Racial, Ethnic, and Language Inequities in Ambulatory Pediatrics Patient Family Experience.

OBJECTIVE: To determine the association of patient race, patient-provider racial congruence, patient ethnicity, and family primary language with patient family experience (PFE) survey responses. METHODS: Cross-sectional review of PFE survey responses from all ambulatory medical encounters at a large, urban children's hospital system June 1, 2020-May 31, 2022. Exposures were patient race, patient-provider racial congruence, patient ethnicity, and family primary language. We adjusted analyses for neighborhood-level socioeconomic deprivation, patient sex and age, encounter specialty, and location of care. Outcomes were PFE survey scores for 5 questions focused on overall experience, respect, and safety; categorized using industry standard metric of presence of a "top-box" score, defined as a 9 or 10 for questions on an 11-point scale or as 4 on a 4-point scale. RESULTS: We included 89,175 surveys (15.6% response rate). The odds of having optimal, "top-box" responses for several assessed questions were lower for patients identified as Asian (e.g., adjusted odds ratio [OR] 0.46; 95% confidence interval [CI] 0.40, 0.52) or Black (e.g., OR 0.65; CI 0.60, 0.70) compared to White, and for Hispanic (e.g., OR 0.84; CI 0.72, 0.97) compared to non-Hispanic. Similarly, the odds of having "top-box" scores were lower for Spanish-primary-language (e.g., OR 0.38; CI 0.30, 0.48) compared to English-primary-language patients. Patient-provider racial congruence had higher odds of "top-box" responses for 2 of 5 assessed questions (e.g., OR 1.18; CI 1.04, 1.35). CONCLUSIONS: We found previously unreported inequities in ambulatory pediatric PFE outcomes, with worse experiences reported by Asian, Black, Hispanic, and Spanish-language patients.

Achieving an Optimal Outcome After Stage 1 Palliation for Hypoplastic Left Heart Syndrome and Variants: Frequency, Associated Factors, and Subsequent Outcomes.

BACKGROUND: We sought to measure frequency of achieving an optimal outcome after stage 1 palliation (S1P) for hypoplastic left heart syndrome and variants, determine factors associated with optimal outcomes, and compare outcomes after stage 2 palliation (S2P) using the National Pediatric Cardiology Quality Improvement Collaborative database (2008-2016). METHODS AND RESULTS: This is a retrospective cohort study with optimal outcome defined a priori as meeting all of the following: (1) discharge after S1P in <19 days (top quartile), (2) no red flag or major event readmissions before S2P, and (3) performing S2P between 90 and 240 days of age. Optimal outcome was achieved in 256 of 2182 patients (11.7%). Frequency varied among centers from 0% to 25%. Factors independently associated with an optimal outcome after S1P were higher gestational age (odds ratio [OR], 1.1 per week [95% CI, 1.0-1.2]; P=0.02); absence of a genetic syndrome (OR, 2.5 [95% CI, 1.2-5]; P=0.02); not requiring a post-S1P catheterization (OR, 2.7 [95% CI, 1.5-4.8]; P=0.01), intervention (OR, 1.5 [95% CI, 1.1-2]; P=0.006), or a procedure (OR, 4.5 [95% CI, 2.8-7.1]; P<0.001) before discharge; and not having a post-S1P complication (OR, 2.7 [95% CI, 1.9-3.7]; P<0.001). Those with an optimal outcome after S1P had improved S2P outcomes including shorter length of stay, less ventilator days, shorter bypass time, and fewer postoperative complications. CONCLUSIONS: Identifying patients at lowest risk for poor outcomes during the home interstage period could shift necessary resources to those at higher risk, alter S2P postoperative expectations, and improve quality of life for families at lower risk.

Initiative to increase family presence and participation in daily rounds on a paediatric acute care cardiology unit.

INTRODUCTION: Family-centred rounds benefit families and clinicians and improve outcomes in general paediatrics, but are understudied in subspecialty settings. We sought to improve family presence and participation in rounds in a paediatric acute care cardiology unit. METHODS: We created operational definitions for family presence, our process measure, and participation, our outcome measure, and gathered baseline data over 4 months of 2021. Our SMART aim was to increase mean family presence from 43 to 75% and mean family participation from 81 to 90% by 30 May, 2022. We tested interventions with iterative plan-do-study-act cycles between 6 January, 2022 and 20 May, 2022, including provider education, calling families not at bedside, and adjustment to rounding presentations. We visualised change over time relative to interventions with statistical control charts. We conducted a high census days subanalysis. Length of stay and time of transfer from the ICU served as balancing measures. RESULTS: Mean presence increased from 43 to 83%, demonstrating special cause variation twice. Mean participation increased from 81 to 96%, demonstrating special cause variation once. Mean presence and participation were lower during high census (61 and 93% at project end) but improved with special cause variation. Length of stay and time of transfer remained stable. CONCLUSIONS: Through our interventions, family presence and participation in rounds improved without apparent unintended consequences. Family presence and participation may improve family and staff experience and outcomes; future research is warranted to evaluate this. Development of high level of reliability interventions may further improve family presence and participation, particularly on high census days.

