National Pediatric Cardiology Quality Improvement Research Articles

Risk Factors for Death or Transplant After Stage 2 Palliation for Single Ventricle Heart Disease

BackgroundFor infants with single ventricle heart disease, the time after stage 2 procedure (S2P) is believed to be a lower risk period compared with the interstage period; however, significant morbidity and mortality still occur. ObjectivesThis study aimed to identify risk factors for mortality or transplantation referral between S2P surgery and the first birthday. MethodsRetrospective cohort analysis of infants in the National Pediatric Cardiology Quality Improvement Collaborative who underwent staged single ventricle palliation from 2016 to 2022 and survived to S2P. Multivariable logistic regression and classification and regression trees were performed to identify risk factors for mortality and transplantation referral after S2P. ResultsOf the 1,455 patients in the cohort who survived to S2P, 5.2% died and 2.3% were referred for transplant. Overall event rates at 30 and 100 days after S2P were 2% and 5%, respectively. Independent risk factors for mortality and transplantation referral included the presence of a known genetic syndrome, shunt type at stage 1 procedure (S1P), tricuspid valve repair at S1P, longer time to extubation and reintubation after S1P, ≥ moderate tricuspid regurgitation prior to S2P, younger age at S2P, and the risk groups identified in the classification and regression tree analysis (extracorporeal membrane oxygenation after S1P and longer S2P cardiopulmonary bypass time without extracorporeal membrane oxygenation). ConclusionsMortality and transplantation referral rates after S2P to 1 year of age remain high ∼7%. Many of the identified risk factors after S2P are similar to those established for interstage factors around the S1P, whereas others may be unique to the period after S2P.

The NEONATE score predicts freedom from interstage mortality or transplant in a modern cohort.

Derived from the National Pediatric Cardiology Quality Improvement Collaborative registry, the NEONATE risk score predicted freedom from interstage mortality or heart transplant for patients with single ventricle CHD and aortic arch hypoplasia discharged home following Stage 1 palliation. We sought to validate the score in an external, modern cohort. This was a retrospective cohort analysis of single ventricle CHD and aortic arch hypoplasia patients enrolled in the National Pediatric Cardiology Quality Improvement Collaborative Phase II registry from 2016 to 2020, who were discharged home after Stage 1 palliation. Points were allocated per the NEONATE score (Norwood type-Norwood/Blalock-Taussig shunt: 3, Hybrid: 12; extracorporeal membrane oxygenation post-op: 9, Opiates at discharge: 6, No Digoxin at discharge: 9, Arch Obstruction on discharge echo: 9, Tricuspid regurgitation ≥ moderate on discharge echo: 12; Extra oxygen plus ≥ moderate tricuspid regurgitation: 28). The composite primary endpoint was interstage mortality or heart transplant. In total, 1026 patients met inclusion criteria; 61 (6%) met the primary outcome. Interstage mortality occurred in 44 (4.3%) patients at a median of 129 (IQR 62,195) days, and 17 (1.7%) were referred for heart transplant at a 167 (114,199) days of life. The median NEONATE score was 0(0,9) in those who survived to Stage 2 palliation compared to 9(0,15) in those who experienced interstage mortality or heart transplant (p < 0.001). Applying a NEONATE score cut-off of 17 points that separated patients into low- and high-risk groups in the learning cohort provided 91% specificity, negative predictive value of 95%, and overall accuracy of 87% (85.4-89.5%). In a modern cohort of patients with single ventricle CHD and aortic arch hypoplasia, the NEONATE score remains useful at discharge post-Stage 1 palliation to predict freedom from interstage mortality or heart transplant.

