Management of Hematochezia in Infants with Congenital Heart Disease Admitted to the Acute Care Cardiology Unit: A Multicenter Retrospective Pilot Study.

OBJECTIVE: To assess the evaluation and prevalence of benign hematochezia (BH) vs necrotizing enterocolitis (NEC) in infants with congenital heart disease (CHD) <6 months old admitted to the acute care cardiology unit. STUDY DESIGN: This was a multicenter retrospective review of patient characteristics and evaluation of all hematochezia events in patients with CHD <6 months admitted to acute care cardiology unit at 3 high-volume tertiary care centers from February 2019 to January 2021. NEC was defined by the Bell staging criteria. Patients with gastrointestinal disorders were excluded. RESULTS: In total, 180 hematochezia events occurred in 121 patients; 42 patients had more than 1 event. In total, 61% of affected patients had single-ventricle physiology (38% hypoplastic left heart syndrome). Median age and weight at hematochezia were 38 days (IQR 24, 79) and 3.7 kg (IQR 3.2, 4.4). In total, 77% of hematochezia events were BH, and 23% were NEC. There were no surgical interventions for NEC or deaths from NEC. Those with NEC were significantly younger (34 vs 56 days, P < .01) and smaller (3.7 vs 4 kg, P < .01). Single-ventricle physiology was significantly associated with NEC. Initial bloodwork and diagnostic imaging at each center were assessed. There was no significant difference in white blood cell count or C-reactive protein in those with NEC compared with BH. Blood culture results were all negative. CONCLUSIONS: The majority of infants with CHD with hematochezia have BH over NEC, although single-ventricle and surgical patients remain at greater risk. Infants <45 days are more vulnerable for developing NEC. Bloodwork was noncontributory in the identification of cardiac NEC. Expansion to a prospective study to develop a treatment algorithm is important to avoid overtreatment.

Feeding Practices in Infants with Hematochezia and Necrotizing Enterocolitis on Acute Care Cardiology Units.

Infants with congenital heart disease (CHD) are at risk for developing both benign hematochezia and necrotizing enterocolitis (NEC). Despite these risks there are very few studies that investigate modifiable risk factors such as feeding practices. It remains unclear what feeding practices should be avoided due to higher incidence of CHD-NEC. We aim to assess the feeding practices across three high volume tertiary centers to establish a relationship between various feeding practices and development of NEC. A multicenter retrospective review of feeding practices at the time of documented hematochezia event that occurred between 1/2019 and 1/2021 in infants with CHD who were less than 6 months of age. NEC was defined as Bells Stage 2 or greater. Age, weight, ventricular morphology, primary diagnoses, feeding route, feed change, and formula type were evaluated. 176 hematochezia events occurred in 121 patients, 72% of these events were considered benign hematochezia with the remaining 28% being true NEC. Single ventricle (SV) physiology (p < 0.05), younger age, < 45 days of life, (p < 0.001), and feeding route were statistically associated with true NEC (p < 0.01). Formula type and recent change in feed administration were not associated with NEC. The caloric density of feeds at the time of hematochezia was nearing significance. The majority of hematochezia events are benign in nature, however, there should be heightened awareness in patients who are SV, younger in age, and those who are post-pylorically fed. There may be some risk in using higher caloric density feeds (> 24 kcal/oz), however, additional research is needed to fully establish this relationship.

Improvement in Palivizumab Administration Prior to Discharge for Hospitalized Infants with Hemodynamically Significant Congenital Heart Disease: A Quality Improvement Initiative.

In this quality improvement initiative, we aimed to increase provider adherence with palivizumab administration guidelines for hospitalized infants with hemodynamically significant congenital heart disease. We included 470 infants over four respiratory syncytial virus (RSV) seasons from 11/2017 to 03/2021 (baseline season: 11/2017-03/2018). Interventions included the following: education, including palivizumab in the sign-out template, identifying a pharmacy expert, and a text alert (seasons 1 and 2: 11/2018-03/2020) that was replaced by an electronic health record (EHR) best practice alert (BPA) in season 3 (11/2020-03/2021). The text alert and BPA prompted providers to add "Need for RSV immunoprophylaxis" to the EHR problem list. The outcome metric was the percentage of eligible patients administered palivizumab prior to discharge. The process metric was the percentage of eligible patients with "Need for RSV immunoprophylaxis" on the EHR problem list. The balancing metric was the percentage of palivizumab doses administered to ineligible patients. A statistical process control P-chart was used to analyze the outcome metric. The mean percentage of eligible patients who received palivizumab prior to hospital discharge increased significantly from 70.1% (82/117) to 90.0% (86/96) in season 1 and to 97.9% (140/143) in season 3. Palivizumab guideline adherence was as high or higher for those with "Need for RSV immunoprophylaxis" on the problem list than for those without it in most time periods. The percentage of inappropriate palivizumab doses decreased from 5.7% (n = 5) at baseline to 4.4% (n = 4) in season 1 and 0.0% (n = 0) in season 3. Through this initiative, we improved adherence with palivizumab administration guidelines for eligible infants prior to hospital discharge.

