National Pediatric Cardiology Quality Improvement Collaborative Research Articles

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<0.0001). Using multivariable logistic regression, predictors of MH vs SS1 were lower BW (p<0.001), lower GA (p<0.001), genetic syndrome (p=0.010), non-cardiac anomaly (p=0.031), and ≥4/12 PRF (p<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 >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.

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.

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 and Outcomes of Arrhythmiaon Stage I Palliation of SingleVentricle Patients.

Arrhythmias are common in single ventricle patients though their effect on outcomes during stage I palliation (S1P) is unclear. The authors sought to study associated risks for arrhythmia in patients undergoing S1P for single ventricle disease and evaluate the outcome of arrhythmias and their treatment strategies on survival. Retrospective patient, surgical, medication, and arrhythmia data were obtained from the NPC-QIC (NationalPediatric Cardiology Quality Improvement Collaborative) database. Bivariate analysis of variables associated with arrhythmias, as well as those associated with survival, was performed at the time of stage II palliation. Appropriate variables were included in multivariate modeling. Of the 2,048 patients included in the study, 36% had arrhythmia noted during their S1P hospitalization, with supraventricular tachycardia (12%) and focal atrial tachycardia (11%) the most common. At S1P discharge, 11% of patients were on an antiarrhythmic medication. Arrhythmias were associated with lower survival and increased hospital length of stay. Heterotaxy syndrome, younger age at S1P, male sex, and additional anomalies were associated with increased risk of arrhythmia in multivariable modeling (P≤ 0.01). Arrhythmia and female sex were associated with increased mortality, whereas antiarrhythmic medication and digoxin use were associated with decreased mortality (P≤ 0.003, model area under the curve=0.79). The use of antiarrhythmic medications within the subcohort of arrhythmia patients was also associated with decreased risk of mortality (P&lt; 0.0001; odds ratio: 2.0-7.2). Arrhythmias are common during admission for S1P and associated with poor outcomes. Theuseofantiarrhythmic medications may improve survival, though future studies are needed.

Impact of Digoxin Use on Interstage Outcomes of Single Ventricle Heart Disease (From a NPC-QIC Registry Analysis)

Digoxin has been associated with lower interstage mortality (ISM) following stage 1 palliation (S1P). Despite a substantial increase in digoxin use nationally, ISM has not declined. We aimed to determine the impact of digoxin on ISM in the current era. This study analyzed data from the National Pediatric Cardiology Quality Improvement Collaborative (NPC-QIC) registry. All patients who survived to hospital discharge following S1P were included. Comparisons were made between pre-specified eras (1: 2010-2015, 2: 2016-2019) based on digoxin use. ISM risk was estimated using the previously published NEONATE score (excluding digoxin). Multivariable Cox proportional hazard models assessed the impact of digoxin on ISM and freedom from unplanned readmission in era 2. A total of 1400 (46.8%) patients were included from era 1 and 1589 (53.2%) from era 2. Digoxin use (22.4% vs 61.7%, p < 0.001) and the proportion of high-risk patients (9.1% vs 20.3%, p < 0.001) increased across eras. There was no difference in predicted ISM risk between those who did vs did not receive digoxin in era 2 (p=0.82). In era 2, digoxin use was independently associated with lower ISM (AHR 0.60, 95%CI 0.36 to 0.98, p=0.043) and greater freedom from unplanned readmission (AHR 0.44, 95%CI 0.32 - 0.59, p < 0.001). In conclusion, digoxin is independently associated with lower ISM and greater freedom from interstage readmission. The lack of improvement in overall ISM in the current era may be secondary to a greater proportion of high-risk patients and/or disproportionately higher digoxin use in lower risk patients, who may not derive the same benefit.

Changes in Provider Prescribing Behavior for Infants with Single Ventricle Physiology After Evidence-Based Publications.