Abstract 17152: Use and Outcomes of the Medical Hybrid Procedure for Stage 1 Palliation in Infants With Hypoplastic Left Heart Syndrome and Variants

Background: Staged palliation of hypoplastic left heart syndrome (HLHS) and variants typically begins with the Norwood operation or the hybrid procedure. Hybrid palliation is used in 13% of patients nationally and is often reserved for high-risk patients. A stented hybrid (SH) consists of bilateral pulmonary artery bands (bPAB) and a ductal stent while a ‘medical hybrid’ (MH) consists of bPAB and prostaglandins. MH use and outcomes have not been well described. Hypothesis: MH is used in higher-risk patients and therefore has lower survival than other stage 1 strategies. Aims: Compare use and outcomes of MH, SH, and surgical stage 1 (SS1) using a multicenter database. Methods: Patients from the National Pediatric Cardiology Quality Improvement Collaborative (NPC-QIC) database were categorized by initial intervention: MH, SH, or SS1. Other/unclear procedures were excluded. Results: The study included 2423 patients from 65 centers: 277 (11%) MH, 133 (5%) SH, 2013 (83%) SS1. MH had the lowest birth weight (BW) and gestational age (GA), most non-cardiac anomalies, and most preoperative risk factors (PRF). Most centers performed MH in a minority of cases, though use ranged from 0% to 82% at centers with ≥10 patients. Transplant-free survival at 1 year was 56% for MH, 66% for SH, and 81% for SS1 (p&lt;0.0001). Using multivariable logistic regression, predictors of MH vs SS1 were lower BW (p&lt;0.001), lower GA (p&lt;0.001), genetic syndrome (p=0.010), non-cardiac anomaly (p=0.031), and ≥4/12 PRF (p&lt;0.001). Predictors of MH vs SH were HLHS (p=0.044) and ≥4/12 PRF (p=0.045). Cox proportional hazards regression was used to compare risk-adjusted outcomes, excluding one center that performed &gt;50% SH: MH had higher risk of one-year mortality/transplant compared to SS1 (HR = 1.89, 95% CI 1.47-2.42) and no difference compared to SH (HR = 1.16, 95% CI 0.76-1.79). Conclusions: Survival after MH is similar to SH and worse than SS1. This may be due to patient risk factors not controlled for in this study.

Abstract 12166: Social Determinants of Health and Acute Interstage Outcomes: An NPC-QIC Analysis

Introduction: The interstage period after Stage 1 palliation (S1P) carries high morbidity and mortality. The impact of social determinants of health (SDOH) on acute interstage outcomes has not been well described. Question: How do SDOH affect interstage outcomes? Methods: Infants discharged after S1P in the National Pediatric Cardiology Quality Improvement Collaborative Phase 2 registry were analyzed. ZIP code-level Childhood Opportunity Index (COI), a composite metric incorporating 29 indicators, was used to classify patients into 5 COI levels. Birth and S1P characteristics were compared. The association between COI level and binary acute interstage outcomes was assessed using log-rank test and logistic regression with and without adjustment for race/ethnicity, interstage risk factors, and interstage duration. Results: There were 1837 patients from 68 centers. There were differences across COI levels in birthweight, proximity to a surgical center, area of residence, age and weight at S1P, S1P length of stay (LOS), and feeding mode at discharge ( Table ). Over 98% of patients in all COI levels had home monitoring. Death or transplant occurred in 101 (5%) patients with unplanned readmission in 987 (53%), ICU admission in 448 (24%), unplanned catheterizations in 345 (19%), and reoperation in 83 (5%). COI level was not associated with differences in interstage outcomes on logistic regression or log-rank test ( Figure) . Conclusions: COI level is associated with differences in pre-operative status, feeding mode, and S1P LOS, but not acute interstage outcomes. The role of home monitoring programs in protecting against interstage disparities merits further investigation.

Abstract 14634: Hybrid vs. Norwood: Analysis of the NPC-QIC Database to Determine Optimal Approach for High-Risk Single Ventricle Patients

