Cardiology Quality Improvement Collaborative Registry Research Articles

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.

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.

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.

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.

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.

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.

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.

Development of a validated risk score for interstage death or transplant after stage I palliation for single-ventricle heart disease

ObjectiveTo develop a risk score to predict mortality or transplant in the interstage period. BackgroundThe “interstage” period between the stage 1 and stage 2 palliation is a time of high morbidity and mortality for infants with single-ventricle congenital heart disease. MethodsThis was an analysis of patients with single-ventricle congenital heart disease requiring arch reconstruction who were enrolled in the National Pediatric Cardiology Quality Improvement Collaborative registry from 2008 to 2015. The primary composite endpoint was interstage mortality or transplant. Multivariable logistic regression and classification and regression tree analysis were performed on two-thirds of the patients (“learning cohort”) to build a risk score for the composite endpoint, that was validated in the remaining patients (“validation cohort”). ResultsIn the 2128 patients analyzed in the registry, the overall event rate was 9% (153 [7%] deaths, 42 [2%] transplants). In the learning cohort, factors independently associated with the composite endpoint were (1) type of Norwood; (2) postoperative ECMO; (3) discharge with Opiates; (4) No Digoxin at discharge; (5) postoperative Arch obstruction, (6) moderate-to-severe Tricuspid regurgitation without an oxygen requirement, and (7) Extra Oxygen required at discharge in patients with moderate-to-severe tricuspid regurgitation. This model was used to create a weighted risk score (“NEONATE” score; 0-76 points), with >75% accuracy in the learning and validation cohorts. In the validation cohort, the event rate in patients with a score >17 was nearly three times those with a score ≤17. ConclusionsWe introduce a risk score that can be used post-stage 1 palliation to predict freedom from interstage mortality or transplant.

Outcomes Associated With Unplanned Interstage Cardiac Interventions After Norwood Palliation

Anatomic lesions are a common cause of decompensation during the interstage period after Norwood stage 1 palliation (S1P). This study describes the risk factors for and outcomes after unplanned surgical and catheter-based interstage cardiac interventions. Participants in the National Pediatric Cardiology Quality Improvement Collaborative registry discharged from the hospital after S1P between 2008 and 2016 were studied. Variables at S1P, interstage, and at stage 2 palliation (S2P) hospitalizations were examined. Multivariable logistic regression was used to compare those who had an unplanned interstage intervention to those who did not. Of 1994 participants from 60 programs, 343 (17.1%) had at least 1 unplanned interstage intervention. Aortic valve dilation before S1P, longer S1P cardiopulmonary bypass time, pulmonary artery stent placement between S1P and discharge, aortic arch obstruction on the S1P discharge echocardiogram, and lower weight at S1P discharge were independently associated with receiving an unplanned interstage intervention. Interstage mortality between groups was similar at 6%, as was interstage duration. Participants undergoing unplanned interstage interventions were more likely to undergo heart transplant before S2P or deemed to be unsuitable for S2P (7.3% vs 2.7%, P < .001). Unplanned interstage interventions after S1P did not increase interstage mortality, but participants with an unplanned intervention were less likely to progress to S2P. Residual anatomic lesions are risk factors for unplanned interstage interventions. For those with progressive ventricular dysfunction in the presence of arch obstruction by echocardiogram, aortic arch reintervention is warranted.

Variability of antithrombotics use in patients with hypoplastic left heart syndrome and its variants following first- and second-stage palliation surgery: a national report using the National Pediatric Cardiology Quality Improvement Collaborative registry.

We carried out a multicentre, retrospective review using the National Pediatric Cardiology Quality Improvement Collaborative registry including patients from 2008 to 2013 across 52 surgical sites. Antithrombotic medications used at Stage I and Stage II discharge were evaluated. Variability of antithrombotics use at the individual patient level and intersite variability, incidence of shunt thrombosis, and other adverse events such as cardiac arrest, seizure, stroke, and need for cardiac catheterisation intervention in the interstage period were identified. Antithrombotic strategies for hybrid Stage I patients were evaluated but they were excluded from the variability and outcomes analysis. A total of 932 Stage I and 923 Stage II patients were included in the study: 93.8% of Stage I patients were discharged on aspirin and 4% were discharged on no antithrombotics, and 77% of Stage II patients were discharged on aspirin and 17.5% were discharged on no antithrombotics. Only three patients (0.2%) presented with interstage shunt thrombosis. The majority of patients who died during interstage or required shunt dilation and/or stenting were discharged home on aspirin. Aspirin is the most commonly used antithrombotic following Stage I and Stage II palliation. There is more variability in the choice of antithrombotics following Stage II compared with Stage I. The incidence of interstage shunt thrombosis and associated adverse events was rare.