The impact of published evidence on clinical practice has been understudied in pediatric cardiology. We sought to assess changes in prescribing behavior for angiotensin-converting enzyme inhibitor (ACEI) and digoxin at discharge after initial palliation of infants with single ventricle (SV) physiology following the publication of two large studies: The Pediatric Heart Network Infant Single Ventricle (PHN-ISV) trial showing no benefit with routine ACEI use and the National Pediatric Cardiology Quality Improvement Collaborative (NPC-QIC) analysis showing an association between digoxin and survival. ICD-9-10 codes identified SV infants from the Pediatric Health Information System (1/2004 to 1/2018) and charge codes identified medications at discharge. Generalized estimating equations implementing segmented logistic regressions modeled medication use, before and after (with a 3-month washout period) the relevant publication (ACEI 7/1/2010; digoxin 4/1/2016). A subgroup analysis was performed for hypoplastic left heart syndrome (HLHS). ACEI use (37 centers, n = 4700) at discharge did not change over time during the pre-publication period. After publication of the PHN-ISV trial, ACEI use decreased (OR: 0.61, CI 0.44-0.84, p = 0.003). Digoxin use (43 centers, n = 4778) decreased by 1% monthly before publication. After the NPC-QIC publication, digoxin use increased (OR: 2.07, CI 1.05-4.08, p = 0.04) with an ongoing increase of 9% per month. Results were similar for the HLHS subgroup. Prescribing behavior changed congruently after the publication of evidence-based studies, with decreased ACEI use and increased digoxin use at discharge following initial palliation of SV infants. Our findings suggest scientific findings were rapidly implemented into clinical practice.

Abstract 13342: Increasing Use of the Right Ventricle to Pulmonary Artery Shunt for Stage 1 Palliation: A Report From the National Pediatric Cardiology Quality Improvement Collaborative

Introduction: Stage 1 palliation (S1P) of single ventricle heart defects with aortic hypoplasia consists of either the Norwood operation with a modified Blalock-Taussig shunt (MBTS) or a right ventricle-to-pulmonary artery shunt (RVPAS), or a hybrid procedure. Changes in national trends over time and factors influencing surgical approach remain unclear. Hypothesis: There has been an increase in national use of the RVPAS for S1P. Methods: Data from the National Pediatric Cardiology Quality Improvement Collaborative (NPC-QIC) phase 1 (6/2008-8/2016) and phase 2 (8/2016-9/2019) databases were used. S1P type was evaluated by year of operation. Factors influencing the choice of MBTS versus RVPAS, as well as length of stay after S1P and mortality prior to Stage 2 palliation (S2P) between shunt types, were evaluated. Sites were stratified by the number of patients in the database per year as small (&lt;5), medium (&lt;10) and large (≥10) centers. Results: The combined database included 3335 eligible patients; 1,028 (30.8%) with MBTS, 1,989 (59.7%) with RVPAS, and 318 (9.5%) with hybrid procedure. Overall, of 62 centers, 14.6% of S1P were at small centers (n=26), 40.6% at medium centers (n=24), and 44.7% at large centers (n=12). There was an increase in RVPAS use over time (p=0.02). In multivariable analysis, increasing hospital volume (OR 1.2 [95% CI 1.1-1.4], p=0.003) and absence of other organ system anomalies (OR 1.5 [95% CI 1.0-2.2], p=0.049) were associated with MBTS use over RVPAS. Median length of stay after S1P with MBTS was longer than with RVPAS (31 [95% CI 20-49] vs 29 [95% CI 19-47] days, p=0.054) and mortality was higher prior to S2P (12.3% vs 9.6%, p=0.03). Conclusions: Use of RVPAS with S1P has increased over time with a decrease in MBTS use and unchanged hybrid frequency. MBTS is used more commonly in centers with higher volume and in patients without other anomalies but is associated with longer post-op hospitalization and lower transplant-free survival to stage 2 palliation.