Background: Infants w/ high risk hypoplastic left heart syndrome and variants (HR-HLHS) due to low birth weight or gestational age experience worse outcomes. Optimal management remains unknown. We sought to 1. compare primary Norwood to hybrid strategies in HR-HLHS 2. identify predictors of futility. Hypothesis: Primary Norwood offers improved outcomes compared to hybrid strategies. Very low birth weight and other anomalies may represent futility. Methods: We reviewed HR-HLHS from the National Pediatric Cardiology Quality Improvement Collaborative database. Patients with birth weight &lt;2.5kg or gestation &lt;35 weeks, and age ≤ 30 days at admission were included (N=398). Norwood (n=225), hybrid (pulmonary artery band (PAB) + ductal stent, n=76), and PAB w/ prostaglandin (PAB/PGE, n=77) were compared. Transplantation referral (n=1) and comfort care (n=19) occurred. Baseline factors, 1-year survival, and stage 2 completion were reviewed. Results: Table shows baseline HR-HLHS features. Norwood had higher risk adjusted 1-year survival and stage 2 completion than hybrid strategies, including in &lt; 2.1 kg (Figure). On multivariable analysis, hybrid (HR 2.8), genetic abnormality (HR 1.5), and ECMO (HR: 7.2) were significantly associated w/ decreased 1-year survival and stage 2 completion, while higher birth weight (HR: 1.5) was associated w/ increased stage 2 completion. Less than 25% of HR-HLHS w/ birthweight &lt; 2.1 kg and ≥ 1 genetic abnormality was alive with stage 2 at the end of follow-up. Conclusions: HR-HLHS have better outcomes following primary Norwood than hybrid palliation. In patients w/ birth weight &lt; 2.1 kg and genetic abnormalities, hybrid or Norwood may be futile.

Association of Digoxin Use With Transplant-Free Interstage Survival in Infants Palliated With a Stage 1 Hybrid Procedure.

Background Digoxin prescription in patients with single-ventricle physiology after stage 1 palliation is associated with reduced interstage death. Prior literature has primarily included patients having undergone the Norwood procedure. We sought to determine if digoxin prescription at discharge in infants following hybrid stage 1 palliation was associated with improved transplant-free interstage survival. Methods and Results A retrospective multicenter cohort analysis was conducted using data from the National Pediatric Cardiology Quality Improvement Collaborative registry data from 2008 to 2021. Infants with functional single ventricles and aortic arch obstruction discharged home after the hybrid stage 1 palliation hospitalization were included. Patients were excluded if they had supraventricular tachycardia or conversion to Norwood operation. The primary outcome was transplant-free survival. Multivariable logistic regression analysis including a propensity score for digoxin use identified associations between digoxin use and interstage death or transplant. Of 259 included infants from 45 sites, 158 (61%) had hypoplastic left heart syndrome. Forty-nine percent had a gestational age ≤38 weeks, 18% had a birth weight <2.5 kg, and 58% had a preoperative risk factor. Of the 259 subjects, 129 (50%) were discharged on digoxin. Interstage death or transplant occurred in 30 (23%) patients in the no-digoxin group compared with 18 (14%) in the digoxin group (P=0.06). With multivariate analysis, discharge digoxin prescription was associated with a lower risk of interstage death or transplant (adjusted odds ratio, 0.48 [95% CI, 0.24-0.93]; P=0.03). Conclusions In infants with single-ventricle physiology who underwent hybrid stage 1 palliation, digoxin prescription at hospital discharge was associated with improved interstage transplant-free survival.

Human Milk Feeding and Direct Breastfeeding Improve Outcomes for Infants With Single Ventricle Congenital Heart Disease: Propensity Score-Matched Analysis of the NPC-QIC Registry.

