National Pediatric Cardiology Quality Improvement Research Articles

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.

Assessing the Association Between Pre-operative Feeding and the Development of Oral Feeding Skills in Infants with Single Ventricle Heart Disease: An Analysis of the NPC-QIC Dataset.

Pre-operative feeding may improve long-term feeding outcomes in single ventricle patients, including weaning from supplemental tube feedings in infancy. This study examines the association between pre-operative enteral feeding and subsequent long-term feeding outcomes while also assessing the counterbalancing risk of necrotizing enterocolitis (NEC). Secondary analysis of the National Pediatric Cardiology Quality Improvement Collaborative database was performed. The association between pre-operative feeding practice and achieving all oral feeds through the first year of life was examined using a multivariable regression model. Similarly, the association between pre-operative oral feeding and NEC was also assessed. Of 944 patients with 1-year feeding outcomes available, 58% were fed preoperatively (41.3% exclusively oral) and 12.3% were not fed per institutional approach. At hospital discharge after Stage 1 palliation, 57% required a feeding tube, while 39% required a feeding tube at their first birthday. In infants who were orally fed, the odds ratio to achieving tube-free feeding at 1year was not significantly increased (1.3, confidence interval 0.8-2.0). Of 1740 infants with pre-operative feeding and Stage 1 there was no statistically significant difference in NEC among patients who were preoperatively fed versus those that were not fed per institutional approach (p = 0.2). Pre-operative feeding of infants with single ventricle heart disease was not associated with early achievement of tube-free feeding in the first year of life. However, pre-operative oral feeding was also not associated with increased risk of NEC, suggesting that it can be safely offered among appropriate patients.

Increased interstage morbidity and mortality following stage 1 palliation in patients with genetic abnormalities.

Hypoplastic left heart syndrome and single ventricle variants with aortic hypoplasia are commonly classified as severe forms of CHD. We hypothesised patients with these severe defects and reported genetic abnormalities have increased morbidity and mortality during the interstage period. This was a retrospective review of the National Pediatric Cardiology Quality Improvement Collaborative Phase I registry. Three patient groups were identified: major syndromes, other genetic abnormalities, and no reported genetic abnormality. Tukey post hoc test was applied for pairwise group comparisons of length of stay, death, and combined outcome of death, not a candidate for stage 2 palliation, and heart transplant. Participating centres received a survey to establish genetic testing and reporting practices. Of the 2182 patients, 110 (5%) had major genetic syndromes, 126 (6%) had other genetic abnormalities, and 1946 (89%) had no genetic abnormality. Those with major genetic syndromes weighed less at birth and stage 1 palliation. Patients with no reported genetic abnormalities reached full oral feeds sooner and discharged earlier. The combined outcome of death, not a candidate for stage 2 palliation, and heart transplant was more common in those with major syndromes. Survey response was low (n = 23, 38%) with only 14 (61%) routinely performing and reporting genetic testing. Patients with genetic abnormalities experienced greater morbidity and mortality during the interstage period than those with no reported genetic abnormalities. Genetic testing and reporting practices vary significantly between participating centres.

Increasing Use of the Right Ventricle-to-Pulmonary Artery Shunt for Stage 1 Palliation

BackgroundStage 1 palliation of hypoplastic left heart syndrome entails use of the Norwood operation with a modified Blalock-Taussig shunt or a right ventricle-to-pulmonary artery shunt, or the hybrid procedure. Use trends and factors influencing palliation selection remain unclear. We aimed to evaluate these questions and to compare outcomes between types of stage 1 palliation. MethodsThe National Pediatric Cardiology Quality Improvement Collaborative phase 1 (June 2008-August 2016) and phase 2 (August 2016-September 2019) databases were used. Procedure type was assessed by operation year. Baseline characteristics and annual hospital volume were evaluated. Postsurgical admission duration and outcomes were compared. ResultsA total of 3497 patients were included, 30.8% with modified Blalock-Taussig shunt, 59.7% with right ventricle-to-pulmonary artery shunt, and 9.5% with hybrid. Use of the right ventricle-to-pulmonary artery shunt increased over time (P = .02). This increase was similar among all hospital volumes. Higher hospital volume (odds ratio [OR], 1.2; 95% CI, 1.1-1.4; P = .003), male sex (OR, 1.3; 95% CI, 1.1-1.6; P = .01), and isolated cardiac disease (OR, 1.33; 95% CI, 1.01-1.55; P = .05) were associated with relatively higher likelihoods of a modified Blalock-Taussig shunt. Mortality/transplant rates before stage 2 palliation were higher with the modified Blalock-Taussig shunt than with the right ventricle-to-pulmonary artery shunt (12.3% vs 9.6%, P = .03). ConclusionsIn stage 1 palliation, use of right ventricle-to-pulmonary artery shunts has increased over time, use of modified Blalock-Taussig shunts has decreased, and use of hybrids was unchanged. The modified Blalock-Taussig shunt has a higher likelihood of use in higher-volume centers, males, and less complex patients but is associated with longer hospitalizations and lower transplant-free survival to stage 2 palliation.

