-per-100patient-years. Multivariate cox regression showed that poor dental hygiene was significantly associated with IE [HR(95% CI):16.9(4.35-66.2)](p value <.001). Kaplan-Meier curves showed a significant difference in freedom from IE between patients with poor and appropriate dental hygiene (p value<.001). Conclusions: There is a 7% incidence of IE with the use of BJV grafts in the pulmonary position at mid-term follow-up. Though the rate in catheter placed BJV seems 3x higher than surgically placed ones, their cohorts are quite different making this comparison flawed. Poor dental hygiene is a strong predictor for post-operative IE and offers a significant opportunity for lowering the rate of infective endocarditis.

Background: Socioeconomic and racial (SER) disparities among patients with congenital heart disease (CHD) may limit access to high-quality care. We characterized the national SER landscape and its relationship to early outcomes and identified interactions among determinants mitigating adverse outcome., Methods: The Pediatric Health Information System (PHIS) database was queried for patients (age <26 years) with CHD between 2016 and 2018. International Classification of Diseases, 10th Revision, codes were mapped to diagnostic categories for complexity adjustment. Correlational and hierarchical regression analyses identified risk factors and characterized interactions., Results: We identified 166,599 unique admissions from 52 hospitals, with 58,395 having interventions. Median age was 0 years (interquartile range [IQR], 4 years). Race/ethnicity was predominantly White (59%), Hispanic (20%), and Black (16%). Median neighborhood household income (NHI) was $41,082 and varied among hospitals. Patient NHI had a parabolic relationship with mortality, with both higher and lower values having increased risk. Black patients had significantly higher death, and this relationship was potentiated by lower NHI and complexity. Hospital length of stay was longer among Black neonates (median, 51 days; IQR, 93 days) compared with neonates of other ethnic groups (median, 32 days; IQR, 71 days; P < .0001. Care pathways, including permanent feeding tubes, were also more prevalent among Black neonates (17.8%) compared with White neonates (15%; P = .02)., Conclusions: Interactions among SER disparities modify CHD outcomes. Specific hospitals have more SER fragile patients but may have developed care pathways that prolong length of stay to mitigate risk among Black neonates. Adverse outcomes among SER-disadvantaged patients are magnified in complex CHD, suggesting tangible benefits to targeted resource allocation and population health initiatives., (Copyright © 2022 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: The impact of complex tracheal surgery (TS) on outcomes of children undergoing cardiac surgery has rarely been investigated in large national databases and could impact anticipated outcomes in those patients., Methods: The Pediatric Health Information System database was reviewed from January 1, 2005 to December 31, 2014, for pediatric (<18 years) patients undergoing select cardiac surgical procedures using International Classification of Disease-9 procedural coding. Patients were divided based upon having TS (excluding tracheostomy) during the same hospitalization. Patients with tracheal and cardiac surgeries (CHS + TS) were propensity matched in a 1 : 2 fashion to patients undergoing isolated cardiac surgeries without TS (CHS) based upon cardiac procedure(s), age at surgery, gender, and discharge year. Hospital mortality and other outcomes were compared between the 2 matched groups., Results: The 283 CHS + TS patients were similar to the 566 CHS patients in gender, race, age, cardiac procedures, and presence of chromosomal abnormalities, all P  > .05. Hospital mortality was 13.8% ( n  = 39) for CHS + TS and 5.8% ( n  = 33) for CHS patients with an unadjusted 2.58 hazard ratio of death (95% confidence interval: 1.59-4.20; P  < .001). CHS + TS was also associated with a greater length of stay (63 days vs 12 days), a higher cost per hospitalization ($322 402 vs $80 273), and more readmissions (64.3% [ n  = 182] vs 41.8% [ n  = 243]), all P  < .001., Conclusions: Patients undergoing cardiac and TS in the same hospitalization are at greater risk of in-hospital mortality than patients undergoing similar cardiac surgeries alone and incur higher resource utilization thereafter. The increased risk of mortality is currently underappreciated, but it is important to recognize when discussing expectations with families and providers.