Abstract 14689: Optimal Prenatal Care Coordination is Associated With Fewer Pre-operative Risk Factors in Babies With a Prenatal Diagnosis of HLHS

Introduction: Prenatal diagnosis (PD) of congenital heart disease permits coordination of care to optimize postnatal outcomes. Using phase II registry data from the National Pediatric Cardiology Quality Improvement Collaborative (NPC-QIC), we sought to identify associations between prenatal care coordination (PNC) and pre-operative status among infants with HLHS and variants intended for Stage I Norwood or hybrid palliation. Hypothesis: We hypothesized that suboptimal PNC would be associated with worse pre-operative status. Methods: The NPC-QIC database was queried for all instances of prenatally diagnosed HLHS and variants intended for Stage 1 Norwood or hybrid palliation from 2016 through 2019. Demographic and clinical data pertaining to the prenatal period through the first birthday were obtained. Optimal PNC was defined as (1) a completed interdisciplinary case conference prior to delivery and (2) a note in the medical record documenting closed-loop communication between the OB and cardiology teams. Associations between PNC and outcomes were identified. Results: Among 1441 registry patients with HLHS and variants, 1242 (86%) had PD. Among patients with PD, optimal PNC was achieved in 68%. Preoperatively, infants without optimal PNC were more likely to have any adverse event (50% vs 40%, p&lt;0.001), receive inotropes (19% vs 13%, p=0.007), require mechanical ventilation (22% vs 16%, p=0.016), and were less likely to feed enterally (53% vs 67%, p &lt;0.001). Lower socio-economic status (SES) was associated with a lower likelihood of PD (p&lt;0.001), and African-American race was associated with a lower likelihood of optimal PNC (p=0.006). Conclusions: Among prenatally diagnosed infants with HLHS and variants, optimal PNC was associated with fewer preoperative risk factors. There also appear to be disparities in PD related to SES and in PNC related to race. Additional study is required to understand the complex interplay between PNC and clinical outcomes, as well as identify and address barriers to PD and optimal PNC.

Abstract 14975: Increased Morbidity and Mortality in Patients With Hypoplastic Left Heart Syndrome and Genetic Abnormalities

Introduction: Hypoplastic left heart syndrome (HLHS) is a genetically heterogenous, severe form of congenital heart disease. Hypothesis: Patients with reported genetic abnormalities will have worse outcomes than those without reported genetic abnormalities. Methods: This was a retrospective review of patients enrolled in the National Pediatric Cardiology Quality Improvement Collaborative (NPC-QIC) phase I registry. Registry enrollment occurred at discharge following stage 1 palliation. Patients were placed into one of three groups: major syndromes (Turner, Down, CHARGE, DiGeorge, Jacobsen, VATER, heterotaxy), other genetic abnormality, or no reported genetic abnormality. Demographic and clinical variables were compared using Pearson Chi-square, one-way ANOVA, or Kruskal-Wallis test. Tukey post-hoc test was applied to adjust p-values for pairwise group comparisons. Outcomes of length of stay, death, and combined outcomes of death, not a candidate for stage 2 palliation and transplant were compared among the groups. Results: Of the 2182 patients, major syndromes were reported in 110 (5%), other genetic abnormalities in 126 (5.8%) and no abnormalities in 1946 (89.2%). Those with major syndromes weighed less at birth and the time of stage I palliation, were more likely to be female and have a primary cardiac diagnosis of unbalanced AVC. Those with major syndromes or other genetic abnormalities were more likely to have moderate to severe AV valve regurgitation, moderate to severe ventricular dysfunction, arrhythmia requiring therapy, and major anomalies of other organ systems compared to those with no abnormalities. Patients with no reported genetic abnormalities reached full oral feeds sooner after the Norwood (19 vs 24 days), were more likely to be discharged on all oral feeds (75% vs 59% and 62%) and were discharged earlier (35 vs 45 and 43 days). For the outcome of death, there was no significant difference among the groups. The combined outcome of death, not a candidate for stage 2 palliation, and heart transplant was more likely in those with a major syndrome after adjusting for covariates. Conclusions: Patients with HLHS and major syndromes or other genetic abnormalities experience greater morbidity and mortality during the interstage period.