Accelerating improvement: The Pediatric Acute Care Cardiology Collaborative data timeliness project.

BACKGROUND: The Pediatric Acute Care Cardiology Collaborative (PAC3) is a learning network focused on improving acute care cardiology patient outcomes. Data submission timeliness is a vulnerability for PAC3 and most clinical registries, directly affecting collaborative benchmarking, quality improvement (QI) and research projects. OBJECTIVE: PAC3 conducted a collaborative-wide QI project addressing data timeliness and efficiency. Data analysis of submitted cases from September 2019 to February 2020 revealed nine 'High Performer' centers who submitted cases within 67 days of hospital discharge (the limit for timeliness) >90% of the time and eight 'High Potential' sites who submitted timely cases <75% of the time. The primary aim was to increase case submission timeliness in 'High Potential' centers from 41% to 80% by December 2020. The secondary aim was to maintain timeliness in 'High Performer' sites. METHOD: During the intervention phase (March-December 2020), plan-do-study-act (PDSA) cycles included webinars, facilitated exploratory conversations, data review and development of a best practice guide ('Getting Started Toolkit'). On-boarded 'New Centers' starting in 2020 were also invited to test intervention effectiveness. Balancing measures included data collector job satisfaction and stress and resubmission rates. RESULTS: 'High Performer' and 'High Potential' centers submitted 11 358 cases from November 2019 to December 2020. Timely submission rates for 'High Potential' centers improved from 40.6% to 74.6% and were maintained at >90% for 'High Performer' centers. 'New Centers' averaged 92.6% timely case submissions during their first 6 months. Data collector job satisfaction and stress were not impacted, and the resubmission rates did not increase. CONCLUSION: PAC3's multicenter QI project increased data submission timeliness in a large pediatric subspecialty registry. The lessons learned and the Toolkit developed can be applied in other registries to improve data submission efficiency, with resultant improvement in benchmarking, QI, research, length of stay and outcomes.

Collective quality improvement in the paediatric cardiology acute care unit: establishment of the Pediatric Acute Care Cardiology Collaborative (PAC3).

Collaborative quality improvement and learning networks have amended healthcare quality and value across specialities. Motivated by these successes, the Pediatric Acute Care Cardiology Collaborative (PAC3) was founded in late 2014 with an emphasis on improving outcomes of paediatric cardiology patients within cardiac acute care units; acute care encompasses all hospital-based inpatient non-intensive care. PAC3 aims to deliver higher quality and greater value care by facilitating the sharing of ideas and building alignment among its member institutions. These aims are intentionally aligned with the work of other national clinical collaborations, registries, and parent advocacy organisations. The mission and early work of PAC3 is exemplified by the formal partnership with the Pediatric Cardiac Critical Care Consortium (PC4), as well as the creation of a clinical registry, which links with the PC4 registry to track practices and outcomes across the entire inpatient encounter from admission to discharge. Capturing the full inpatient experience allows detection of outcome differences related to variation in care delivered outside the cardiac ICU and development of benchmarks for cardiac acute care. We aspire to improve patient outcomes such as morbidity, hospital length of stay, and re-admission rates, while working to advance patient and family satisfaction. We will use quality improvement methodologies consistent with the Model for Improvement to achieve these aims. Membership currently includes 36 centres across North America, out of which 26 are also members of PC4. In this report, we describe the development of PAC3, including the philosophical, organisational, and infrastructural elements that will enable a paediatric acute care cardiology learning network.

Prevalence and Risk Factors for Pericardial Effusions Requiring Readmission After Pediatric Cardiac Surgery.