Background Infants with single ventricle congenital heart disease undergo 3 staged surgeries/interventions, with risk for morbidity and mortality. We estimated the effect of human milk (HM) and direct breastfeeding on outcomes including necrotizing enterocolitis, infection-related complications, length of stay, and mortality. Methods and Results We analyzed the National Pediatric Cardiology Quality Improvement Collaborative (NPC-QIC) registry (2016-2021), examining HM/breastfeeding groups during stage 1 and stage 2 palliations. We calculated propensity scores for feeding exposures, then fitted Poisson and logistic regression models to compare outcomes between propensity-matched cohorts. Participants included 2491 infants (68 sites). Estimates for all outcomes were better in HM/breastfeeding groups. Infants fed exclusive HM before stage 1 palliation (S1P)had lower odds of preoperative necrotizing enterocolitis (odds ratio [OR], 0.37 [95% CI, 0.17-0.84]; P=0.017) and shorter S1P length of stay (rate ratio [RR], 0.87 [95% CI, 0.78-0.98]; P=0.027). During the S1P hospitalization, infants with high HM had lower odds of postoperative necrotizing enterocolitis (OR, 0.28 [95% CI, 0.15-0.50]; P<0.001) and sepsis (OR, 0.29 [95% CI, 0.13-0.65]; P=0.003), and shorter S1P length of stay (RR, 0.75 [95% CI, 0.66-0.86]; P<0.001). At stage 2 palliation, infants with any HM (RR, 0.82 [95% CI, 0.69-0.97]; P=0.018) and any breastfeeding (RR, 0.71 [95% CI, 0.57-0.89]; P=0.003) experienced shorter length of stay. Conclusions Infants with single ventricle congenital heart disease in high-HM and breastfeeding groups experienced multiple significantly better outcomes. Given our findings of improved health, strategies to increase the rates of HM/breastfeeding in these patients should be implemented. Future research should replicate these findings with granular feeding data and in broader congenital heart disease populations, and should examine mechanisms (eg, HM components, microbiome) by which HM/breastfeeding benefits these infants.

Center-level factors associated with shorter length of stay following stage 1 palliation: An analysis of the national pediatric cardiology quality improvement collaborative registry

Stage 1 single ventricle palliation (S1P) has the longest length of stay (LOS) of all benchmark congenital heart operations. Center-level factors contributing to prolonged hospitalization are poorly defined. We analyzed data from infants status post S1P included in the National Pediatric Cardiology Quality Improvement Collaborative Phase II registry. Our primary outcome was patient-level LOS with days alive and out of hospital before stage 2 palliation (S2P) used as a balancing measure. We compared patient and center-level characteristics across quartiles for median center LOS, and used multivariable regression to calculate center-level factors associated with LOS after adjusting for case mix. Of 2,510 infants (65 sites), 2037 (47 sites) met study criteria (61% male, 61% white, 72% hypoplastic left heart syndrome). There was wide intercenter variation in LOS (first quartile centers: median 28 days [IQR 19, 46]; fourth quartile: 62 days [35, 95], P < .001). Mortality prior to S2P did not differ across quartiles. Shorter LOS correlated with more pre-S2P days alive and out of hospital, after accounting for readmissions (correlation coefficient -0.48, P < .001). In multivariable analysis, increased use of Norwood with a right ventricle to pulmonary artery conduit (aOR 2.65 [1.1, 6.37]), shorter bypass time (aOR 0.99 per minute [0.98,1.0]), fewer additional cardiac operations (aOR 0.46 [0.22, 0.93]), and increased use of NG tubes rather than G tubes (aOR 7.03 [1.95, 25.42]) were all associated with shorter LOS centers. Modifiable center-level practices may be targets to standardize practice and reduce overall LOS across centers.

Assessing enrollment of eligible infants in the national pediatric cardiology quality improvement collaborative (NPC-QIC) through linkage to the pediatric cardiac critical care consortium (PC4) registry.

The National Pediatric Cardiology Quality Improvement Collaborative (NPC-QIC) lacks a rigorous enrollment audit process, unlike other collaborative networks. Most centers require individual families to consent to participate. It is unknown whether there is variation across centers or biases in enrollment. We used the Pediatric Cardiac Critical Care Consortium (PC4) registry to assess enrollment rates in NPC-QIC for those centers participating in both registries using indirect identifiers (date of birth, date of admission, gender, and center) to match patient records. All infants born 1/1/2018-12/31/2020 and admitted 30 days of life were eligible. In PC4, all infants with a fundamental diagnosis of hypoplastic left heart or variant or who underwent a surgical or hybrid Norwood or variant were eligible. Standard descriptive statistics were used to describe the cohort and center match rates were plotted on a funnel chart. Of 898 eligible NPC-QIC patients, 841 were linked to 1,114 eligible PC4 patients (match rate 75.5%) in 32 centers. Match rates were lower in patients of Hispanic/Latino ethnicity (66.1%, p = 0.005), and those with any specified chromosomal abnormality (57.4%, p = 0.002), noncardiac abnormality (67.8%, p = 0.005), or any specified syndrome (66.5%, p = 0.001). Match rates were lower for patients who transferred to another hospital or died prior to discharge. Match rates varied from 0 to 100% across centers. It is feasible to match patients between the NPC-QIC and PC4 registries. Variation in match rates suggests opportunities for improvement in NPC-QIC patient enrollment.