Parent Participation in Morbidity and Mortality Review: Parent and Physician Perspectives.

This study examined Morbidity and Mortality (M&M) review practices and perspectives of physicians and parents regarding parent participation in M&M review. Surveys were distributed to parents of children with a prior hospitalization for congenital heart disease (CHD) and physicians caring for pediatric CHD patients. Response distributions and Fisher's exact tests were performed to compare parent and physician responses. Qualitative survey data were thematically analyzed. Ninety-two parent and 36 physician surveys were analyzed. Physicians reported parent input or participation was rarely sought in M&M review. Parents with direct experience of adverse events or death of their child reported providers discussed events with them in a timely manner and answered their questions; however, nearly half wished their healthcare team had done something differently during the disclosure. There was no statistical difference between groups regarding transparency (P = .37, .79); however, there was a significant difference in perspectives regarding parental involvement in the M&M review (P < .001). Common themes important to parents which emerged from the qualitative analysis were being adequately informed, feeling their perspectives were acknowledged and respected, having attentive and empathetic providers, and receiving consistent messaging. Although rarely included in current practice, parent participation in M&M could offer unique insight and increase accountability to proposed change elucidated by M&M review.

Emergency Department Visits Before Sudden Unexpected Infant Death: A Touchpoint for Unsafe Sleep Reduction.

OBJECTIVES: Unsafe sleep remains a leading cause of preventable sudden unexpected infant death (SUID). Infants frequently visit emergency departments (EDs), but the frequency of visits before SUID is unknown. The objective of this study was to determine how often SUID infants visited a pediatric ED or urgent care (UC) before death. METHODS: We performed a retrospective study of infant deaths in the county of a large, academic pediatric institution. We linked institutional records with coroner reports and death scene investigations. We excluded deaths associated with childbirth, prematurity, injury, or underlying medical condition. We characterized all SUID infants, focusing on unsafe sleep factors detailed in the medical record and scene reports. The main outcome was ED/UC visit(s) before the visit for SUID. RESULTS: Seventy-three of 122 infant deaths met inclusion criteria for SUID over 76 months (April 2014-July 2020). Median age at death was 87 days (IQR 58, 137); 68 (93%) died before 6 months-of-age. Twenty infants (27%) had an ED/UC visit before SUID; mean visits for these infants were 1.7 (SD 0.8). Median days between the last ED/UC visit and SUID was 39; five infants visited the ED/UC within 2 weeks of SUID. Most visits were for minor medical conditions. All 73 SUID infants had at least one unsafe sleep factor; 88% had ≥2 and 56% ≥3. CONCLUSIONS: Many SUID infants visited a pediatric ED/UC before death, and unsafe sleep factors were found in every case. Early infancy ED/UC visits may present an opportunity for targeted prevention efforts.

Improving Discharge Efficiency and Charge Containment on a Pediatric Acute Care Cardiology Unit.

BACKGROUND: Hospital discharge delays can negatively affect patient flow and hospital charges. Our primary aim was to increase the percentage of acute care cardiology patients discharged within 2 hours of meeting standardized medically ready (MedR) discharge criteria. Secondary aims were to reduce length of stay (LOS) and lower hospital charges. METHODS: A multidisciplinary team used quality improvement methods to implement and study MedR discharge criteria in our hospital electronic health record. The criteria were ordered on admission and modified on daily rounds. Bedside nurses documented the time when all MedR discharge criteria were met. A statistical process control chart measured interventions over time. Discharge before noon and 30-day readmissions were also tracked. Average LOS was examined, comparing the first 6 months of the intervention period to the last 6 months. Inpatient charges were reviewed for patients with >2 hours MedR discharge delay. RESULTS: The mean percentage of patients discharged within 2 hours of meeting MedR discharge criteria increased from 20% to 78% over 22 months, with more patients discharged before noon (19%-32%). Median LOS decreased from 11 days (interquartile range: 6-21) to 10 days (interquartile range: 5-19) (P = .047), whereas 30-day readmission remained stable at 16.3%. A total of 265 delayed MedR discharges beyond 2 hours occurred. The sum of inpatient charges from care provided after meeting MedR criteria was $332 038 (average $1253 per delayed discharge). CONCLUSIONS: Discharge timeliness in pediatric acute care cardiology patients can be improved by standardizing medical discharge criteria, which may shorten LOS and decrease medical charges.