Center Variability in Timing of Stage 2 Palliation and Association with Interstage Mortality: A Report from the National Pediatric Cardiology Quality Improvement Collaborative.

For infants with single-ventricle lesions with aortic arch hypoplasia, the interstage period from discharge following stage 1 palliation (S1P) until stage 2 palliation (S2P) remains high risk. Significant variability among institutions exists around the timing of S2P. We sought to describe institutional variation in timing of S2P, determine the association between timing of S2P and interstage mortality, and determine the impact of earlier S2P on hospital morbidity and mortality. The National Pediatric Cardiology Quality Improvement Collaborative registry was queried. Centers were divided based on median age at S2P into early (n=15) and late (n=16) centers using a cutoff of 153days. Groups were compared using Chi-squared or Wilcoxon rank-sum test. Multivariable logistic regression was used to determine risk factors for interstage mortality. The final cohort included 789 patients from 31 centers. There was intra- and inter-center variability in timing of S2P, with the median age by center ranging from 109 to 214days. Late centers had a higher mortality (9.9 vs. 5.7%, p=0.03) than early centers. However, the event rate (late: 8.2 vs. early: 5.8 deaths per 10,000 interstage days) was not different by group (p=0.26). Survival to hospital discharge and hospital length of stay following S2P were similar between groups. In conclusion, in a large multi-institution collaborative, the median age at S2P varies among centers. Although optimal timing of S2P remains unclear, centers performing early S2P did not experience worse S2P outcomes and experienced less interstage mortality.

Risk Factors for Unanticipated Readmissions During the Interstage: A Report From the National Pediatric Cardiology Quality Improvement Collaborative

This study describes unanticipated interstage readmissions in patients with hypoplastic left heart syndrome, identifies independent risk factors for unanticipated interstage readmissions, and evaluates variation in unanticipated readmission rates among collaborative centers. Retrospective data of patients enrolled in the National Pediatric Cardiology Quality Improvement Collaborative registry from July 2008 to July 2013 were analyzed. Risk factors present at the beginning of the interstage were captured. Competing risks time to event analyses determined the association between these factors and unanticipated interstage readmission. Readmission center variation was examined using funnel plots. Unanticipated interstage readmissions occurred in 66% of 815 patients at 50 centers. The median readmission length of stay was 2 days (interquartile range: 0-6) and median time to first readmission was 29 days (interquartile range: 9-63). Most readmissions were prompted by minor changes in clinical status (64%), whereas only 6% were major adverse event readmissions. Independent readmission risk factors included genetic syndrome (HR = 1.40, 95% CI: 1.05-1.88), center volume (small vs large HR = 1.32, CI: 1.04-1.66, medium vs large HR = 1.35, CI: 1.09-1.68), preoperative ventricular dysfunction (HR = 2.02, CI: 1.31-3.10), tricuspid regurgitation (HR = 1.36, CI: 1.08-1.72), duration of circulatory arrest (HR = 0.99, CI: 0.989-0.998), and undergoing Hybrid procedure relative to Norwood/right ventricle to pulmonary artery conduit (HR = 1.40, CI: 1.02-1.93). There was significant center variation in the number of readmissions and duration of readmissions. Unanticipated readmissions are common during the interstage period with notable center variation. However, these readmissions are short and are rarely in response to major adverse events.

Abstract 12605: Center Variability in Timing of Stage 2 Palliation and Association With Interstage Mortality: A Report From the National Pediatric Cardiology Quality Improvement Collaborative

Introduction: The interstage period from discharge following stage 1 palliation (S1P) until stage 2 palliation (S2P) remains high risk. Significant variability between institutions exists around the timing of S2P. We sought to describe the variability in a multi-institution cohort and assess its association with interstage mortality. Methods: The National Pediatric Cardiology Quality Improvement Collaborative registry, with data from 52 centers, was queried. Patients undergoing a hybrid S1P, transplanted prior to S2P, lost to follow up prior to S2P or deemed not candidates for S2P were excluded. Only centers with 10 or greater patients meeting eligibility were included to reduce the impact of outliers. Centers were divided based on median age at S2P into early (n=15) and late (n=16) centers using a cutoff of 153 days. Groups were compared using Chi-squared or Wilcoxon rank sum test. Results: The final cohort included 789 patients from 31 centers. Center specific median age at S2P varied from 109 to 214 days, with a center mean of 158 ± 27 days. At S1P, the late centers had a higher prevalence of preoperative ventilation (34.7% vs. 26.9%, p=0.02) and longer average post-S1P duration of intubation (14.4 ± 19.7 vs. 10.2 ± 11.4 days, p&lt;0.001) and S1P hospital length of stay (48.5 ± 30.4 vs. 38.5 ± 22.3 days, p&lt;0.0001). Interstage mortality was significantly higher in centers performing late vs. early S2P (9.9% vs. 5.7%, p=0.03). Interstage event rate (late: 8.2 vs. early: 5.8 deaths per 10000 interstage days) was not different by group (p=0.26), but interstage duration was significantly longer (133.9 ± 71.5 vs. 103.4 ± 37.8 days, p&lt;0.0001) in the late group. Survival to hospital discharge (98.9% in both groups, p&gt;0.98) and hospital length of stay following S2P (late: 15.6 ± 22.3 vs. early: 13.7 ± 22.4, p=0.68) were similar between groups. Conclusions: In a large multi-institution collaborative, the median age at S2P varies between centers. Centers performing S2P at a later median age have higher interstage mortality. This may be in part due to a higher severity of illness, reflected by higher S1P morbidity in this group. Although optimal timing of S2P remains unclear, centers performing early S2P did not experience worse S2P outcomes, and experienced less interstage mortality.