Predictors of Human Milk Feeding and Direct Breastfeeding for Infants with Single Ventricle Congenital Heart Disease: Machine Learning Analysis of the National Pediatric Cardiology Quality Improvement Collaborative Registry

To identify factors that support or limit human milk (HM) feeding and direct breastfeeding (BF) for infants with single ventricle (SV) congenital heart disease (CHD) at neonatal stage 1 palliation (S1P) discharge and at stage 2 palliation (S2P) (∼4-6 months old). Analysis of the National Pediatric Cardiology Quality Improvement Collaborative registry (2016-2021; 67 sites). Primary outcomes were any HM, exclusive HM, and any direct BF at S1P discharge and at S2P. The main analysis involved multiple phases of elastic net logistic regression on imputed data to identify important predictors. For 1944 infants, the strongest predictor domain areas included preoperative feeding, demographics/social determinants of health (SDoH), feeding route, clinical course, and site. Significant findings included: preoperative BF was associated with any HM at S1P discharge (OR=2.02, 95% CI=1.74-3.44) and any BF at S2P (2.29, 1.38-3.80); private/self-insurance was associated with any HM at S1P discharge (1.91, 1.58-2.47); Black/African-American infants had lower odds of any HM at S1P discharge (0.54, 0.38-0.65) and at S2P (0.57, 0.30-0.86). Adjusted odds of HM/BF practices varied among NPC-QIC sites. Preoperative feeding practices predict later HM and BF for infants with SV CHD; therefore, family-centered interventions focused on HM/BF during the S1P preoperative time are needed. These interventions should include evidence-based strategies to address implicit bias and seek to minimize disparities related to SDoH. Future research is needed to identify supportive practices common to high-performing NPC-QIC sites.

Patterns of Breastfeeding and Human Milk Feeding in Infants with Single-Ventricle Congenital Heart Disease: A Population Study of the National Pediatric Cardiology Quality Improvement Collaborative Registry.

Introduction: Infants with single-ventricle (SV) congenital heart disease (CHD) undergo staged surgical and/or catheter-based palliation and commonly experience feeding challenges and poor growth. Little is known about human milk (HM) feeding or direct breastfeeding (BF) in this population. Aim: To determine (1) HM and BF prevalence for infants with SV CHD, and (2) whether BF at neonatal stage 1 palliation (S1P) discharge is associated with any HM at stage 2 palliation (S2P; ∼4-6 months old). Materials and Methods: Analysis of the National Pediatric Cardiology Quality Improvement Collaborative registry (2016-2021) using (1) descriptive statistics for prevalence, and (2) logistic regression adjusted for multiple variables (e.g., prematurity, insurance, length of stay) to examine early BF/later HM feeding. Results: Participants included 2,491 infants from 68 sites. HM prevalence ranged from 49.3% any/41.5% exclusive before S1P to 37.1% any/7.0% exclusive at S2P. Direct BF ranged from 16.1% any/7.9% exclusive before S1P to 9.2% any/3.2% exclusive at S2P discharge. Prevalence varied among sites; for example, 0-100% any HM before S1P. Infants BF at S1P discharge had greater odds of any HM (odds ratio = 4.11, 95% confidence interval [CI] = 2.79-6.07, p < 0.001) and exclusive HM (1.85, 95% CI 1.03-3.30, p = 0.039) at S2P. Conclusions: The prevalence of HM and BF for infants with SV CHD was low and declined over time. Direct BF at S1P discharge was associated with increased odds of any HM at S2P. Wide variation suggests that site-specific practices impact feeding outcomes. HM and BF prevalence are suboptimal in this population, and identification of supportive institutional practices is needed.

Machine Learning to Predict Interstage Mortality Following Single Ventricle Palliation: A NPC-QIC Database Analysis.