Performance of Pediatric Readmission Measures.

BACKGROUND: Readmission rates are frequently used as a hospital quality metric; yet multiple measures exist to evaluate pediatric readmission rates. We sought to assess how four different measures of pediatric readmission compare with assessment of both preventable and unplanned readmission. METHODS: Clinicians on hospital medicine, cardiology, neonatology, and neurology teams reviewed medical records for 30-day readmissions using an abstraction tool with high interrater reliability for preventability assessment. Readmissions between July 2014 and June 2016 were classified separately as preventable or not preventable and planned or unplanned. We compared the classifications to four existing readmission metrics: all-cause readmission, unplanned readmission/time flag classification, the pediatric all-condition readmission, and potentially preventable readmission. We calculated sensitivity and specificity for all readmission metrics. RESULTS: Among 30-day readmissions considered, 1,643 were eligible for medical record review; 1,125 reviews were completed by the clinical teams (68%). On medical record review, the majority of readmissions were determined not preventable (85%). Only 15% were classified as unplanned and preventable. None of the four readmission measures had appropriate sensitivity or specificity for identifying preventable readmission. The unplanned readmission/time flag classification had the highest sensitivity (95%) and specificity (90%) in identifying unplanned readmissions. CONCLUSION: None of the existing pediatric readmission measures can reliably determine preventability. The unplanned readmission/time flag measure performed best in identifying unplanned readmissions.

Conservation and Diversity in the Primary Reverse Photodynamics of the Canonical Red/Green Cyanobacteriochrome Family.

In this report, we compare the femtosecond to nanosecond primary reverse photodynamics (15EPg → 15ZPr) of eight tetrapyrrole binding photoswitching cyanobacteriochromes in the canonical red/green family from the cyanobacterium Nostoc punctiforme. Three characteristic classes were identified on the basis of the diversity of excited-state and ground-state properties, including the lifetime, photocycle initiation quantum yield, photointermediate stability, spectra, and temporal properties. We observed a correlation between the excited-state lifetime and peak wavelength of the electronic absorption spectrum with higher-energy-absorbing representatives exhibiting both faster excited-state decay times and higher photoisomerization quantum yields. The latter was attributed to both an increased number of structural restraints and differences in H-bonding networks that facilitate photoisomerization. All three classes exhibited primary Lumi-Go intermediates, with class II and III representatives evolving to a secondary Meta-G photointermediate. Class II Meta-GR intermediates were orange absorbing, whereas class III Meta-G had structurally relaxed, red-absorbing chromophores that resemble their dark-adapted 15ZPr states. Differences in the reverse and forward reaction mechanisms are discussed within the context of structural constraints.

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

Background The 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 Results Analysis 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). Conclusions ACEI 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.

Clinically Asymptomatic Sleep-Disordered Breathing in Infants with Single-Ventricle Physiology.

OBJECTIVES: To assess clinically asymptomatic infants with single-ventricle physiology (SVP) for sleep-disordered breathing (SDB) in the supine and car seat positions using polysomnography. Polysomnography results also were compared with results of a standard Car Seat Challenge to measure the dependability of the standard Car Seat Challenge. STUDY DESIGN: This was an observational study of 15 infants with SVP. Polysomnography data included Obstructive Index, Central Index, Arousal Index, Apnea Hypopnea Index, and sleep efficiency. Polysomnography heart rate and oxygen saturation data were used to compare polysomnography with the standard Car Seat Challenge. RESULTS: Polysomnography demonstrated that all 15 infants had SDB and 14 had obstructive sleep apnea (Obstructive Index ≥1/hour) in both the supine and car seat positions. Infants with SVP had a statistically significant greater median Obstructive Index in the car seat compared with supine position (6.3 vs 4.2; P = .03), and median spontaneous Arousal Index was greater in the supine position compared with the car seat (20.4 vs 15.2; P = .01). Comparison of polysomnography to standard Car Seat Challenge results demonstrated 5 of 15 (33%) of infants with SVP with abnormal Obstructive Index by polysomnography would have passed a standard Car Seat Challenge. CONCLUSIONS: Infants with SVP without clinical symptoms of SDB may be at high risk for SDB that appears worse in the car seat position. The standard Car Seat Challenge is not dependable in the identification of infants with SVP and SDB. Further studies are warranted to further delineate its potential impact of SDB on the clinical outcomes of infants with SVP.

