Rationale and Design of the Randomized COmparison of Methods for Pulmonary Blood Flow Augmentation: Shunt Versus Stent (COMPASS) Trial: A Pediatric Heart Network Study.

Neonates with congenital heart disease and ductal-dependent pulmonary blood flow (DD-PBF) require early intervention. Historically, this intervention was most often a surgical systemic-to-pulmonary shunt (SPS; eg, Blalock-Thomas-Taussig shunt). However, over the past two decades, an alternative to SPS has emerged in the form of transcatheter ductal artery stenting (DAS). While many reports have indicated safety and durability of the DAS approach, few studies compare outcomes between DAS and SPS. The reports that do exist are comprised primarily of small-cohort single-center reviews. Two multicenter retrospective studies suggest that DAS is associated with similar or superior survival compared with SPS. These studies offer the best evidence to-date, and yet both have important limitations. The authors describe herein the rationale and design of the COMPASS (COmparison of Methods for Pulmonary blood flow Augmentation: Shunt vs Stent [COMPASS]) Trial (NCT05268094, IDE G210212). The COMPASS Trial aims to randomize 236 neonates with DD-PBF to either DAS or SPS across approximately 27 pediatric centers in North America. The goal of this trial is to compare important clinical outcomes between DAS and SPS over the first year of life in a cohort of neonates balanced by randomization in order to assess whether one method of palliation demonstrates therapeutic superiority.

Associations between Child Opportunity Index and Pediatric Cardiac Surgical Outcomes.

OBJECTIVE: To assess the relationship between the Child Opportunity Index (COI), a comprehensive measurement of social determinants of health, and specific COI domains on patient-specific outcomes following congenital cardiac surgery in the metropolitan region of Atlanta, Georgia. STUDY DESIGN: In this retrospective chart review, we included patients who underwent an index operation for congenital heart disease between 2010 and 2020 in a single pediatric health care system. Patients' addresses were geocoded and mapped to census tracts. Descriptive statistics, univariable analysis, and multivariable regression models were employed to assess associations between variables and outcomes. RESULTS: Of the 7460 index surgeries, 3798 (51%) met eligibility criteria. Presence of an adverse outcome, defined as either mortality or 1 of several other major postoperative morbidities, was significantly associated with COI in the univariable model (P = .008), but not the multivariable regression model (P = .39). Postoperative hospital length of stay was significantly associated with COI (P < .001) in univariable and multivariable regression models. There was no significant association between COI and readmission within 30 days of hospital discharge in univariable (P < .094) and multivariable (P = .49) models. CONCLUSION: COI is associated with postoperative hospital length of stay but not all outcomes in patients after congenital heart surgery. By understanding the role of COI in outcomes related to cardiac surgery, targeted interventions can be developed to improve health equity.

Fetal growth and gestational age improve outcome predictions in neonatal heart surgery.

OBJECTIVE: Current risk adjustment models for congenital heart surgery do not fully incorporate multiple factors unique to neonates such as granular gestational age (GA) and birth weight (BW) z score data. This study sought to develop a Neonatal Risk Adjustment Model for congenital heart surgery to address these deficiencies. METHODS: Cohort study of neonates undergoing cardiothoracic surgery during the neonatal period captured in the Pediatric Cardiac Critical Care Consortium database between 2014 and 2020. Candidate predictors were included in the model if they were associated with mortality in the univariate analyses. GA and BW z score were both added as multicategory variables. Mortality probabilities were predicted for different GA and BW z scores while keeping all other variables at their mean value. RESULTS: The C statistic for the mortality model was 0.8097 (95% confidence interval, 0.7942-0.8255) with excellent calibration. Mortality prediction for a neonate at 40 weeks GA and a BW z score 0 to 1 was 3.5% versus 9.8% for the same neonate at 37 weeks GA and a BW z score -2 to -1. For preterm infants the mortality prediction at 34 to 36 weeks with a BW z score 0 to 1 was 10.6%, whereas it was 36.1% for the same infant at <32 weeks with a BW z score of -2 to -1. CONCLUSIONS: This Neonatal Risk Adjustment Model incorporates more granular data on GA and adds the novel risk factor BW z score. These 2 factors refine mortality predictions compared with traditional risk models. It may be used to compare outcomes across centers for the neonatal population.