There is high risk of mortality between stage I and stage II palliation of single ventricle heart disease. This study aimed to leverage advanced machine learning algorithms to optimize risk-prediction models and identify features most predictive of interstage mortality. This study utilized retrospective data from the National Pediatric Cardiology Quality Improvement Collaborative and included all patients who underwent stage I palliation and survived to hospital discharge (2008-2019). Multiple machine learning models were evaluated, including logistic regression, random forest, gradient boosting trees, extreme gradient boost trees, and light gradient boosting machines. A total of 3267 patients were included with 208 (6.4%) interstage deaths. Machine learning models were trained on 180 clinical features. Digoxin use at discharge was the most influential factor resulting in a lower risk of interstage mortality (p < 0.0001). Stage I surgery with Blalock-Taussig-Thomas shunt portended higher risk than Sano conduit (7.8% vs 4.4%, p = 0.0002). Non-modifiable risk factors identified with increased risk of interstage mortality included female sex, lower gestational age, and lower birth weight. Post-operative risk factors included the requirement of unplanned catheterization and more severe atrioventricular valve insufficiency at discharge. Light gradient boosting machines demonstrated the best performance with an area under the receiver operative characteristic curve of 0.642. Advanced machine learning algorithms highlight a number of modifiable and non-modifiable risk factors for interstage mortality following stage I palliation. However, model performance remains modest, suggesting the presence of unmeasured confounders that contribute to interstage risk.

Abstract 10283: Socioeconomic Disparities in Overall and Morbidity Free Survival of Infants With Single Ventricle Heart Disease

Prior studies have demonstrated higher odds of mortality for non-white and lower socioeconomic status patients with single ventricle (SV) heart disease. However, these studies have not examined survival through staged surgical palliation or rates of morbidity in this patient population. We hypothesized that non-white race/ethnicity and lower socioeconomic status were associated with lower overall survival and increased morbidity through 1 st birthday in patients undergoing SV palliation. Patients with SV heart disease, live birth, and planned SV palliation in the National Pediatric Cardiology Quality Improvement Collaborative between January 1, 2016, and December 31, 2020, were included. Race/ethnicity, payer, and neighborhood characteristics were analyzed. Morbidity was defined as acute neurological events, unplanned procedures, renal support, cardiac arrest, extracorporeal life support, necrotizing enterocolitis, or surgical site infection. Morbidity-free survival was defined as survival through each stage of palliation without experiencing one of the above morbidities. Cox proportional hazards and multivariable regression modeling were performed to evaluate the association between socioeconomic variables, race/ethnicity, and overall morbidity-free survival to 1st birthday. There were 2,184 live births with an overall survival to 1st birthday of 76%. There was no difference in survival to 1st birthday between non-Hispanic White, non-Hispanic Black, and Hispanic patients. Patients with government insurance had decreased survival prior to their 1st birthday (HR 0.79, 95% CI 0.64, 0.98 vs commercial insurance). Infants with government insurance had lower morbidity-free survival during stage 1 (OR 0.72, 95% CI 0.59, 0.87) and stage 2 (OR 0.67, 95% CI 0.52, 0.85) hospitalizations. Patients with “Other” race/ethnicity had lower odds of morbidity-free survival to one year of age (OR 0.68 95% CI 0.47, 0.98). Household income and deprivation index were not associated with survival or morbidity. Infants with government insurance experienced decreased survival to 1st birthday, and those of “Other” race/ethnicity had lower morbidity-free survival. Addressing disparities in these patient populations is an area of ongoing need.