Improved National Outcomes Achieved in a Cardiac Learning Health Collaborative Based on Early Performance Level

Within the National Pediatric Cardiology Quality Improvement Collaborative (NPC-QIC), a learning health network developed to improve outcomes for patients with hypoplastic left heart syndrome and variants, we assessed which centers contributed to reductions in mortality and growth failure. Centers within the NPC-QIC were divided into tertiles based on early performance for mortality and separately for growth failure. These groups were evaluated for improvement from the early to late time period and compared with the other groups in the late time period. Mortality was 3.8% for the high-performing, 7.6% for the medium-performing, and 14.4% for the low-performing groups in the early time period. Only the low-performing group had a significant change (P<.001) from the early to late period. In the late period, there was no difference in mortality between the high- (5.7%), medium- (7%), and low- (4.6%) performing centers (P=.5). Growth failure occurred in 13.9% for the high-performing, 21.9% for the medium-performing, and 32.8% for the low-performing groups in the early time period. Only the low-performing group had a significant change (P<.001) over time. In the late period, there was no significant difference in growth failure between the high- (19.8%), medium- (21.5%), and low- (13.5%) performing groups (P=.054). Improvements in the NPC-QIC mortality and growth measures are primarily driven by improvement in those performing the worst in these areas initially without compromising the success of high-performing centers. Focus for improvement may vary by center based on performance.

Angiotensin-Converting Enzyme Inhibitor Prescription for Patients With Single Ventricle Physiology Enrolled in the NPC-QIC Registry.

BackgroundThe routine use of angiotensin‐converting enzyme inhibitors (ACEI) during palliation of hypoplastic left heart syndrome is controversial. We sought to describe ACEI prescription in the interstage between stage 1 palliation (stage I Norwood procedure) discharge and stage 2 palliation (stage II superior cavopulmonary anastomosis procedure) admission using the NPC‐QIC (National Pediatric Cardiology Quality Improvement Collaborative) registry.Methods and ResultsAnalysis of all patients (n=2180) enrolled in NPC‐QIC from 2008 to 2016 included preoperative anatomy, risk factors, and echocardiographic data. ACEI were prescribed at stage I Norwood procedure discharge in 38% of patients. ACEI prescription declined from 2011 to 2016 compared with pre‐2010 (36.8% versus 45%; P=0.005) with significant variation across centers (range 7–100%; P<0.001) and decreased prescribing rates associated with increased center volume (P=0.004). There was no difference in interstage mortality (P=0.662), change in atrioventricular valve regurgitation (P=0.101), or change in ventricular dysfunction (P=0.134) between groups. In multivariable analysis of all patients, atrioventricular septal defect (odds ratio [OR], 1.84; 95% CI, 1.28–2.65) or double outlet right ventricle (OR, 1.47; CI, 1.02–2.11), and preoperative mechanical ventilation (OR, 1.37; 95% CI, 1.12–1.68) were associated with increased ACEI prescription. In multivariable analysis of patients with complete echocardiographic data (n=812), ACEI prescription was more common with at least moderate atrioventricular valve regurgitation (OR, 1.88; 95% CI, 1.22–2.31).ConclusionsACEI prescription remains common in the interstage despite limited evidence of benefit. ACEI prescription is associated with preoperative mechanical ventilation, double outlet right ventricle, and atrioventricular valve regurgitation with marked inter‐center variation. ACEI prescription is not associated with reduction in mortality, ventricular dysfunction, or atrioventricular valve regurgitation during the interstage.

FACTORS ASSOCIATED WITH CHANGES IN MORTALITY OVER TIME AND HOME DEATHS IN PHASE 1 OF THE NATIONAL PEDIATRIC CARDIOLOGY QUALITY IMPROVEMENT COLLABORATIVE

Quality improvement (QI) efforts in the National Pediatric Cardiology Quality Improvement Collaborative (NPC-QIC) decreased interstage (hospital discharge post-stage 1 to stage 2 palliation) mortality in infants with hypoplastic left heart syndrome and variants from 9.5% to 5.3% starting in June

Power of a Learning Network in Congenital Heart Disease.