Pericardial effusion (PE) may require readmission after cardiac surgery and has been associated with postoperative morbidity and mortality. We sought to identify the prevalence and risk factors for postoperative PE requiring readmission in children. A retrospective analysis of the Pediatric Health Information System database was performed between January 1, 2003, and September 30, 2014. All patients ≤18 years old who underwent cardiac surgery were identified by ICD-9 codes. Those readmitted within 1 year with an ICD-9 code for PE were identified. Logistic regression analysis was performed to determine risk factors for PE readmissions. Of the 142,633 surgical admissions, 1535 (1.1%) were readmitted with PE. In multivariable analysis, older age at the initial surgical admission [odds ratio (OR) 1.17, p < 0.001], trisomy 21 (OR 1.24, p = 0.015), geographic region (OR 1.33-1.48, p ≤ 0.001), and specific surgical procedures [heart transplant (OR 1.82, p < 0.001), systemic-pulmonary artery shunt (OR 2.23, p < 0.001), and atrial septal defect surgical repair (OR 1.34, p < 0.001)] were independent risk factors for readmission with PE. Of readmitted patients, 44.2% underwent an interventional PE procedure. Factors associated with interventions included shorter length of stay (LOS) for the initial surgical admission (OR 0.85, p = 0.008), longer LOS for the readmission (OR 1.37, p < 0.001), and atrial septal defect surgery (OR 1.40, p = 0.005). In this administrative database of children undergoing cardiac surgery, readmissions for PE occurred after 1.1% of cardiac surgery admissions. The risk factors identified for readmissions and interventions may allow for improved risk stratification, family counseling, and earlier recognition of PE for children undergoing cardiac surgery.

Relationship Between Serum Brain-Type Natriuretic Peptide and Biomarkers of Growth in Infants With Shunt-Dependent Single Cardiac Ventricle.

For infants with shunt-dependent or ductal-dependent single ventricle heart disease, poor growth is common and associated with morbidity and impaired neurodevelopmental outcomes. Although attention has focused on nutrition to promote weight gain, little is known about the relation between heart failure and growth factors. A prospective observational pilot study was performed to assess the relation between heart failure, assessed by brain natriuretic peptide (BNP), and growth factors (insulin-like growth factor 1 [IGF-1] and insulin-like growth factor-binding protein 3) at 3 visits: (1) before discharge from neonatal intervention with the establishment of stable pulmonary blood flow, (2) immediately before superior cavopulmonary connection, and (3) before discharge after superior cavopulmonary connection operation. The relation between BNP and growth factors was analyzed using Spearman pairwise correlations at each visit and modeled over time with a linear mixed-effects model. Correlations were considered worthy of further exploration using a p <0.10, given the exploratory nature of the study. The study included 38 infants (66% male, 68% hypoplastic left heart syndrome). Median BNP was elevated at visit 1 and decreased over time (287 pg/dl [interquartile range 147 to 794], 85 pg/dl [52 to 183], and 90 pg/dl [70 to 138]). Median IGF-1 Z score was <0 at each visit but increased over time (-0.9 [interquartile range -1.1 to 0.1], -0.7 [-1.2 to 0.1], and -0.5 [-1.2 to 0]). Inverse correlations were found between BNP and IGF-1 at visit 1 (r = -0.40, p = 0.097), BNP and IGF-1 and insulin-like growth factor-binding protein 3 at visit 2 (r = -0.33, p = 0.080 and r = -0.33, p = 0.085, respectively) and BNP and IGF-1 Z score at visit 3 (r = -0.42, p = 0.049). Significant relations were likewise found between the change in BNP and the change in IGF-1 between visits 1 and 3 (p = 0.046) and between visits 2 and 3 (p = 0.048). In conclusion, this pilot study demonstrates an inverse correlation between BNP and growth factors, suggesting that the heart failure state associated with this physiology may play a mechanistic role in impaired growth.

Successful Reduction of Postoperative Chest Tube Duration and Length of Stay After Congenital Heart Surgery: A Multicenter Collaborative Improvement Project.

Background Congenital heart disease practices and outcomes vary significantly across centers, including postoperative chest tube (CT) management, which may impact postoperative length of stay (LOS). We used collaborative learning methods to determine whether centers could adapt and safely implement best practices for CT management, resulting in reduced postoperative CT duration and LOS. Methods and Results Nine pediatric heart centers partnered together through 2 learning networks. Patients undergoing 1 of 9 benchmark congenital heart operations were included. Baseline data were collected from June 2017 to June 2018, and intervention-phase data were collected from July 2018 to December 2019. Collaborative learning methods included review of best practices from a model center, regular data feedback, and quality improvement coaching. Center teams adapted CT removal practices (eg, timing, volume criteria) from the model center to their local resources, practices, and setting. Postoperative CT duration in hours and LOS in days were analyzed using statistical process control methodology. Overall, 2309 patients were included. Patient characteristics did not differ between the study and intervention phases. Statistical process control analysis showed an aggregate 15.6% decrease in geometric mean CT duration (72.6 hours at baseline to 61.3 hours during intervention) and a 9.8% reduction in geometric mean LOS (9.2 days at baseline to 8.3 days during intervention). Adverse events did not increase when comparing the baseline and intervention phases: CT replacement (1.8% versus 2.0%, P=0.56) and readmission for pleural effusion (0.4% versus 0.5%, P=0.29). Conclusions We successfully lowered postoperative CT duration and observed an associated reduction in LOS across 9 centers using collaborative learning methodology.