Background: Previous studies suggest that birth before 39 weeks' gestational age (GA) is associated with higher perioperative mortality and morbidity after congenital heart surgery. The optimal approach to timing of cardiac operation in premature infants remains unclear. We investigated the impact of GA at birth and corrected GA at surgery on postoperative outcomes using the Pediatric Cardiac Critical Care Consortium (PC 4 ) database., Methods: Infants undergoing selected index cardiac operations before the end of the neonatal period were included (n = 2298). GA at birth and corrected GA at the time of the index cardiac operation were used as categorical predictors and fitted as a cubic spline to assess nonlinear relationships. The primary outcome was hospital mortality. Multivariable logistic regression models assessed the association between predictors and outcomes while adjusting for confounders., Results: Late-preterm (34-36 weeks) birth was associated with increased odds of mortality compared with full-term (39-40 weeks) birth, while early-term (37-38 weeks) birth was not associated with increased mortality. Corrected GA at surgery of 34 to 37 weeks compared with 40 to 44 weeks was associated with increased mortality. When analyzing corrected GA at surgery as a continuous predictor of outcome, odds of survival improve as patients approach 39 weeks corrected GA., Conclusions: Contrary to previous literature, we did not find an association between early-term birth and hospital mortality at PC 4 hospitals. Our analysis of the relationship between corrected GA and mortality suggests that operating closer to full-term corrected GA may improve survival., (Copyright © 2021 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

To examine the accuracy between analyzers, the Terumo CDI 500 and the Spectrum Medical Quantum were compared to each other and to the ABL90 FLEX benchtop blood analyzer. Patients were retrospectively identified who underwent cardiac surgery requiring cardiopulmonary bypass between August 1, 2018 and November 1, 2019. Hemoglobin and venous saturation (SvO 2 ) values from all three analyzers were collected. Measurements from the Quantum and the CDI 500 were averaged over 1 minute to provide a single value for the minute for the given device. Blood analysis on the ABL90 benchtop device was performed at a minimum of every hour during congenital cardiopulmonary bypass (CPB). There were 519 patients included in the analysis. Data points numbering 69,404 and 70,598 were analyzed when comparing the CDI 500 to the Quantum for hemoglobin and SvO 2 , respectively. Comparison of hemoglobin and SvO 2 for the CDI 500 and Quantum versus ABL90 used 2283 and 1414 data points respectively, in each group. The CDI 500 and Quantum reported hemoglobin within 1 g/dL of the ABL90 86.9% and 87.5% of the time, respectively. The CDI 500 and Quantum reported SvO 2 within 3% of the ABL90 61.0% and 57.9% of the time, respectively. The mean difference between the CDI 500 and Quantum hemoglobin and SvO 2 measurements equaled .194 g/dL ( p  < .001) and .861% ( p  < .001), respectively and were both significantly different from zero. All device comparisons were statistically significantly different when compared to zero difference, likely due to the large data set as the magnitudes of these differences are all quite small and may not be clinically significant. However, while the reader should judge for themselves based upon their specific practice, in our opinion, the 95% Limit of Agreement was too large for either the CDI 500 or Quantum hemoglobin and SvO 2 values to be substituted for ABL90 values. As recommended by the manufacturers, the CDI 500 and Quantum should only be used as a trending device., (© Copyright 2021 AMSECT.)

Various patch materials with variable cost are used for pulmonary artery reconstruction. An analysis of reintervention based on type of patch material might inform value-based decision making. This was a retrospective review of 214 sites of pulmonary artery reconstruction at a single center from 2000 to 2014. We excluded patients with unifocalization of aortopulmonary collaterals. Primary outcome was reintervention for each type of patch. Total number of patch sites was 214 (180 patients). Median follow-up was 3.7 years. Patch materials and number of sites were branch patch homograft (92), bovine pericardium (44), autologous pericardium (41), and porcine intestinal submucosal patch (37). Median age and weight at the time of patch reconstruction were 12.1 months and 8.5 kg. Reintervention occurred at 34 sites (15.9%). With Cox proportional hazards regression, the following variables were associated with reinterevention: preoperative renal failure - hazard ratio of 4.36 (1.87-10.16), P < 0.001 and weight at surgery - hazard ratio 0.93 (0.89-0.98), P = 0.004. Patch type was not related to reintervention (P = 0.197). Cost per unit patch ranged from $0 (dollars, US) for untreated autologous pericardium to $6,105 for homograft branch patch. In this retrospective analysis, there was no relationship between type of patch used for main or central branch pulmonary artery reconstruction and subsequent reintervention on that site. This finding, combined with the widely disparate costs of patches, may help inform value-based decision making., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Blood-primed cardiopulmonary bypass circuits are frequently necessary to achieve safe support during pediatric open-heart surgery. Literature is lacking regarding suitable prime constituents or methods for achieving a physiologically appropriate blood-primed circuit. We examined the chemistry and hematology of neonatal blood-primed circuits from the conclusion of the priming procedure until the initiation of bypass. Base deficit/excess, pH, pO 2 , pCO 2 , HCO 3 , glucose, sodium, potassium, calcium, hematocrit, lactate, and osmolality were analyzed. Any deviation over time from the original prime value was compared for significance. Statistically significant changes were found between T0 and all time points for all parameters, except for pH and pO 2 out to 1 hour. Among all parameters, various rates of change were observed. Although most changes in the parameters were found to be statistically significant, those changes may not be clinically significant based on clinician interpretation. Attention to the prime quality beyond the immediate post-priming period may be beneficial. Should the time period between validation of the prime quality and initiation of bypass be extended, it may be advisable to reevaluate the prime quality., (© Copyright 2021 AMSECT.)