Site of Interstage Care, Resource Utilization, and Interstage Mortality: A Report from the NPC-QIC Registry

Morbidity and mortality remain high for patients with hypoplastic left heart syndrome during the interstage period between Norwood and Glenn despite ongoing QI efforts. We sought to identify associations between the site of interstage care, interstage events, and mortality. Data for patients enrolled in the National Pediatric Cardiology Quality Improvement Collaborative registry from July 2008 through February 2013 were reviewed. Patients had outpatient interstage care at (1) the surgical site (SS) performing Norwood, (2) a non-surgical site (NSS), or (3) a combination. Interstage events were compared among these groups and, when applicable, by distance from SS to NSS. 688 patients from 47 sites met entry criteria. Patients were followed at the SS 411 (60%), NSS 121 (17%), or a combination 143 (21%). Data were not available for 13 (2%). There were 66 deaths (10%) among the entire cohort: 37 (9%) at SS, 13 (11%) at NSS, 15 (10%) at a combination. The proportion of deaths among these groups was not statistically significant (p = 0.60), nor was there a difference based on SS-to-NSS distance. Patients followed at the SS were more likely to have problems detected with feeding (p = 0.03) and breathing (p = 0.002), and ED visits (p < 0.001). The site of interstage care was not associated with mortality, nor was there a difference based on SS-to-NSS distance. Patients followed at the SS had more detected breathing and feeding problems, and ED visits. Further study is required to elucidate the clinical significance of these differences.

Impact of Pharmacotherapy on Interstage Mortality and Weight Gain in Children with Single Ventricle

OBJECTIVE.: Infants with single ventricle physiology have a high mortality and poor somatic growth during the interstage period. We retrospectively assessed the impact of pharmacotherapy in this population using a multicenter database. DESIGN AND RESULTS.: Records for 395 patients (63.5% boys) with single ventricle were obtained from the National Pediatric Cardiology Quality Improvement Collaborative registry. Median of five medications were prescribed per patient at discharge after stage 1 palliation (interquartile range 3 to 6); the most common medications being aspirin (95.7%), diuretics (90.4%), angiotensin convertase enzyme inhibitors (37.7%), proton pump inhibitors (33.4%), H2 receptor blockers (30.6%), and digoxin (27.6%). Interstage mortality was 9.4%. Digoxin use was associated with lower risk of death (P =.03) on univariable analysis, however no single medication was an independent predictor on regression analysis. Change in weight-for-age z-score was studied as outcome of somatic growth with 36.3% patients showing a decrease during the interstage period. Total number of medications prescribed to a patient showed a negative correlation with the interstage change in z-score (r = -0.19, P =.002). On univariable comparisons, use of metoclopramide and lansoprazole were associated with decreased z-score (P =.004 and.041, respectively) although linear regression failed to identify any agent as independent predictor. CONCLUSIONS.: Children with single ventricle have high mortality and a profound medication burden. No individual medication is independently associated with better survival or weight gain during interstage period. Despite widespread use, proton pump inhibitors and prokinetic agents are not associated with better outcomes and may be associated with poor growth.