Predicting and Surviving Prolonged Critical Illness After Congenital Heart Surgery.

OBJECTIVES: Prolonged critical illness after congenital heart surgery disproportionately harms patients and the healthcare system, yet much remains unknown. We aimed to define prolonged critical illness, delineate between nonmodifiable and potentially preventable predictors of prolonged critical illness and prolonged critical illness mortality, and understand the interhospital variation in prolonged critical illness. DESIGN: Observational analysis. SETTING: Pediatric Cardiac Critical Care Consortium clinical registry. PATIENTS: All patients, stratified into neonates (≤28 d) and nonneonates (29 d to 18 yr), admitted to the pediatric cardiac ICU after congenital heart surgery at Pediatric Cardiac Critical Care Consortium hospitals. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: There were 2,419 neonates and 10,687 nonneonates from 22 hospitals. The prolonged critical illness cutoff (90th percentile length of stay) was greater than or equal to 35 and greater than or equal to 10 days for neonates and nonneonates, respectively. Cardiac ICU prolonged critical illness mortality was 24% in neonates and 8% in nonneonates (vs 5% and 0.4%, respectively, in nonprolonged critical illness patients). Multivariable logistic regression identified 10 neonatal and 19 nonneonatal prolonged critical illness predictors within strata and eight predictors of mortality. Only mechanical ventilation days and acute renal failure requiring renal replacement therapy predicted prolonged critical illness and prolonged critical illness mortality in both strata. Approximately 40% of the prolonged critical illness predictors were nonmodifiable (preoperative/patient and operative factors), whereas only one of eight prolonged critical illness mortality predictors was nonmodifiable. The remainders were potentially preventable (postoperative critical care delivery variables and complications). Case-mix-adjusted prolonged critical illness rates were compared across hospitals; six hospitals each had lower- and higher-than-expected prolonged critical illness frequency. CONCLUSIONS: Although many prolonged critical illness predictors are nonmodifiable, we identified several predictors to target for improvement. Furthermore, we observed that complications and prolonged critical care therapy drive prolonged critical illness mortality. Wide variation of prolonged critical illness frequency suggests that identifying practices at hospitals with lower-than-expected prolonged critical illness could lead to broader quality improvement initiatives.

Quality Improvement Basics: A Crash Course for Pediatric Cardiac Care.

BACKGROUND: Lack of knowledge of quality improvement (QI) methodology and change management principles can explain many of the difficulties encountered when trying to develop effective QI initiatives in health care. METHODS: An interactive QI workshop at the 14th Annual Meeting of the Pediatric Cardiac Intensive Care Society provided an overview of the role of QI in health care, basic QI frameworks and tools, and leadership and organizational culture pitfalls. The top five QI projects submitted to the meeting were later presented to an expert QI panel in a separate session to illustrate examples of QI principles. RESULTS: Workshop presenters introduced two major QI methodologies used to design QI projects. Important first steps include identifying a problem, forming a multidisciplinary team, and developing an aim statement. Key driver diagrams were highlighted as an important tool to develop a project's framework. Several diagnostic tools used to understand the problem were discussed, including the "5 Why's," cause-and-effect charts, and process flowcharts. The importance of outcome, process, and balancing measures was emphasized. Identification of interventions, the value of plan-do-study-act cycles to fuel continuous QI, and use of statistical process control, including run charts or control charts, were reviewed. The importance of stakeholder engagement, transparency, and sustainability was discussed. Later, the top five QI projects presented highlighted multiple "QI done well" practices discussed during the preconference QI workshop. CONCLUSIONS: Understanding QI methodology and appropriately applying basic QI tools are pivotal steps to realizing meaningful and sustained improvement.