Abstract 10294: Center Level Factors Associated With Improved Length of Stay Following Stage 1 Palliation: An Analysis of the NPC-QIC Registry

Introduction: Stage 1 single ventricle palliation has the longest associated length of stay (LOS) of STS benchmark operations. Patient level factors that contribute to prolonged hospitalization have been identified, but center level factors have not been explored. We aim to identify center level factors that drive post-Stage 1 palliation LOS. Methods: We analyzed data prospectively collected by the National Pediatric Cardiology Quality Improvement Collaborative Phase II registry. We included all infants who underwent stage 1 followed by either stage 2 or death. We excluded centers with fewer than 10 cases and centers or patients with missing key variables. Our primary outcome was center level LOS. We grouped centers into quartiles based on median LOS for all patients at each center and compared patient and center level characteristics between highest and lowest performing quartiles using univariable analyses. Results: Of 2510 infants (65 sites), 1831 (30 sites) met study criteria (61% male, 61% white, 17% premature, 73% with hypoplastic left heart as primary cardiac diagnosis). There was wide inter-center variation in LOS (1st quartile centers: median 28 days [IQR 19, 47]; 4th quartile: 62 days [35, 96], p&lt;0.001). Shorter LOS correlated with more outside hospital days alive prior to stage 2, after accounting for readmissions (correlation coefficient -0.49, p&lt;0.001). Compared to 7 low performing centers, 8 high performing centers were more likely to perform the Norwood with an RV-PA conduit (64% v 48%, p&lt;0.001), extubate patients earlier (10 days [7, 15] v 14 days [11, 25], p&lt;0.001), start enteral feeds sooner (9 days [7, 13] v 13 days [10, 22], p&lt;0.001), and were more likely to discharge patients on nasogastric feeds (57% v 19%, p&lt;0.001) vs G-tube feeds (7% v 41%, p&lt;0.001) (Table 1). Conclusions: There was significant inter-center variation in post-Stage 1 LOS. Modifiable center-level practices may be targets to standardize practice and reduce overall LOS across centers.

Abstract 11135: Association of Digoxin Use With Transplant-Free Survival in Infants Palliated With a Stage I Hybrid Procedure

Introduction: Digoxin use in patients with single ventricle physiology after stage I palliation (S1P) has been associated with reduced interstage mortality (IM). However, the S1P hybrid procedure comprised a small fraction of the patients in these evaluations. As it is being performed with increasing frequency, data regarding digoxin effects on IM after this procedure are needed. We sought to determine if digoxin prescription at discharge in infants with the S1P hybrid was associated with improved transplant-free interstage survival. Methods: National Pediatric Cardiology Quality Improvement Collaborative registry data from 2008 to 2021 were utilized. Infants discharged home after the S1P hybrid hospitalization were included. Patients were excluded if they had 1) supraventricular tachycardia, 2) death during S1P hospitalization, 3) conversion to Norwood during S1P hospitalization, or 4) remained hospitalized until stage II surgery. The primary outcome was transplant-free survival. Chi-square, Fisher’s Exact, Mann-Whitney U tests and multivariable logistic regression were used to compare patients receiving and not receiving interstage digoxin following S1P hybrid. Results: Of the 259 patients from 45 sites with S1P hybrid, 129 (50%) were discharged home on digoxin. Baseline characteristics including birth weight, gestational age at birth, race/ethnicity, cardiac diagnosis, presence of aortic atresia, noncardiac abnormalities or genetic syndromes, and one or more pre-operative risk factors were similar between groups. IM or heart transplant occurred in 30 (23%) patients in the no digoxin group compared to 18 (14%) in the digoxin group (P=0.06). With multivariate logistic regression analysis adjusting for home feeding route, degree of right ventricular dysfunction, atrioventricular valve regurgitation and oxygen saturation at discharge, home digoxin prescription was independently associated with a lower risk of IM or transplant (adjusted OR 0.49; 95% CI 0.25-0.98; P=0.04). Conclusions: In patients with single ventricle physiology who underwent a S1P hybrid procedure, digoxin prescription at hospital discharge was associated with improved transplant-free survival during the interstage period.