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.

Usability Testing of an Interactive Dashboard for Surgical Quality Improvement in a Large Congenital Heart Center.

BACKGROUND: Interactive data visualization and dashboards can be an effective way to explore meaningful patterns in large clinical data sets and to inform quality improvement initiatives. However, these interactive dashboards may have usability issues that undermine their effectiveness. These usability issues can be attributed to mismatched mental models between the designers and the users. Unfortunately, very few evaluation studies in visual analytics have specifically examined such mismatches between these two groups. OBJECTIVES: We aimed to evaluate the usability of an interactive surgical dashboard and to seek opportunities for improvement. We also aimed to provide empirical evidence to demonstrate the mismatched mental models between the designers and the users of the dashboard. METHODS: An interactive dashboard was developed in a large congenital heart center. This dashboard provides real-time, interactive access to clinical outcomes data for the surgical program. A mixed-method, two-phase study was conducted to collect user feedback. A group of designers (N = 3) and a purposeful sample of users (N = 12) were recruited. The qualitative data were analyzed thematically. The dashboards were compared using the System Usability Scale (SUS) and qualitative data. RESULTS: The participating users gave an average SUS score of 82.9 on the new dashboard and 63.5 on the existing dashboard (p = 0.006). The participants achieved high task accuracy when using the new dashboard. The qualitative analysis revealed three opportunities for improvement. The data analysis and triangulation provided empirical evidence to the mismatched mental models. CONCLUSION: We conducted a mixed-method usability study on an interactive surgical dashboard and identified areas of improvements. Our study design can be an effective and efficient way to evaluate visual analytics systems in health care. We encourage researchers and practitioners to conduct user-centered evaluation and implement education plans to mitigate potential usability challenges and increase user satisfaction and adoption.

Development of a clinician-parent home care education intervention.

OBJECTIVES: To examine the strengths and opportunities for improvement of current home care education practices to inform the development of the Home Care for Heart Health intervention, and to develop a web-based intervention for parents and clinicians with complimentary print materials that could provide the right education at the right time to foster a safer transition from hospital to home. METHODS: An inter-professional focus group of parents, clinicians, and designers was formed to co-create a home care education intervention for parents of children with congenital heart disease (CHD) and their care team. We used the Integrated New Product Development process model created by Jonathon Cagan and Craig Vogel at Carnegie Mellon University to develop the intervention. This process model is a way of thinking that combines horizontal and inter-disciplinary teams, stakeholder-centric focus, and a system of qualitative discovery and development evolving towards quantitative methods of refinement. RESULTS: Our team developed the Home Care for Heart Health intervention. The evidenced-based intervention includes a quick reference guide for parents of children with CHD, an accompanying app, family-friendly pathways, and clinician education. CONCLUSION: Using an inter-professional approach, our team of clinicians, parents, and design experts were able to co-create a clinician-parent home care education intervention with broad application and lifelong relevance to the Congenital Heart Disease Community. PRACTICE IMPLICATIONS: Our intervention has the potential to be used as a model for other home care education interventions for parents of children with chronic illnesses.

Development of a System to Measure and Improve Outcomes in Congenital Heart Disease: Heart Institute Safety, Quality, and Value Program.

Achieving excellent outcomes for patients with congenital heart disease requires coordinated effort and resources, and a need has developed for a structure that facilitates improvement and measures cardiac centers' progress toward optimal patient care. METHODS: The Heart Institute (HI) at Cincinnati Children's Hospital developed a Safety, Quality and Value (SQV) program to formalize the use of quality improvement (QI) methods with the goal of optimizing patient outcomes, experience, and value. The SQV program adopted a conceptual framework that considers aspects of structure, process, outcome, and value in defining quality metrics, and the program used the Model for Improvement to guide design and implementation of QI interventions. RESULTS: In the first four years since its inception, the SQV program facilitated important improvements in clinical outcomes, cost reductions, and safety. In addition to achieving measurable improvements, the creation of a formal SQV program fostered a culture of transparency and accountability, providing a new structure for how the HI shares clinical data among clinicians, hospital leadership, and the public. CONCLUSION: The creation of an infrastructure to strategically design, implement, and support QI efforts in a clinically busy pediatric acquired and congenital heart institute was successful in meeting its initial aims and is a promising approach and model for other programs.

