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

BACKGROUND: The impact of published evidence on clinical practice has been understudied in pediatric cardiology. OBJECTIVE: 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. METHODS: 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). RESULTS: 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. CONCLUSIONS: 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.

Technical Performance Score: A Predictor of Outcomes After the Norwood Procedure.

BACKGROUND: The Technical Performance Score (TPS) can predict outcomes after congenital cardiac surgery. We sought to validate TPS as a predictor of both short- and long-term outcomes of the Norwood procedure. METHODS: We conducted a retrospective review of patients who underwent the Norwood procedure from 1997 to 2017. We assigned TPS (class 1, no residua; class 2, minor residua; class 3, major residua or reintervention for major residua before discharge) based on subcomponent scores from discharge echocardiograms or unplanned reinterventions, or both. Multivariable Cox or competing risk analysis, adjusted for preoperative patient- and procedure-related covariates, examined the association of TPS with postoperative hospital length of stay, transplant-free survival, and postdischarge reinterventions. RESULTS: Among 500 patients, 319 (64%) were male, 54 (11%) were premature, 56 (11%) had noncardiac anomalies/syndromes, 146 (29%) had preoperative risk factors, and 480 (96%) were assigned TPS. On multivariable analysis, class 3 had greater hazard for reinterventions in transplant-free survivors (class 3: subdistribution hazard ratio [HR], 2.06; 95% confidence interval [CI] 1.34-3.16; P = .001) and was associated with increased hospital length of stay vs class 1 (HR, 0.25; 95% CI, 0.18-0.34; P < .001). Transplant-free survival after Norwood surgery was shorter for both class 2 (HR, 2.48; 95% CI, 1.68-3.66; P < .001) and class 3 (HR, 3.29; 95% CI, 2.18-4.95; P < .001). CONCLUSIONS: TPS predicts early and late outcomes after Norwood. Absence of residual lesions results in improved long-term prognosis for single-ventricle patients. TPS may improve outcomes after Norwood by identifying patients warranting closer follow-up and potentially earlier reintervention.

Arch intervention following stage 1 palliation in hypoplastic left heart syndrome is associated with slower feed advancement: a report from the National Pediatric Quality Cardiology Improvement Collaborative.

INTRODUCTION: Infants undergoing stage 1 palliation for hypoplastic left heart syndrome may have post-operative feeding difficulties. Although the cause of feeding difficulties in these patients is multi-factorial, residual arch obstruction may affect gut perfusion, contributing to feeding intolerance. We hypothesised that undergoing arch reintervention following stage 1 palliation would be associated with post-operative feeding difficulties. METHODS: This was a retrospective cohort study. We analysed data from the National Pediatric Cardiology Quality Improvement Collaborative, which maintains a multicentre registry for infants with hypoplastic left heart syndrome discharged home following stage 1 palliation. Patients who underwent arch reintervention (percutaneous or surgical) prior to discharge following stage 1 palliation were compared with those who underwent non-aortic arch interventions after stage 1 palliation and those who underwent no intervention. Median post-operative days to full enteral feeds and weight for age z-scores were compared. Predictors of post-operative days to full feeds were identified. RESULTS: Among patients who underwent arch reintervention, post-operative days to full enteral feeds were greater than for those who underwent non-aortic arch interventions (25 versus 16, p = 0.003) or no intervention (median days 25 versus 12, p < 0.001). Arch intervention, multiple interventions, gestational age, and the presence of a gastrointestinal anomaly were predictors of days to full feeds. CONCLUSIONS: Repeat arch intervention is associated with a longer time to achieve full enteral feeding in patients with hypoplastic left heart syndrome after stage 1 palliation. Further investigation of this association is needed to understand the role of arch obstruction in feeding problems in these patients.

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.

Identifying best practices in interstage care: using a positive deviance approach within the National Pediatric Cardiology Quality Improvement Collaborative.

IntroductionTo identify interstage best practices associated with lower mortality, we studied National Pediatric Cardiology Quality Improvement Collaborative centres registry using a positive deviance approach. METHODS: Positive deviant and control centre team members were interviewed to identify potential interstage best practices. Subsequently, all collaborative centres were surveyed on the use of these practices to test their associations with centre mortality. Questionnaires were scored using Likert scales; the overall score was linearly transformed to a 0-100-point scale with higher scores indicating increased use of practices. Mortality was based on patients enrolled after a centre's first year in the collaborative. Centre mortality rates were divided into tertiles. Survey scores for the low mortality tertile were compared with the other tertiles. RESULTS: For this study, seven positive deviant and four control teams were interviewed. A total of 20 potential best practices were identified, including team composition, improvement practices, and parent involvement. Questionnaires were completed by 36/43 eligible centres, providing 1504 patients for analysis. Average survey score was 50.2 (SD 13.4). Average mortality was 6.1% (SD 4.1). There was no correlation between survey scores and mortality (r=0.14, p=0.41). The one practice associated with the low mortality tertile was frequency of discussion of interstage results: 58.3% of low mortality teams discussed results at least monthly versus 8.4% of the middle and high tertile centres (p=0.02). CONCLUSIONS: Low-mortality centres more frequently discuss interstage results than high-mortality centres. Heightened awareness of outcomes may influence practice; however, further study is needed to understand the variation in outcomes across centres.