The National Pediatric Cardiology Quality Improvement Collaborative (NPC-QIC) formed to improve outcomes in infants with hypoplastic left heart syndrome. The collaborative sought to (1) decrease mortality, (2) reduce growth failure, and (3) reduce hospital readmissions due to major medical problems during the interstage period between discharge following stage 1 palliation (S1P) and admission for stage 2 palliation (S2P). The NPC-QIC is a learning network, coproduced by parents and clinicians, of 65 pediatric cardiology centers that contribute clinical data on care processes and outcomes to a shared registry. The adapted Breakthrough Series Model structure brings teams together regularly to review data, share lessons, and plan improvements. Outcomes are monitored using statistical process control methods. Between 2008 and 2016, interstage mortality decreased by >40%, from 9.5% to 5.3%. Identification and use of a nutrition bundle led to improved infant growth, with a 28% reduction in interstage growth failure. The rate of serious hospital readmissions was low and did not significantly change. Importantly, a formed partnership with the parent group Sisters by Heart fostered the coproduction of tools and strategies and an emphasis on data transparency and outcomes. The NPC-QIC's initial efforts led to improvements in interstage growth and mortality. The NPC-QIC has modeled the use of data for improvement and research, the value of coproduction with parents, and the concept "all teach, all learn," demonstrating the power of the learning network model.

IMPACT Registry and National Pediatric Cardiology Quality Improvement Collaborative: Contributions to Quality in Congenital Heart Disease.

The IMproving Pediatric and Adult Congenital Treatments (IMPACT) Registry and the National Pediatric Cardiology Quality Improvement Collaborative (NPC-QIC) are two efforts initiated to improve outcomes in the congenital heart disease field. The IMPACT Registry is focused on evaluating the use, risks, adverse events (AEs), and outcomes associated with diagnostic and common interventional catheterization procedures in all children and adults with congenital heart disease. Utilizing a modular approach, the common procedures include diagnostic cardiac catheterization, atrial septal defect device closure, patent ductus arteriosus device closure, pulmonary valvuloplasty, aortic valvuloplasty, balloon and stent angioplasty of coarctation of the aorta, pulmonary artery balloon stent angioplasty, transcatheter pulmonary valve replacement, and electrophysiology procedures including radiofrequency ablation. To date, important observations on the common procedures have been made and a risk stratification methodology has been created to allow comparisons between centers in AEs and quality improvement activity. The registry is open to international participation. The NPC-QIC was developed to reduce mortality and improve the quality of life of infants with Hypoplastic Left Heart Syndrome (HLHS) during the interstage period between discharge from the Norwood operation and admission for the bidirectional Glenn procedure. Mortality in the interstage has been reduced by 44%. The IMPACT Registry and the NPC-QIC have demonstrated value to the congenital heart disease community. The IMPACT Registry, however, has not yet demonstrated an impact on patient outcomes. The NPC-QIC, which combines both a registry with a learning collaborative with specific aims, key drivers, and change strategies, has made more significant gains with reductions in variation, growth failures, and mortality.

Reintervention following stage 1 palliation: A report from the NPC-QIC Registry.

Single ventricle heart disease with aortic arch hypoplasia has high morbidity and mortality, with the greatest risk after stage 1 palliation. Residual lesions often require catheter-based or surgical reintervention to minimize risk. We sought to describe the types, frequency, and risk factors for re-intervention between stage 1 and stage 2 palliation, utilizing the National Pediatric Cardiology Quality Improvement Collaborative (NPC-QIC) registry. The NPC-QIC registry, consisting of patients discharged after stage 1 palliation, was queried. Hybrid stage 1 palliation patients were excluded from this study. The primary risk factor was shunt type and the primary outcome was re-intervention. Of 1156 patients, (50%) had re-intervention. There was no difference in total rate of re-intervention by shunt type (BT shunt 52% vs. RVPA shunt 48%; P=.17). Patients with a BT shunt had increased re-intervention during stage 1 hospitalization (P=.002). During the interstage period, following discharge from stage 1 palliation, patients with a BT shunt had increased aortic arch re-intervention (P<.005), while patients with an RVPA shunt had increased re-intervention on the shunt and the pulmonary arteries (P=.02). Postoperative mechanical ventilation >14 d (P<.01) was the only risk factor associated with re-intervention by multivariable analysis, regardless of shunt type. Re-intervention between stage I and stage 2 palliation is common. There is no difference in cumulative frequency of re-intervention between shunt types, though types and timing of re-intervention varied between shunt types. Longitudinal assessment of the NPC-QIC database is important to identify long term outcomes of patients requiring re-intervention.