The optimal training of the highly specialized congenital heart surgeon is a long and complex process, which is a significant challenge in most parts of the world. The World Society for Pediatric and Congenital Heart Surgery (WSPCHS) has established the Global Council on Education for Congenital Heart Surgery as a nonprofit organization with the goal of assessing current training and certification and ultimately establishing standardized criteria for the training, evaluation, and certification of congenital heart surgeons around the world. The Global Council and the WSPCHS have reviewed the present status of training and certification for congenital cardiac surgery around the world. There is currently lack of consensus and standardized criteria for training in congenital heart surgery, with significant disparity between continents and countries. This represents significant obstacles to international job mobility of competent congenital heart surgeons and to the efforts to improve the quality of care for patients with Congenital Heart Disease worldwide. The purpose of this article is to summarize and document the present state of training and certification in congenital heart surgery around the world.

Background: Mortality after stage 1 palliation of hypoplastic left heart syndrome remains significant. Both cardiac output (CO) and systemic vascular resistance (SVR) contribute to hemodynamic vulnerability. Simultaneous measures of mean arterial pressure and somatic regional near infrared spectroscopy saturation can classify complex hemodynamics into 4 distinct states, with a low-CO state of higher risk. We sought to identify interventions associated with low-CO state occupancy and transition., Methods: Perioperative data were prospectively collected in an institutional review board-approved database. Hemodynamic state was classified as high CO, high SVR, low SVR, and low CO using bivariate analysis. Associations of static and dynamic support levels and state classifications over 48 postoperative hours were tested between states and across transitions using mixed regression methods in a quasi-experimental design., Results: Data from 10,272 hours in 214 patients were analyzed. A low-CO state was observed in 142 patients for 1107 hours. Both low CO and extracorporeal membrane oxygenation had increased mortality risk. The low-CO state was characterized by lower milrinone but higher catecholamine dose. Successful transition out of low CO was associated with increased milrinone dose and hemoglobin concentration. Increasing milrinone and hemoglobin levels predicted reduced risk of low CO in future states., Conclusions: Bivariate classification objectively defines hemodynamic states and transitions with distinct support profiles. Maintaining or increasing inodilator and hemoglobin levels were associated with improved hemodynamic conditions and were predictive of successful future transitions from the low-CO state., (Copyright © 2021 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Objectives: Airway anomalies are common in children with cardiac disease but with an unquantified impact on outcomes. We sought to define the association between airway anomalies and tracheal surgery with cardiac surgery outcomes using the Society of Thoracic Surgery Congenital Heart Surgery Database., Methods: Index cardiac operations in children aged less than 18 years (January 2010 to September 2018) were identified from the Society of Thoracic Surgery Congenital Heart Surgery Database. Patients were divided on the basis of reported diagnosis of an airway anomaly and subdivided on the basis of tracheal lesion and tracheal surgery. Multivariable analysis evaluated associations between airway disease and outcomes controlling for covariates from the Society of Thoracic Surgery Congenital Heart Surgery Database Mortality Risk Model., Results: Of 198,674 index cardiovascular operations, 6861 (3.4%) were performed in patients with airway anomalies, including 428 patients (0.2%) who also underwent tracheal operations during the same hospitalization. Patients with airway anomalies underwent more complex cardiac operations (45% vs 36% Society of Thoracic Surgeons/European Association for Cardiothoracic Surgery Congenital Heart Surgery Mortality category ≥3 procedures) and had a higher prevalence of preoperative risk factors (73% vs 39%; both P < .001). In multivariable analysis, patients with airway anomalies had increased odds of major morbidity and tracheostomy (P < .001). Operative mortality was also increased in patients with airway anomalies, except those with malacia. Tracheal surgery within the same hospitalization increased the odds of operative mortality (adjusted odds ratio, 3.9; P < .0001), major morbidity (adjusted odds ratio, 3.7; P < .0001), and tracheostomy (adjusted odds ratio, 16.7; P < .0001)., Conclusions: Patients undergoing cardiac surgery and tracheal surgery are at significantly higher risk of morbidity and mortality than patients receiving cardiac surgery alone. Most of those with unoperated airway anomalies have higher morbidity and mortality, which makes it an important preoperative consideration., (Copyright © 2020 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.)