Building a comprehensive team for the longitudinal care of single ventricle heart defects: Building blocks and initial results.

INTRODUCTION: With increasing survival of children with HLHS and other single ventricle lesions, the complexity of medical care for these patients is substantial. Establishing and adhering to best practice models may improve outcome, but requires careful coordination and monitoring. METHODS: In 2013 our Heart Center began a process to build a comprehensive Single Ventricle Team designed to target these difficult issues. RESULTS: Comprehensive Single Ventricle Team in 2014 was begun, to standardize care for children with single ventricle heart defects from diagnosis to adulthood within our institution. The team is a multidisciplinary group of providers committed to improving outcomes and quality of life for children with single ventricle heart defects, all functioning within the medical home of our heart center. Standards of care were developed and implemented in five target areas to standardize medical management and patient and family support. Under the team 100 patients have been cared for. Since 2014 a decrease in interstage mortality for HLHS were seen. Using a team approach and the tools of Quality Improvement they have been successful in reaching high protocol compliance for each of these areas. CONCLUSIONS: This article describes the process of building a successful Single Ventricle team, our initial results, and lessons learned. Additional study is ongoing to demonstrate the effects of these interventions on patient outcomes.

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

OBJECTIVE: 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. DESIGN: 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. RESULTS: 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. CONCLUSIONS: 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.

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.

Quality improvement through collaboration: the National Pediatric Quality improvement Collaborative initiative.

PURPOSE OF REVIEW: The National Pediatric Quality Improvement Collaborative (NPCQIC) was established to improve outcomes and quality of life in children with hypoplastic left heart syndrome and other single ventricle lesions requiring a Norwood operation. The NPCQIC consists of a network of providers and families collecting longitudinal data, conducting research, and using quality improvement science to decrease variations in care, develop and spread best practices, and decrease mortality. RECENT FINDINGS: Initial descriptive investigation of the collaborative data found interstage care process variations, different surgical strategies, diverse feeding practices, and variable ICU approaches between centers and within sites. Analysis and evaluation of these practice variations have allowed centers to learn from each other and implement change to improve processes. There has been an improvement in performance measures and most importantly, a 39.7% reduction in mortality. SUMMARY: The NPCQIC has shown, in a rare disease such as hypoplastic left heart syndrome that a network based on multicenter collaboration, patient (parent) engagement, and quality improvement science can facilitate change in practices and improvement in outcomes.

Predictors of Prolonged Hospital Length of Stay Following Stage II Palliation of Hypoplastic Left Heart Syndrome (and Variants): Analysis of the National Pediatric Cardiology Quality Improvement Collaborative (NPC-QIC) Database.

The objective of this study is to identify predictors of prolonged hospital length of stay (LOS) for single ventricle patients following stage 2 palliation (S2P), excluding patients who underwent a hybrid procedure. We explore the impact of demographic features, stage 1 palliation (S1P), interstage I (IS1) management, S2P, and post-surgical care on hospital LOS following S2P. We conducted a retrospective analysis of the National Pediatric Cardiology Quality Improvement Collaborative (NPC-QIC) database. The NPC-QIC database is an established registry of patients with hypoplastic left heart syndrome (HLHS) and its variants. It contains detailed information regarding the demographic features, S1P, IS1, S2P, and interstage 2 (IS2) management of children with HLHS and related single ventricle cardiac malformations. Between 2008 and 2012, there were 477 participants with recorded LOS data in the NPC-QIC registry. Excluding the 29 patients who underwent hybrid procedure, there were 448 participants who underwent a Norwood (or Norwood-variant procedure) as S1P. In order to be included in the NPC-QIC database, participants were discharged to home following S1P and prior to S2P. We found that postoperative LOS among the 448 S2P procedure recipients is most strongly influenced by the need for reoperation following S2P, the need for an additional cardiac catheterization procedure following S2P, the use of non-oral methods of nutrition (e.g., nasogastric tube, total parental nutrition, gastrostomy tube), and the development of postoperative complications. Factors such as age at the time of S2P, the presence of a major non-cardiac anomaly, site participant volume, IS1 course, the type and number of vasoactive agents used following S2P, and the need for more than 1 intensive care unit (ICU) hospitalization (following discharge to the ward but prior to discharge to home) were significant predictors by univariate analysis but not by multivariate analysis. We excluded participants undergoing the hybrid procedure as S1P from this analysis given that the S2P following the initial hybrid is typically a more complicated procedure. Hospital LOS following S2P among children undergoing the Norwood or Norwood-variant procedure as S1P is most strongly influenced by events following S2P and not demographic or S1P factors. Factors most predictive of prolonged LOS include the need for reoperation, the need for an additional cardiac catheterization procedure following S2P, the need for non-oral methods of nutrition, and the development of postoperative complications.

Use of a learning network to improve variation in interstage weight gain after the Norwood operation.