Clinical Outcomes and Resource Use for Infants With Hypoplastic Left Heart Syndrome During Bidirectional Glenn: Summary From the Joint Council for Congenital Heart Disease National Pediatric Cardiology Quality Improvement Collaborative Registry

The National Pediatric Cardiology Quality Improvement Collaborative (NPC-QIC) registry captures information on interstage management of infants with hypoplastic left heart syndrome (HLHS). The purpose of this study was to identify interstage risk factors for increased resource use and adverse outcomes during bidirectional Glenn (BDG) hospitalization. All infants in the NPC-QIC registry (31 United States hospitals) undergoing BDG surgery were included (December 2009 to August 2010). Patient demographics, interstage variables, operative procedures, and complications were recorded. Days of hospitalization, ventilation, inotrope use, and complications were surrogates of resource use. Logistic regression analysis determined the associations between predictor variables and resource use. Of 162 infants, 105 (65 %) were males. At BDG, the median age was 155 days (range 78-128), mean weight-for-age z-score was -1.6 ± 1.1, mean length-for-age z-score was -1.5 ± 1.7, and mean preoperative oxygen saturation was 78 % ± 7 %. Caloric recommendations were met in 60 % of patients, and 85 % of patients participated in a home-surveillance program. Median days of intubation, inotrope use, and hospitalization were 1, 2, and 7, respectively. There were 4 post-BDG deaths and 55 complications. In multivariate analysis, lower weight-for-age z-score, female sex, and aortic atresia with mitral stenosis were associated with a higher risk of BDG complications. Meeting caloric recommendations before BDG was associated with fewer hospitalization days. Lower weight-for-age z-score was an independent and potentially modifiable risk factor for BDG complications. HLHS infants who met caloric recommendations before BDG had a lower duration of hospitalization at BDG. These data justify targeting nutrition in interstage strategies to improve outcomes and decrease costs for patients with HLHS.

Variation in Growth of Infants with a Single Ventricle

The study goal was to evaluate interstage growth variation among sites participating in the National Pediatric Cardiology Quality Improvement Collaborative registry caring for infants with hypoplastic left heart syndrome and to identify nutritional practices common among sites achieving best growth outcomes. This was a retrospective analysis of infants in the registry who had presented due to their superior cavopulmonary connection (SCPC) and whose surgical site had enrolled ≥ 4 eligible patients in the registry. The primary outcome variable was weight-for-age z-score (WAZ) change between Norwood discharge and presentation for SCPC (interstage period). Blinded, structured interviews were performed with each site regarding site-specific nutritional practices. Practices common among sites with positive interstage WAZ changes were identified. Sixteen centers enrolled 132 infants from December 2008 through December 2010. Median age at SCPC was 5 months (2.6-12.6), and median interstage WAZ change was -0.29 (-3.2 to 2.3). Significant variation in WAZ changes among sites was demonstrated (P < .001). Sites that used standard feeding evaluation prior to Norwood discharge and that closely monitored for specific weight gain/loss red flags in the interstage period demonstrated significantly better patient growth than those that did not use these practices (P = .002). Considerable variation exists in interstage growth among patients receiving care at these 16 surgical sites. Standardization of interstage nutritional management with focus on best nutritional practices may lead to improved growth in this high-risk population of infants.

Variation in Preoperative and Intraoperative Care for First-stage Palliation of Single-ventricle Heart Disease: A Report from the Joint Council on Congenital Heart Disease National Quality Improvement Collaborative

As the first multicenter quality improvement collaborative in pediatric cardiology, the Joint Council on Congenital Heart Disease National Pediatric Cardiology Quality Improvement Collaborative registry collects information on the clinical care and outcomes of infants discharged home after first-stage palliation of single-ventricle heart disease, the Norwood operation, and variants. We sought to describe the preoperative and intraoperative characteristics of the first 100 patients enrolled in the National Pediatric Cardiology Quality Improvement Collaborative registry. From 21 contributing centers, 59% of infants were male, with median birth weight of 3.1 kg (1.9-5.0 kg); the majority had hypoplastic left heart syndrome (71%). A prenatal diagnosis of congenital heart disease was made in 75%; only one had fetal cardiac intervention. Chromosomal anomalies were present in 8%, and major noncardiac organ system anomalies were present in 9%. Preoperative risk factors were common (55%) but less frequent in those with prenatal cardiac diagnosis (P= .001). Four patients underwent a preoperative transcatheter intervention. Substantial variation across participating sites was demonstrated for choice of initial palliation for the 93 patients requiring a full first-stage approach, with 50% of sites performing stage I with right ventricle to pulmonary artery conduit as the preferred operation; 89% of hybrid procedures were performed at a single center. Significant intraoperative variation by site was noted for the 83 patients who underwent traditional surgical stage I palliation, particularly with use of regional perfusion and depth of hypothermia. In summary, there is substantial variation across surgical centers in the successful initial palliation of infants with single-ventricle heart disease, particularly with regard to choice of palliation strategy, and intraoperative techniques including use of regional perfusion and depth of hypothermia. Further exploration of the relationship of such variables to subsequent outcomes after hospital discharge may help reduce variability and improve long-term outcomes.