This article reviews the literature, focusing on publications from the third millennium and the results of mitral valve replacement in children younger than 1 year of age. Special consideration has been given to neonatal and infant valve replacement to provide insights into valve choice and technique. Mitral valve replacement is an important topic because it carries the highest mortality and poorer long-term prognosis than any other valve replacement in children., (Copyright © 2021 Elsevier Inc. All rights reserved.)

The coronavirus disease 2019 (COVID-19) pandemic currently gripping the globe is impacting the entire health care system with rapidly escalating morbidities and mortality. Although the infectious risk to the pediatric population appears low, the effects on children with congenital heart disease (CHD) remain poorly understood. The closure of congenital heart surgery programs worldwide to address the growing number of infected individuals could have an unintended impact on future health for COVID-19-negative patients with CHD. Pediatric and congenital heart surgeons, given their small numbers and close relationships, are uniquely positioned to collectively assess the impact of the pandemic on surgical practice and care of children with CHD. We present the results of an international survey sent to pediatric and congenital heart surgeons characterizing the early impact of COVID-19 on the care of patients with CHD.

The Ross procedure in children, teenagers, and young adults, especially among those with a bicuspid aortic valve with aortic regurgitation, has been associated with neoaortic root dilatation and recurrence of aortic regurgitation. We have shown that at intermediate follow-up, patients who underwent a supported Ross technique were less likely to have neoaortic root dilatation. This study summarizes our most recent outcomes. This was a retrospective review of 40 consecutive pediatric and young adult patients undergoing a supported Ross procedure from 2005 to 2018. Clinical outcomes were reviewed in addition to echocardiographic measures of neoaortic dimension and neoaortic valve function. The median age at surgery was 16.0 years (range 10 - 35 years). Preoperative diagnosis was aortic regurgitation in 15 (37.5%) and mixed regurgitation/stenosis in 20 (50%). Median follow-up was 3.5 years (1.4-5.6) with 3 patients followed for more than 10 years. There were no deaths. Five patients had a reintervention, but only 1 on the aortic valve. One patient returned to the operating room on postoperative day 1 for revision of the right coronary button. Two patients required biventricular pacemakers for reduced ejection fraction. One patient developed aortic regurgitation and underwent mechanical valve replacement and another required a reintervention on the homograft with a percutaneously placed pulmonary valve replacement. At last follow-up, 39 patients had mild or less aortic regurgitation with median sinus z-score of 1.40 (0.48-2.07). Mid-term follow-up of pediatric and young adult patients undergoing a supported Ross operation for various aortic valve pathologies demonstrate excellent results with minimal neoaortic root dilation and reintervention., (Copyright © 2019 Elsevier Inc. All rights reserved.)

As recovery of congenital heart surgery programs begins during this COVID-19 pandemic, we review key considerations such as screening, protection of patients and health care workers (HCWs), case prioritization, barriers to reactivation, redesign of patient care teams, contribution of telemedicine, modification of trainees' experiences, preparation for potential resurgence, and strategies to maintain HCW wellness. COVID-19 has tested the resolve and grit of our specialty and we have an opportunity to emerge more refined.