The Norwood Stage 1 procedure - conduct of perfusion: 2017 Survey results from NPC-QIC member institutions.

Hypoplastic left heart syndrome (HLHS) is a rare and severe congenital cardiac defect. Approximately 1000 infants are born with HLHS in the United States every year. Healthcare collaboratives over the last decade have focused on sharing patient experiences and techniques in an effort to improve outcomes. In 2010, cardiologists and patient families joined together to improve the care of HLHS patients by forming the National Pediatric Cardiology Quality Improvement Collaborative (NPC-QIC). Sixty-six of the approximately 110 institutions caring for patients with HLHS in the United States and Canada are now members of NPC-QIC. In 2017, cardiovascular perfusionists joined the collaborative as another specialty involved in the care of HLHS patients. Perfusionists and cardiac surgeons developed the collaborative's first conduct of perfusion survey for the Norwood Stage 1 procedure, specifically targeting the provision of cardiopulmonary bypass for patients with HLHS. This manuscript discusses the results of this survey, unveiling a significant variance in the conduct of perfusion for this patient population.

Practice trends over time in the care of infants with hypoplastic left heart syndrome: A report from the National Pediatric Cardiology Quality Improvement Collaborative.

The National Pediatric Cardiology Quality Improvement Collaborative (NPC-QIC) was established in 2008 to improve outcomes of hypoplastic left heart syndrome (HLHS) during the interstage period. They evaluated changes in patient variables and practice variation between early and late eras. Data including demographic, operative, discharge, and follow-up variables from the first 100 patients (6/2008-1/2010) representing 18 centers were compared with the most recent 100 patients (1/2014-11/2014) from these same centers. Prenatal diagnosis increased from 69% to 82% (P = .05). There were no differences in gestational age or weight at Norwood. A composite of any preoperative risk factor occurred more frequently in the early era (59% vs. 34%, P < .01). While mean age at Norwood was similar (8.3 vs. 6.6 days, P = .2), the standard deviation was significantly lower in the recent era (10.4-6.4 days, P = .04). Use of RV-PA conduit increased (67%-84%, P < .01). Rates of complete discharge communication with both the primary care physician (31%-97%, P < .01) and primary cardiologist (44%-97%, P < .01) increased substantially. There were limited changes in feeding strategies. Use of home monitoring program increased (76%-99%, P < .01) with all participants in the late era monitoring both oxygen saturation and weight. Among NPC-QIC centers contributing patients to both eras, there were significant changes in preoperative risk factors, surgical strategy, discharge communication, and interstage care. Further study is required to determine an association between these changes and decreased mortality.

Abstract 13524: Examining Variation in Interstage Mortality Rates Across the National Pediatric Cardiology Quality Improvement Collaborative: Do Lower Mortality Centers Have Lower Risk Patients?

Introduction: Although overall interstage mortality for infants with hypoplastic left heart syndrome has declined within the National Pediatric Cardiology Quality Improvement Collaborative (NPC-QIC), variation in outcomes across centers persists. It remains unclear whether centers with lower mortality rates have lower risk interstage patients or whether differences in the type of interstage care provided may explain this variation. We examined previously established patient risk factors across NPC-QIC centers with higher and lower interstage mortality rates. Methods: Lower mortality NPC-QIC centers were defined as those with &gt;25 consecutive interstage survivors, and included 7 centers. Higher mortality centers were defined as those with cumulative interstage mortality rates greater than the NPC-QIC historic baseline rate (&gt;10%), and included 4 centers. Patient risk factors were compared between lower and higher mortality centers, accounting for within center clustering. Results: The 7 lower mortality centers (n=331 patients) had a lower interstage mortality rate of 2.7% compared to 13.3% in the 4 higher mortality centers (n=173 patients); p&lt;0.0001. Post-natal diagnosis was the only risk factor less prevalent in the lower mortality centers (Figure) compared to higher mortality centers (18.4% vs. 31.8%, p=0.04). All other risk factors examined were either similar between the lower and higher mortality centers (including gestational age, anatomic diagnoses, birth weight), or more prevalent in the lower mortality centers (major syndrome- 6.9% vs. 4.0%, p=0.03; major anomalies- 7.3% vs. 4%, p=0.047). Conclusions: Differences in interstage mortality rates among NPC-QIC centers do not appear to be explained by a lower rate of patient risk factors in lower mortality centers. Further study is necessary to evaluate differences in interstage care practices across centers in order to identify targets for efforts to reduce variation and improve outcomes.