Predictors and outcomes of heart block during surgical stage I palliation of patients with a single ventricle: A report from the NPC-QIC.

Mortality in cohorts with a single ventricle remains high with multiple associated factors. The effect of heart block during stage I palliation remains unclear. The purpose of this study was to study patient and surgical risks of heart block and its effect on 12-month transplant-free survival in patients with a single ventricle. Patient, surgical, outcome data and heart block status (transient and permanent) were obtained from the National Pediatric Cardiology Quality Improvement Collaborative single ventricle database. Bivariate analysis was performed comparing patients with and without heart block, and multivariate modeling was used to identify variables associated with block. One-year outcomes were analyzed to identify variables associated with lower 12-month transplant-free survival. In total, 1423 patients were identified, of whom 28 (2%) developed heart block (second degree or complete) during their surgical admission. Associated risk factors for block included heterotaxy syndrome (odds ratio [OR] 6.4) and atrial flutter/fibrillation (OR 3.8). Patients with heart block had lower 12-month survival, though only in patients with complete heart block as opposed to second degree block. At 12 months of age, 43% (12/28) of patients with heart block died and were more likely to experience mortality at 12 months than patients without block (OR 4.9; 95% confidence interval 1.4-17.5; P = .01). Although rare, complete heart block after stage I palliation represents an additional risk of poor outcomes in this high-risk patient population. Heterotaxy syndrome was the most significant risk factor for the development of heart block after stage I palliation. The role of transient block in outcomes and potential rescue with long-term pacing remains unknown and requires additional study.

Socioeconomic Impact on Outcomes During the First Year of Life of Patients with Single Ventricle Heart Disease: An Analysis of the National Pediatric Cardiology Quality Improvement Collaborative Registry.

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

Birth Location in Infants with Prenatally Diagnosed Hypoplastic Left Heart Syndrome.

There are conflicting data on how delivery location impacts outcomes in neonates with ductal-dependent heart disease. Our goal was to evaluate the impact of delivery location on hospital length of stay and survival in infants with prenatally diagnosed hypoplastic left heart syndrome (HLHS) after stage 1 palliation (S1P). A multicenter cohort study was performed utilizing the National Pediatric Cardiology Quality Improvement Collaborative dataset for infants with prenatally diagnosed HLHS who underwent S1P from August 2016 to December 2018. Univariate comparisons of demographics, clinical, and outcome data were made and multivariable logistic regression was performed between groups stratified by distance from surgical center. A total of 790 patients from 33 centers were analyzed: 85% were born < 5 miles from the surgical center with 72% of those (486/673) born at the surgical center. Infants born < 5 miles from the surgical center were significantly (p < 0.05) more likely to be male, white, full term, have no non-cardiac anomaly, and have commercial health insurance; they were significantly more likely to breastfeed pre-operatively, and less likely to have pre-operative cardiac catheterizations, pre-operative mechanical ventilation, or delayed surgery. There was no significant difference between groups in hospital length of stay, 30-day survival, or survival to hospital discharge. In this multicenter dataset, hospital length of stay and survival after S1P did not differ based on distance from birth location to surgical center. However, neonates born < 5 miles from the surgical center had lower rates of potentially modifiable pre-operative risk factors including mechanical ventilation and delays to surgery.

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.

Characteristics of Interstage Death After Discharge from Stage I Palliation.

Interstage mortality (IM) remains high for patients with single-ventricle congenital heart disease (SVCHD) in the period between Stage 1 Palliation (S1P) and Glenn operation. We sought to characterize IM. This was a descriptive analysis of 2184 patients with SVCHD discharged home after S1P from 60 National Pediatric Cardiology Quality Improvement Collaborative sites between 2008 and 2015. Patients underwent S1P with right ventricle-pulmonary artery conduit (RVPAC), modified Blalock-Taussig-Thomas shunt (BTT), or Hybrid; transplants were excluded. IM occurred in 153 (7%) patients (median gestational age 38weeks, 54% male, 77% white), at 88 (IQR 60,136) days of life, and 39 (IQR 17,84) days after hospital discharge; 13 (8.6%) occurred ≤ 30days after S1P. The mortality rate for RVPAC was lower (5.2%; 59/1138) than BTT (9.1%; 65/712) and Hybrid (20.1%; 27/134). More than half of deaths occurred at home (20%) or in the emergency department (33%). The remainder occurred while inpatient at center of S1P (cardiac intensive care unit 36%, inpatient ward 5%) or at a different center (5%). Fussiness and breathing problems were most often cited as harbingers of death; distance to surgical center was the biggest barrier cited to seeking care. Cause of death was unknown in 44% of cases overall; in the subset of patients who underwent post-mortem autopsy, the cause of death remained unknown in 30% of patients, with the most common diagnosis being low cardiac output. Most IM occurred in the outpatient setting, with non-specific preceding symptoms and unknown cause of death. These data indicate the need for research to identify occult causes of death, including arrhythmia.