Variation in Adjusted Mortality for Medical Admissions to Pediatric Cardiac ICUs.

OBJECTIVES: Pediatric cardiac ICUs should be adept at treating both critical medical and surgical conditions for patients with cardiac disease. There are no case-mix adjusted quality metrics specific to medical cardiac ICU admissions. We aimed to measure case-mix adjusted cardiac ICU medical mortality rates and assess variation across cardiac ICUs in the Pediatric Cardiac Critical Care Consortium. DESIGN: Observational analysis. SETTING: Pediatric Cardiac Critical Care Consortium clinical registry. PATIENTS: All cardiac ICU admissions that did not include cardiac surgery. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: The primary endpoint was cardiac ICU mortality. Based on multivariable logistic regression accounting for clustering, we created a case-mix adjusted model using variables present at cardiac ICU admission. Bootstrap resampling (1,000 samples) was used for model validation. We calculated a standardized mortality ratio for each cardiac ICU based on observed-to-expected mortality from the fitted model. A cardiac ICU was considered a statistically significant outlier if the 95% CI around the standardized mortality ratio did not cross 1. Of 11,042 consecutive medical admissions from 25 cardiac ICUs (August 2014 to May 2017), the observed mortality rate was 4.3% (n = 479). Final model covariates included age, underweight, prior surgery, time of and reason for cardiac ICU admission, high-risk medical diagnosis or comorbidity, mechanical ventilation or extracorporeal membrane oxygenation at admission, and pupillary reflex. The C-statistic for the validated model was 0.87, and it was well calibrated. Expected mortality ranged from 2.6% to 8.3%, reflecting important case-mix variation. Standardized mortality ratios ranged from 0.5 to 1.7 across cardiac ICUs. Three cardiac ICUs were outliers; two had lower-than-expected (standardized mortality ratio <1) and one had higher-than-expected (standardized mortality ratio >1) mortality. CONCLUSIONS: We measured case-mix adjusted mortality for cardiac ICU patients with critical medical conditions, and provide the first report of variation in this quality metric within this patient population across Pediatric Cardiac Critical Care Consortium cardiac ICUs. This metric will be used by Pediatric Cardiac Critical Care Consortium cardiac ICUs to assess and improve outcomes by identifying high-performing (low-mortality) centers and engaging in collaborative learning.

Hypoplastic left heart syndrome: from fetus to fontan.

The care of children with hypoplastic left heart syndrome is constantly evolving. Prenatal diagnosis of hypoplastic left heart syndrome will aid in counselling of parents, and selected fetuses may be candidates for in utero intervention. Following birth, palliation can be undertaken through staged operations: Norwood (or hybrid) in the 1st week of life, superior cavopulmonary connection at 4-6 months of life, and finally total cavopulmonary connection (Fontan) at 2-4 years of age. Children with hypoplastic left heart syndrome are at risk of circulatory failure their entire life, and selected patients may undergo heart transplantation. In this review article, we summarise recent advances in the critical care management of patients with hypoplastic left heart syndrome as were discussed in a focused session at the 12th International Conference of the Paediatric Cardiac Intensive Care Society held on 9 December, 2016, in Miami Beach, Florida.

Epidemiology of Noninvasive Ventilation in Pediatric Cardiac ICUs.