Digoxin Use Is Associated With Reduced Interstage Mortality in Patients With No History of Arrhythmia After Stage I Palliation for Single Ventricle Heart Disease.

BackgroundInterstage mortality (IM) remains significant after stage 1 palliation (S1P) for single‐ventricle heart disease (SVD), with many deaths sudden and unexpected. We sought to determine whether digoxin use post‐S1P is associated with reduced IM, utilizing the multicenter database of the National Pediatric Cardiology Quality Improvement Collaborative (NPCQIC).Methods and ResultsFrom June 2008 to July 2013, 816 infants discharged after S1P from 50 surgical sites completed the interstage to stage II palliation, transplant, or IM. Arrhythmia during S1P hospitalization or discharge on antiarrhythmic medications were exclusions (n=270); 2 patients were lost to follow‐up. Two analyses were performed: (1) propensity‐score adjusted logistic regression with IM as outcome and (2) retrospective cohort analysis for patients discharged on digoxin versus not, matched for surgical site and other established IM risk factors. Of 544 study patients, 119 (21.9%) were discharged on digoxin. Logistic regression analysis with propensity score, site‐size group, and digoxin use as predictor variables showed an increased risk of IM in those not discharged on digoxin (odds ratio, 8.6; lower confidence limit, 1.9; upper confidence limit, 38.3; P<0.01). The retrospective cohort analysis for 60 patients on digoxin (matched for site of care, type of S1P, post‐S1P ECMO use, genetic syndrome, discharge feeding route, ventricular function, tricuspid regurgitation, and aortic arch gradient) showed 0% IM in the digoxin at discharge group and an estimated IM difference between the 2 groups of 9% (P=0.04).ConclusionsAmong SVD infants in the NPCQIC database discharged post‐S1P with no history of arrhythmia, use of digoxin at discharge was associated with reduced IM.

Abstract 14020: Practice Trends in the Care of Infants With Hypoplastic Left Heart Syndrome: A Report From the National Pediatric Cardiology Quality Improvement Collaborative (NPC-QIC)

Introduction: The NPC-QIC was established in 2008 to improve outcomes of hypoplastic left heart syndrome (HLHS) patients during the interstage period using quality improvement to reduce unnecessary practice variation. Our objective was to evaluate early and recent interstage practice variation within the collaborative. Methods: Data from the first 100 patients (6/2008 - 1/2010) representing 18 centers were compared with the most recent 100 patients (1/2014 - 11/2014) from these same centers. Results: Prenatal diagnosis increased from 72% to 82% (p = 0.09). There were no differences in gestational age or weight at Norwood. A composite of any preoperative risk factor (mechanical ventilation, acidosis, renal insufficiency, arrhythmia, NEC, sepsis) occurred more frequently in the early era (58% vs 34%, p &lt;0.01). While mean age at Norwood was similar (8.3 vs 6.6 days, p = 0.2), the standard deviation for this variable was significantly lower in the recent era (10.5 to 6.4 days, p = 0.03). Use of RV-PA conduit increased (69% to 84%, p = 0.02). Rates of complete discharge communication with both the primary care physician (31 to 97%, p &lt; 0.01) and primary cardiologist (44 to 97%, p &lt; 0.01) increased substantially. There was no change in interstage feeding strategies between eras. Use of interstage outpatient surveillance program increased (76% to 99%, p &lt; 0.01) with all participants in the late era monitoring both oxygen saturation and weight. Conclusions: Among NPC-QIC centers contributing patients to both early and recent cohorts, there were significant changes in preoperative risk factors, Norwood surgical strategy, discharge communication, and interstage surveillance. Practice variation in timing of Norwood has been reduced. Further study is required to determine an association between these practice trends and decreased mortality.