Poor Weight Recovery Between Stage 1 Palliation and Hospital Discharge for Infants with Single Ventricle Physiology: An Analysis of the National Pediatric Cardiology Quality Improvement Collaborative Phase II Dataset

To investigate change in weight-for-age z-scores (WAZ) and risk factors for impaired weight gain between stage 1 palliation (S1P) for single ventricle physiology and discharge. This was a secondary analysis of the National Pediatric Cardiology Quality Improvement Collaborative Phase II database. The primary outcome was change in WAZ between S1P and discharge. Risk factors were selected using multivariable mixed effects regression constructed by step-wise model selection, with adjustment for WAZ at S1P and a random effect for center. Of 730 infants who were discharged after S1P, WAZ decreased in 98.6% (-1.5±0.7). WAZ at discharge was <-1 but >-2 (at risk) in 40% and <-2 (failure to thrive) in 35% of participants. Males, higher WAZ at S1P, non-S1P procedures (mostly noncardiac), increased length of stay, necrotizing enterocolitis, and angiotensin-converting enzyme inhibitor use at discharge were associated with a greater decrease in WAZ. Preoperative enteral feeding and respiratory medications were associated with a lesser decrease in the WAZ. Nearly all infants lose weight after S1P with little recovery by hospital discharge. At discharge, three-quarters of the infants in the cohort were at risk for impaired weight gain or had failure to thrive. Most risk factors associated with change in WAZ were unmodifiable or surrogates of disease severity. Novel interventions are needed to minimize the early catabolic effects and promote anabolic recovery after S1P.

Birth Location and Timing of Surgical Center ICU Admission in Infants with Prenatally Diagnosed Hypoplastic Left Heart Syndrome: A Report from the National Pediatric Cardiology Quality Improvement Collaborative

Birth Location and Timing of Surgical Center ICU Admission in Infants with Prenatally Diagnosed Hypoplastic Left Heart Syndrome: A Report from the National Pediatric Cardiology Quality Improvement Collaborative

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 17146: Late Gestation Fetal Growth in Infants Undergoing Stage 1 Palliation for Single Ventricle Heart Disease

Introduction: Ideal timing for elective delivery of infants with single ventricle heart disease, balancing benefits of fetal growth with risk of fetal demise, remains unclear. Improved understanding of fetal growth in late gestation and postnatal outcomes related to gestational age (GA) may improve insight. Hypothesis: Patients with single ventricle heart disease and aortic hypoplasia have decreased fetal growth velocity in late gestation, but longer gestation results in improved outcomes. Methods: This was a retrospective cohort analysis of patients in phase 2 of the National Pediatric Cardiology Quality Improvement Collaborative database from 2016-2019 who were anticipated to need stage 1 palliation (S1P) and had a GA of &gt; 36 weeks. Birth weight (BW) and BW z-scores were compared across GA to normative values. Outcome measures of survival to stage 2 palliation (S2P), post-S1P length of stay, and duration of mechanical ventilation were compared by GA. Results: The cohort included 1290 infants. There was an increase in mean BW with increasing GA (2.83 kg at 36-37 weeks, 3.15 kg at 38 weeks, 3.33 kg at 39 weeks, 3.40 kg at &gt; 40 weeks; p &lt;.0001). BW z-scores were below zero at all points in late gestation (-0.29 at 36-37 weeks, -0.17 at 38 weeks, -0.20 at 39 weeks, -0.52 at &gt; 40 weeks; p &lt;.0001), with a slower rate of growth beyond 39 weeks GA. Of 1216 patients with available mechanical ventilation data, patients born at later GA had shorter durations of ventilation (11.3 days at 36-37 weeks GA, 9.2 days at 38 weeks, 7.6 days at 39 weeks, 6.6 days at &gt; 40 weeks; p=0.0001). Among 1083 survivors to discharge or S2P if not discharged, there was no difference in post S1P length of hospitalization across GA (p=0.19). Infants who reached S2P readiness milestones were more likely to undergo S2P if born after longer gestation (p=0.0001). Conclusions: Infants with single ventricle heart disease and aortic hypoplasia have decreased growth velocity in late gestation, but growth continues. Infants born after longer gestation have improved outcomes with shorter duration of ventilation and greater likelihood of undergoing S2P.

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.

Perinatal Palliative Care.

University of Pennsylvania, Philadelphia, Pennsylvania; Mended Little Hearts of Delaware, Wilmington, Delaware; National Pediatric Cardiology Quality Improvement Collaborative, Cincinnati, Ohio; Cardiac Neurodevelopmental Outcome Collaborative, Richmond, Virginia Guidelines for Letters. Letters posing a question or challenge to an article appearing in Obstetrics & Gynecology should be submitted within 8 weeks of the article’s publication online. Letters received after 8 weeks will rarely be considered. Letters should not exceed 350 words, including signatures and 5 references. Letters will be published at the discretion of the Editor. The Editor may send the letter to the authors of the original paper so their comments may be published simultaneously. The Editor reserves the right to edit and shorten letters. Letters should be submitted using the Obstetrics & Gynecology online submission and review system, Editorial Manager (http://ong.edmgr.com). Financial Disclosure The author did not report any potential conflicts of interest.

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.