Impact of Reintervention During Stage 1 Palliation Hospitalization: A National, Multicenter Study

BackgroundStage 1 palliation (S1P) for hypoplastic left heart syndrome remains associated with high morbidity and mortality. Previous studies on burden of reinterventions did not include patients who remain hospitalized before stage 2 palliation (S2P). This study described the rate of reintervention during S1P hospitalization and sought to determine the impact of reintervention on outcomes. MethodsAll participants enrolled in phase II of the National Pediatric Cardiology Quality Improvement Collaborative after S1P were included in this study. The primary outcome was the rate of reintervention during hospitalization after S1P and before hospital discharge or S2P. Reintervention was defined as 1 or more unplanned interventional cardiac catheterizations or surgical reoperations. ResultsBetween March 1, 2016 and October 1, 2019, 1367 participants underwent S1P and 339 (24.8%) had a reintervention; most commonly to address the source of pulmonary blood flow. Gestational age, weight at S1P, atrioventricular septal defect, heterotaxy, preoperative pulmonary artery bands, hybrid S1P, and an additional bypass run or early extracorporeal membrane oxygenation were significantly associated with reintervention. Participants in the reintervention group experienced higher rates of nearly all postoperative complications, were less likely to be discharged before S2P (57.1% vs 86%; P < .001), and more likely to experience in-hospital mortality (17% vs 5%; P < .001). ConclusionsUnplanned reintervention during hospitalization after S1P palliation occurred in 25% of participants in a large, registry-based national cohort. Participants who underwent reintervention were more likely to remain as inpatient and were less likely to survive to S2P. Reintervention was associated with a multitude of postoperative complications that affect survival and long-term outcome.

Feeding challenges in the newborn with congenital heart disease.

Feeding challenges and growth failure are lifelong issues for infants with congenital heart disease. The purpose of this review is to summarize the literature on the topic from North America. Despite recognition of feeding challenges and ongoing national collaboration, >50% of infants with univentricular physiology continue to require supplemental tube feeds at the time of discharge from neonatal surgery. Preoperative feeding is now commonly used in prostaglandin dependent neonates with congenital heart disease. The value of a structured nutritional program with establishment of best practices in nutrition is well recognized in the current era. Despite implementation of these best practices, neonates undergoing cardiac surgery continue to struggle with weight gain prior to discharge. This suggests that there is more to growth than provision of adequate nutrition alone. The National Pediatric Cardiology Quality Improvement Collaborative continues to play a major role in optimizing nutrition in infants with congenital heart disease. This among other registries underscores the importance of collaboration in improving overall outcomes for children with congenital heart disease. Nurses should be encouraged to lead both clinical and research efforts to overcome feeding challenges encountered by these children.

The Prevalence of Congenital Anomalies of the Airway or Lung in Infants with Hypoplastic Left Heart Syndrome and Differences in Midterm Outcomes: A National Pediatric Cardiology Quality Improvement Collaborative Registry Analysis.

Infants with single ventricle physiology and congenital anomalies of the airway and/or lung are potentially poor candidates for staged palliation. The prevalence and midterm outcomes for patients with anomalies of the airway or lung with hypoplastic left heart syndrome are unknown. We performed an analysis of data in infants with hypoplastic left heart syndrome from the National Pediatric Cardiology Quality Improvement Collaborative registry. The prevalence of congenital anomalies of the airway or lung in the registry was determined. Clinical characteristics and midterm outcomes were compared between infants with hypoplastic left heart syndrome with and without anomalies of the airway or lung. Fifty-seven (2.3%) of 2467 infants with hypoplastic left heart syndrome enrolled in the registry had congenital airway or lung anomalies. Infants congenital anomalies of the airway or lung had significantly lower transplant-free survival at 1year (49.5 vs 77.2%, p < 0.001). Infants with airway or lung anomalies had longer combined hospital length of stay for stage 1 and stage 2 palliation (102 vs 65.1days, p < 0.001) and underwent more major procedures (2.04 vs 0.93 procedures, p < 0.001) than those without. There was no difference in the number of interstage readmissions (1.85 vs 1.89, p = 0.87) or need for non-oral feeding supplementation (71.4 vs 54.5%, p = 0.12) between groups. Infants enrolled in the National Pediatric Cardiology Quality Improvement Collaborative registry with hypoplastic left heart syndrome and anomalies of the airway or lung have increased morbidity and mortality at 1year compared to those with hypoplastic left heart syndrome alone.

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

ObjectivePrematurity, 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. MethodsThe 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. ResultsOf 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. ConclusionsThe 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.

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.