Center Variation in Chest Tube Duration and Length of Stay After Congenital Heart Surgery.

BACKGROUND: Nearly every child undergoing congenital heart surgery has chest tubes placed intraoperatively. Center variation in removal practices and impact on outcomes has not been well described. This study evaluated variation in chest tube management practices and outcomes across centers. METHODS: The study included patients undergoing any of 10 benchmark operations from June 2017 to May 2018 at participating Pediatric Acute Care Cardiology Collaborative (PAC3) and Pediatric Cardiac Critical Care Consortium (PC4) centers. Clinical data from PC4 centers were merged with chest tube data from PAC3 centers. Practices and outcomes were compared across centers in univariate and multivariable analysis. RESULTS: The cohort included 1029 patients (N = 9 centers). Median chest tube duration varied significantly across centers for 9 of 10 benchmark operations (all P ≤ .03), with a "model" center noted to have the shortest duration for 9 of 10 operations (range, 27.9% to 87.4% shorter duration vs other centers across operations). This effect persisted in multivariable analysis (P < .0001). The model center had higher volumes of chest tube output before removal (median, 8.5 mL/kg/24 h [model] vs 2.2 mL/kg/24 h [other centers]; P < .001], but it did not have higher rates of chest tube reinsertion (model center 1.3% vs 2.1%; P = .59) or readmission for pleural effusion (model center 4.4% vs 3.0%; P = .31), and had the shortest length of stay for 7 of 10 operations. CONCLUSIONS: This study suggests significant center variation in chest tube removal practices and associated outcomes after congenital heart surgery. Best practices used at the model center have informed the design of an ongoing collaborative learning project aimed at reducing chest tube duration and length of stay.

Aortopulmonary fistula after outflow tract stent in repaired truncus.

We present a case of an iatrogenic aortopulmonary (AP) fistula in a 9-year-old patient with a history of repaired truncus arteriosus without the use of a right ventricle to pulmonary artery conduit and subsequent transcatheter placement of a right ventricular outflow tract (RVOT) stent. Redilation of the stent resulted in a defect in the aortic wall and the creation of an AP fistula with an associated hemodynamically significant left to right shunt. This case demonstrates a previously unreported adverse event of transcatheter RVOT reintervention after truncus arteriosus repair.

A unique pattern of Tie1 expression in the developing murine lung.

The molecular mechanisms responsible for organ-specific differences in vascular development are not well established. Animals lacking the receptor tyrosine kinase Tie1 die of hemorrhage and pulmonary edema. Furthermore, cells lacking Tie1 are excluded from blood vessels of the mature lung. These findings suggest the importance of Tie1 in the pulmonary vasculature. We quantified the organ-specific expression of Tie1 during embryonic and postnatal murine development using both quantitative real-time polymerase chain reaction (PCR) and chemiluminescence employing a tie1.lacZ reporter. In the lung, Tie1 expression increases markedly immediately prior to birth and rises further in the newborn animal, a pattern not found in other organs. Furthermore, expression of Tie1 in the lung is also unique by its persistent increase in the adult animal. This unique pattern of Tie1 gene expression in the embryonic and mature lung supports a distinct role for Tie1 in the development and function of the pulmonary vasculature.

Notch1 and Jagged1 expression by the developing pulmonary vasculature.

The molecular mechanisms of pulmonary vascular development are poorly understood. Cell-specific developmental pathways are influenced by cell-cell signaling. Notch signaling molecules are highly conserved receptors active in many cell-fate determination systems. Recent observations of Notch molecules and a Notch ligand, Jagged1, suggest their importance in vascular morphogenesis, and particularly pulmonary vascular development. We performed a systematic evaluation of Notch1/Jagged1 gene and protein expression in the developing mouse lung from embryonic day 11 until adulthood by using quantitative PCR, immunofluorescence, and electron microscopic analysis. mRNA transcripts for Notch1-4 and Jagged1 increased progressively from early to later lung development, accompanied by a simultaneous rise in endothelial cell-specific gene expression, a pattern not seen in other organs. Notch1 mRNA was identified on both epithelial and mesenchymal structures of the embryonic lung. Immunofluorescence staining revealed the progressive acquisition of Notch1 and Jagged1 proteins by the emerging endothelium. Notch1 and Jagged1 were seen initially on well-formed, larger vessels within the embryonic lung bud and progressively on finer vascular networks. Each was also expressed on surrounding nonvascular structures. The localization of Notch1 and Jagged1 on endothelial cell surface membranes within the alveolar microvasculature was confirmed by immuno-electron microscopy. These temporal and spatial patterns in Notch1/Jagged1 gene and protein expression suggest multiple potential paths of cell-cell signaling during lung development and vascular morphogenesis.