OBJECTIVE: To describe the epidemiology of noninvasive ventilation therapy for patients admitted to pediatric cardiac ICUs and to assess practice variation across hospitals. DESIGN: Retrospective cohort study using prospectively collected clinical registry data. SETTING: Pediatric Cardiac Critical Care Consortium clinical registry. PATIENTS: Patients admitted to cardiac ICUs at PC4 hospitals. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: We analyzed all cardiac ICU encounters that included any respiratory support from October 2013 to December 2015. Noninvasive ventilation therapy included high flow nasal cannula and positive airway pressure support. We compared patient and, when relevant, perioperative characteristics of those receiving noninvasive ventilation to all others. Subgroup analysis was performed on neonates and infants undergoing major cardiovascular surgery. To examine duration of respiratory support, we created a casemix-adjustment model and calculated adjusted mean durations of total respiratory support (mechanical ventilation + noninvasive ventilation), mechanical ventilation, and noninvasive ventilation. We compared adjusted duration of support across hospitals. The cohort included 8,940 encounters from 15 hospitals: 3,950 (44%) received noninvasive ventilation and 72% were neonates and infants. Medical encounters were more likely to include noninvasive ventilation than surgical. In surgical neonates and infants, 2,032 (55%) received postoperative noninvasive ventilation. Neonates, extracardiac anomalies, single ventricle, procedure complexity, preoperative respiratory support, mechanical ventilation duration, and postoperative disease severity were associated with noninvasive ventilation therapy (p < 0.001 for all). Across hospitals, noninvasive ventilation use ranged from 32% to 65%, and adjusted mean noninvasive ventilation duration ranged from 1 to 4 days (3-d observed mean). Duration of total adjusted respiratory support was more strongly correlated with duration of mechanical ventilation compared with noninvasive ventilation (Pearson r = 0.93 vs 0.71, respectively). CONCLUSIONS: Noninvasive ventilation use is common in cardiac ICUs, especially in patients admitted for medical conditions, infants, and those undergoing high complexity surgery. We observed wide variation in noninvasive ventilation use across hospitals, though the primary driver of total respiratory support time seems to be duration of mechanical ventilation.

Perioperative feeding management of neonates with CHD: analysis of the Pediatric Cardiac Critical Care Consortium (PC4) registry.

UNLABELLED: Introduction The optimal perioperative feeding strategies for neonates with CHD are unknown. In the present study, we describe the current feeding practices across a multi-institutional cohort. METHODS: Inclusion criteria for this study were as follows: all neonates undergoing cardiac surgery admitted to the cardiac ICU for ⩾24 hours preoperatively between October, 2013 and July, 2014 in the Pediatric Cardiac Critical Care Consortium registry. RESULTS: The cohort included 251 patients from eight centres. The most common diagnoses included the following: hypoplastic left heart syndrome (17%), coarctation/aortic arch hypoplasia (18%), and transposition of the great arteries (22%); 14% of the patients were <37weeks of gestational age. The median total hospital length of stay was 21 days (interquartile range (IQR) 14-35) and overall mortality was 8%. Preoperative feeding occurred in 133 (53%) patients. The overall preoperative feeding rates across centres ranged from 29 to 79%. Postoperative feeds started on median day 2 (IQR 1-4); for patients with hypoplastic left heart syndrome postoperative feeds started on median day 4. Postoperative feeds were initiated in 89 (35%) patients before extubation (range across centres: 21-61%). The median cardiac ICU discharge feeding volume was 108 cc/kg/day, varying across centres. The mean discharge weight was 280 g above birth weight, ranging from +100 to 430 g across centres. A total of 110 (44%) patients had discharge feeding tubes, ranging from 6 to 80% across centres, and 40/110 patients had gastrostomy/enterostomy tubes placed. In addition, eight (3.2%) patients developed necrotising enterocolitis - three preoperatively and five postoperatively. CONCLUSION: In this cohort, neonatal feeding practices and outcomes appear to vary across diagnostic groups and institutions. Only half of the patients received preoperative enteral nutrition; almost half had discharge feeding tubes. Multi-institutional collaboration is necessary to determine feeding strategies associated with best clinical outcomes.

Heterotaxy Syndrome: Proceedings From the 10th International PCICS Meeting.

A session dedicated to heterotaxy syndrome was included in the program of the Tenth International Conference of the Pediatric Cardiac Intensive Care Society in Miami, Florida in December 2014. An invited panel of experts reviewed the anatomic considerations, surgical considerations, noncardiac issues, and long-term outcomes in this challenging group of patients. The presentations, summarized in this article, reflect the current approach to this complex multiorgan syndrome and highlight future areas of clinical interest and research.

Collaborative quality improvement in the cardiac intensive care unit: development of the Paediatric Cardiac Critical Care Consortium (PC4).