BACKGROUND: Growth failure is common in infants with single ventricle. This study evaluated the use of a learning network, the National Pediatric Cardiology Quality Improvement Collaborative (NPC-QIC), to spread optimized nutritional practices and improve infant growth. METHODS: A previously identified Nutritional Bundle was spread among NPC-QIC sites. PRIMARY OUTCOME: interstage weight-for-age z-score change (ΔWAZ) between discharge from stage 1 palliation (S1) and stage 2 surgical palliation (S2). Variation among sites in interstage ΔWAZ was evaluated before (Period 1) and after (Period 2) spread of Nutritional Bundle. We performed an analysis of NPC-QIC registry infants presenting for S2 at sites previously shown to have significant variation in interstage patient growth. RESULTS: Four hundred seven infants from 15 sites underwent S2 between 2008 and 2013: 158 in Period 1 (December 2008-December 2010) and 249 in Period 2 (December 2010-April 2013). Median age at S2 was 4.9 months (2.6-12.8) with no difference between periods. There was significant variation in interstage ΔWAZ among sites in Period 1 (P = .01) but not in Period 2 (P = .39). More patients had an interstage ΔWAZ <0 in Period 1 (43%) than Period 2 (32%) (P = .03). In Period 1, the median interstage ΔWAZ was <0 in six sites while in Period 2 no site had median interstage ΔWAZ <0. Sites with the worst patient growth in Period 1 had marked improvement in Period 2 (P = .02, .06, and .06, respectively). CONCLUSIONS: Spread of optimal nutritional practices led to decreased variation in interstage growth with most improvement observed at sites with the worst baseline growth outcomes.

Technical performance score is associated with outcomes after the Norwood procedure.

OBJECTIVES: The technical performance score (TPS) has been reported in a single center study to predict the outcomes after congenital cardiac surgery. We sought to determine the association of the TPS with outcomes in patients undergoing the Norwood procedure in the Single Ventricle Reconstruction trial. METHODS: We calculated the TPS (class 1, optimal; class 2, adequate; class 3, inadequate) according to the predischarge echocardiograms analyzed in a core laboratory and unplanned reinterventions that occurred before discharge from the Norwood hospitalization. Multivariable regression examined the association of the TPS with interval to first extubation, Norwood length of stay, death or transplantation, unplanned postdischarge reinterventions, and neurodevelopment at 14 months old. RESULTS: Of 549 patients undergoing a Norwood procedure, 356 (65%) had an echocardiogram adequate to assess atrial septal restriction or arch obstruction or an unplanned reintervention, enabling calculation of the TPS. On multivariable regression, adjusting for preoperative variables, a better TPS was an independent predictor of a shorter interval to first extubation (P=.019), better transplant-free survival before Norwood discharge (P<.001; odds ratio, 9.1 for inadequate vs optimal), shorter hospital length of stay (P<.001), fewer unplanned reinterventions between Norwood discharge and stage II (P=.004), and a higher Bayley II psychomotor development index at 14 months (P=.031). The TPS was not associated with transplant-free survival after Norwood discharge, unplanned reinterventions after stage II, or the Bayley II mental development index at 14 months. CONCLUSIONS: TPS is an independent predictor of important outcomes after Norwood and could serve as a tool for quality improvement.

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.

BACKGROUND AND METHODS: 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. RESULTS: 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. CONCLUSIONS: 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.

Variation in postoperative care following stage I palliation for single-ventricle patients: a report from the Joint Council on Congenital Heart Disease National Quality Improvement Collaborative.

BACKGROUND AND METHODS: The National Pediatric Cardiology Quality Improvement Collaborative (NPC-QIC) has established a national data registry for patients with hypoplastic left heart syndrome and its variants undergoing staged palliation. The goal of this collaborative is to better understand current care practices and to improve outcomes in children with these severe and complex forms of congenital heart disease. In this study, we describe the postoperative intensive care course, and its variations, for the first 100 patients enrolled into the registry. RESULTS: Patients were enrolled from 21 contributing sites and were discharged home after stage I palliation between July 2008 and February 2010. Following stage I palliation, enrolled participants remained in the intensive care unit for a median duration of 11 days (range: 3-68 days). Duration of intensive care unit stay varied and was greatest for those patients with aortic atresia versus aortic hypoplasia (P= 0.04) and for those who underwent a modified Blalock-Taussig shunt as part of their palliation. The duration of intensive care unit stay also varied by contributing site (medians ranged from 8 to 18 days). Participants requiring reoperation had significantly prolonged lengths of stay (P= .0003). Inotropic agent use among univentricular registry participants also varied by site. The majority of recipients received milrinone (87%), dopamine (64%), and epinephrine (62%). Cardiac catheterization following surgery occurred in 20 patients. Fifteen percent of participants underwent an interventional procedure. Complication following stage I palliation was also fairly common. CONCLUSIONS: Considerable variation exists in the postoperative course and management of univentricular patients following stage I palliation. Variation in length of intensive care unit stay, inotropic agent use, need for reoperation or cardiac catheterization, and postoperative complications are described. Further studies to determine etiologies for observed variation may result in improved standards of care and better outcomes during the interstage period.