Power of a Learning Network in Congenital Heart Disease.

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

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.

Predicting long-term mortality after Fontan procedures: A risk score based on 6707 patients from 28 studies.

BACKGROUND: Reported long-term outcome measures vary greatly between studies in Fontan patients making comprehensive appraisal of mortality hazard challenging. We sought to create a clinical risk score to assist monitoring of Fontan patients in the outpatient setting. METHODS: A systematic review was conducted to evaluate risk factors for long-term (beyond the first postoperative year) mortality in Fontan patients. Studies were eligible for inclusion if ≥90 patients were included or ≥20 long-term mortalities we reported. Risk factors for long-term mortality were determined. The pooled hazard ratios were used to create components of a clinical score for long-term mortality using meta-analysis techniques. RESULTS: Twenty-eight studies were included. The total number of patients was 6707 with an average follow-up of 8.23 ± 5.42 years. There were 1000 deaths. Thirty-five risk factors for late mortality were identified and classified into 9 categories and their relative hazards were used to derive the initial components of a weighted, practical and clinically based Fontan risk score (ranging from 0 to 100). The final score included 8 risk factors: anatomic risk factors, elevated preoperative pulmonary artery pressure, atriopulmonary Fontan, heart failure symptoms, arrhythmia, moderate/severe ventricular dysfunction or atrioventricular valve regurgitation, protein losing enteropathy, and end organ disease (cirrhosis or renal insufficiency). CONCLUSION: In patients with Fontan circulation, the influence of readily available risk factors can be quantified in an integer score to predict long-term mortality. Prospective validation and refinement of this risk score will be undertaken.

Factors associated with long-term mortality after Fontan procedures: a systematic review.

BACKGROUND: Despite an ageing Fontan population, data on late outcomes are still scarce. Reported outcome measures and determinants vary greatly between studies making comprehensive appraisal of mortality hazard challenging. METHODS: We conducted a systematic review to evaluate causes and factors associated with late mortality in patients with Fontan circulation. Late mortality was defined as mortality beyond the first postoperative year. Studies were included if they had ≥90 patients or ≥20 late mortalities and/or transplants. Studies with overlapping patients were rationalised to include only the most recent studies to avoid duplication. RESULTS: From 28 studies, a total of 6707 patients with an average follow-up time of 8.23±5.42 years was identified. There were 1000 deaths. Causes of late death were reported in 697 cases. The five most common causes were heart/Fontan failure (22%), arrhythmia (16%), respiratory failure (15%), renal disease (12%) and thrombosis/bleeding (10%). Factors associated with late mortality were evaluated and classified into 9 categories. CONCLUSIONS: Causes and factors associated with late mortality after the Fontan operation are summarised in this study. The presented information will aid in identifying patients at highest risk for mortality and guide our risk stratification efforts in this patient population.

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

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

Using Improvement Methodology to Optimize Echocardiographic Imaging of Coronary Arteries in Children.

BACKGROUND: According to the American Society of Echocardiography, coronary artery (CA) imaging is recommended in pediatric examinations to identify CA anomalies. A review of the authors' center's echocardiographic studies revealed that CA images were often nondiagnostic. The aim of this study was to utilize quality improvement methodology to increase the percentage of first-time pediatric studies with definitive CA identification from a baseline of 45% to a goal of at least 75% in 9 months. METHODS: A scoring system was developed to characterize the completeness of CA imaging. One point was scored for demonstration of each of the following: right CA origin by two-dimensional imaging, right CA origin by color flow Doppler imaging, left CA origin by two-dimensional imaging, and left CA origin by color flow Doppler imaging. A score of 4 was considered to represent definitive imaging. A baseline was obtained on 100 first-time echocardiograms with normal findings. During the intervention, 10 randomly selected first-time studies with normal findings were scored weekly for assessment of CA imaging. Interventions were focused on the following domains: excellence in image quality, shared ownership, transparency, and effective communication. Key interventions included labeling CA images, requiring two-dimensional and color Doppler images, optimization of settings, and elimination of macros for CA reporting. RESULTS: The percentage of definitive CA identification increased from 45% to 82.5% over 4 months and was sustained for 7 months. Accurate reporting of incomplete CA imaging increased from 17% to 77.5%. CONCLUSIONS: Improved pediatric CA imaging and reporting were achieved through the implementation of key interventions.