Despite many advances in recent years for patients with critical paediatric and congenital cardiac disease, significant variation in outcomes remains across hospitals. Collaborative quality improvement has enhanced the quality and value of health care across specialties, partly by determining the reasons for variation and targeting strategies to reduce it. Developing an infrastructure for collaborative quality improvement in paediatric cardiac critical care holds promise for developing benchmarks of quality, to reduce preventable mortality and morbidity, optimise the long-term health of patients with critical congenital cardiovascular disease, and reduce unnecessary resource utilisation in the cardiac intensive care unit environment. The Pediatric Cardiac Critical Care Consortium (PC4) has been modelled after successful collaborative quality improvement initiatives, and is positioned to provide the data platform necessary to realise these objectives. We describe the development of PC4 including the philosophical, organisational, and infrastructural components that will facilitate collaborative quality improvement in paediatric cardiac critical care.

The relationship between in-house attending coverage and nighttime extubation following congenital heart surgery*.

OBJECTIVES: Many cardiac ICUs have instituted 24/7 attending physician in-house coverage, which theoretically may allow for more expeditious weaning from ventilation and extubation. We aimed to determine whether this staffing strategy impacts rates of nighttime extubation and duration of mechanical ventilation. DESIGN: National data were obtained from the Virtual PICU System database for all patients admitted to the cardiac ICU following congenital heart surgery in 2011 who required postoperative mechanical ventilation. Contemporaneous data from our local institution were collected in addition to the Virtual PICU System data. The combined dataset (n = 2,429) was divided based on the type of nighttime staffing model in order to compare rates of nighttime extubation and duration of mechanical ventilation between units that used an in-house attending staffing strategy and those that employed nighttime residents, fellows, or midlevel providers only. MEASUREMENTS AND MAIN RESULTS: Institutions that currently use 24/7 in-house attending coverage did not demonstrate statistically significant differences in rates of nighttime extubation or the duration of mechanical ventilation in comparison to units without in-house attendings. Younger patients cared for in non-in-house attending units were more likely to require reintubation. CONCLUSIONS: Pediatric patients who have undergone congenital heart surgery can be safely and effectively extubated without the routine presence of an attending physician. The utilization of nighttime in-house attending coverage does not appear to have significant benefits on the rate of nighttime extubation and may not reduce the duration of mechanical ventilation in units that already use in-house residents, fellows, or other midlevel providers.

Early renal replacement therapy during pediatric cardiac extracorporeal support increases mortality.

BACKGROUND: Acute kidney injury is a common comorbidity for children placed on extracorporeal membrane oxygenation (ECMO) because of primary cardiac disease. Continuous venovenous hemofiltration (CVVH) can optimize fluid status and lessen inflammatory response during ECMO. However, published data are derived primarily from children without primary cardiac disease. METHODS: A retrospective analysis of our institutional ECMO database from 2002 to 2011 was performed. To limit the bias that CVVH initiation was after evidence of end-organ injury, we considered "early CVVH" to be instituted within 48 hours of ECMO initiation. Multivariate logistic regression was undertaken to adjust for covariates. RESULTS: Of 153 cardiac ECMO patients, 59 (39%) received early CVVH. Time from ECMO initiation to CVVH initiation was 1.7±0.7 days (median 1 day). Pre-ECMO and post-ECMO serum creatinine levels were similar in both groups. However, peak serum creatinine was 1.1±0.4 mg/dL (median 1.0 mg/dL) in the ECMO and CVVH group and 0.9±0.4 mg/dL (median 0.8 mg/dL) in the ECMO alone group (p=0.003). Patients who received CVVH had a higher mortality (p<0.0001), were less likely to have had ECPR (p=0.004), and had a longer duration on ECMO (p<0.0001). In multivariate analysis subjects receiving CVVH support within 48 hours of ECMO cannulation were 3 times more likely to die during their hospitalization (odds ratio 3.02; 95% confidence interval 1.32 to 6.9, p=0.009) after adjusting for other significant risk factors. CONCLUSIONS: Early CVVH in pediatric cardiac patients requiring ECMO is associated with increased mortality. Early CVVH in the cardiac ECMO population does not appear justified.

