Recommendations for Centers Performing Pediatric Heart Surgery in the United States.

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.

Recommendations for centers performing pediatric heart surgery in the United States.

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.

Recommendations for Centers Performing Pediatric Heart Surgery in the United States.

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.

Contemporary Relationship Between Hospital Volume and Outcomes in Congenital Heart Surgery.

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.

Intramyocardial cell-based therapy with Lomecel-B during bidirectional cavopulmonary anastomosis for hypoplastic left heart syndrome: the ELPIS phase I trial.

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.

Defining Expectations for Infants With Hypoplastic Left Heart Syndrome Who Survive Initial Surgical Palliation.

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.

Mathematical analysis of hemoglobin target in univentricular parallel circulation.

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/m2/min, oxygen consumption of 150 mL/min/m2, and a Qp/Qs ∼ 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 âˆ¼ 9 L/m2/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.

Targeting the contact system in a rabbit model of extracorporeal membrane oxygenation.

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.

Cumulative comorbid conditions influence mortality risk after staged palliation for hypoplastic left heart syndrome and variants.

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.

The Supported vs Unsupported Ross in Pediatric Patients: Neoaortic Root and Ventricular Function.

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.

A Murine Model of Veno-Arterial Extracorporeal Membrane Oxygenation.

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.

Infants Who Require Total Parenteral Nutrition and Paralytics at Time of Heart Transplant Experience Inferior Post-Transplant Mortality.

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.

Factors Associated with Inability to Discharge After Stage 1 Palliation for Single Ventricle Heart Disease: An Analysis of the National Pediatric Cardiology Quality Improvement Collaborative Database.

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.

Endocarditis in Bovine Vein Grafts in the Pulmonary Position Placed Surgically & Percutaneously.

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.

Mortality Prediction After Cardiac Surgery in Children: An STS Congenital Heart Surgery Database Analysis.

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.

Reevaluating Congenital Heart Surgery Center Performance Using Operative Mortality.

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.

Management of Neonates Admitted With Tetralogy of Fallot: Changing Patterns Across the United States.

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.

Widening our Focus: Characterizing Socioeconomic and Racial Disparities in Congenital Heart Disease.

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.

Digoxin Associated With Greater Transplant-Free Survival in High- vs Low-Risk Interstage Patients.

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.