Cryoablation for atrioventricular nodal reentrant tachycardia in young patients: predictors of recurrence.

BACKGROUND: Recurrence rates of atrioventricular nodal reentry tachycardia (AVNRT) after cryoablation continue to remain high despite excellent initial success rates. Our objective was to evaluate the clinical outcomes of cryoablation for AVNRT with the 4-mm and 6-mm tip cryoablation catheters in a young population and to elicit predictors of arrhythmia recurrence. METHODS: We retrospectively reviewed all patients who underwent cryoablation for AVNRT at the UCSF/Stanford Pediatric Arrhythmia Center from January 2004 to February 2007. RESULTS: One hundred fifty-four patients (age 13.7 years (3.2-24.4)) underwent cryoablation for AVNRT of which 144 patients had inducible AVNRT (123 sustained and 21 nonsustained) and 10 had presumed AVNRT. Initial success was achieved in 95% (146/154), with no difference between the 4-mm (93%) and 6-mm (98%) cryoablation catheter tips (P = 0.15). There was no permanent atrioventricular (AV) block. Transient third-degree AV block occurred in nine patients (6%), with no difference between the 4-mm (4%) and 6-mm (9%) tip (P = 0.13). AVNRT recurrence was documented in 14% in a median time of 2.5 months (0.25-20). Recurrences were lower with the 6-mm (9%) versus the 4-mm (18%) tip, but this did not reach statistical significance (P = 0.16). With univariate analysis, a longer fluoroscopy time was the only significant factor associated with recurrence. Multivariate analysis failed to identify any significant predictor of AVNRT recurrence. CONCLUSION: Outcomes of cryoablation for AVNRT continue to be good without the complication of AV block. We could not identify any specific parameter associated with AVNRT recurrence. Further improvements in cryoablation technique will be necessary to reduce recurrences.

Differential effects of amrinone and milrinone upon myocardial inflammatory signaling.

BACKGROUND: Mounting evidence links systemic and local inflammatory cytokine production to myocardial dysfunction and injury occurring during ischemia-reperfusion, cardiopulmonary bypass, and heart failure. Phosphodiesterase inhibitors (PDEIs), used frequently in these states, can modulate inflammatory signaling. The mechanisms for these effects are unclear. We therefore examined the effects of 2 commonly used PDEIs, amrinone and milrinone, on cardiac cell inflammatory responses. METHODS AND RESULTS: Primary rat cardiomyocyte cultures were treated with endotoxin (LPS) or tumor necrosis factor-alpha (TNF-alpha), alone or in the presence of clinically relevant concentrations of amrinone or milrinone. Regulation of nuclear factor-kappa B (NFkappaB), nitric oxide synthase and cyclooxygenase isoforms, and cytokine production were assessed by electrophoretic mobility shift assays, Western immunoblotting, and enzyme-linked immunoassays, respectively. Both LPS and TNF-alpha induced significant NFkappaB activation, cyclooxygenase-2 (COX-2) expression, and inducible NO synthase (iNOS) and cytokine production; with the exception of COX-2 expression, all were significantly reduced by amrinone, beginning at concentrations of 10 to 50 micro mol/L. In contrast, milrinone increased nuclear NFkappaB translocation, iNOS and COX-2 expression, and cardiomyocyte production of interleukin-1beta. Cell-permeable cAMP increased inflammatory gene expression, whereas cell-permeable cGMP had no effect, indicating that the effects of amrinone were not due to phosphodiesterase inhibition. Similar results were seen in macrophages and coronary vascular endothelial cells. CONCLUSIONS: Both amrinone and milrinone have significant effects on cardiac inflammatory signaling. Overall, amrinone reduces activation of the key transcription factor NFkappaB and limits the production of pro-inflammatory cytokines, whereas milrinone does not.