Diuretic response after neonatal cardiac surgery: a report from the NEPHRON collaborative.

BACKGROUND: Multicenter early diuretic response (DR) analysis of single furosemide dosing following neonatal cardiac surgery is lacking to inform whether early DR predicts adverse clinical outcomes. METHODS: We performed a retrospective cohort study utilizing data from the NEPHRON registry. Random forest machine learning generated receiver operating characteristic-area under the curve (ROC-AUC) and odds ratios for mechanical ventilation (MV) and respiratory support (RS). Prolonged MV and RS were defined using ≥ 90th percentile of observed/expected ratios. Secondary outcomes were prolonged CICU and hospital length of stay (LOS) and kidney failure (stage III acute kidney injury (AKI), peritoneal dialysis, and/or continuous kidney replacement therapy on postoperative day three) assessed using covariate-adjusted ROC-AUC curves. RESULTS: A total of 782 children were included. Cumulative urine output (UOP) metrics were lower in prolonged MV and RS patients, but DR poorly predicted prolonged MV (highest AUC 0.611, OR 0.98, sensitivity 0.67, specificity 0.53, p = 0.006, 95% OR CI 0.96-0.99 for cumulative 6-h UOP) and RS (highest AUC 0.674, OR 0.94, sensitivity 0.75, specificity 0.54, p < 0.001, 95% CI 0.91-0.97 UOP between 3 and 6 h). Secondary outcome results were similar. DR had fair discrimination for kidney failure (AUC 0.703, OR 0.94, sensitivity 0.63, specificity 0.71, 95% OR CI 0.91-0.98, p < 0.001, cumulative 6-h UOP). CONCLUSIONS: Early DR poorly discriminated patients with prolonged MV, RS, and LOS in this cohort, though it may identify severe postoperative AKI phenotype. Future work is warranted to determine if early DR or late postoperative DR later, in combination with other AKI metrics, may identify a higher-risk phenotype.

Peritoneal catheters in neonates undergoing complex cardiac surgery: a multi-centre descriptive study.

BACKGROUND: The use of peritoneal catheters for prophylactic dialysis or drainage to prevent fluid overload after neonatal cardiac surgery is common in some centres; however, the multi-centre variability and details of peritoneal catheter use are not well described. METHODS: Twenty-two-centre NEonatal and Pediatric Heart Renal Outcomes Network (NEPHRON) study to describe multi-centre peritoneal catheter use after STAT category 3-5 neonatal cardiac surgery using cardiopulmonary bypass. Patient characteristics and acute kidney injury/fluid outcomes for six post-operative days are described among three cohorts: peritoneal catheter with dialysis, peritoneal catheter with passive drainage, and no peritoneal catheter. RESULTS: Of 1490 neonates, 471 (32%) had an intraoperative peritoneal catheter placed; 177 (12%) received prophylactic dialysis and 294 (20%) received passive drainage. Sixteen (73%) centres used peritoneal catheter at some frequency, including six centres in >50% of neonates. Four centres utilised prophylactic peritoneal dialysis. Time to post-operative dialysis initiation was 3 hours [1, 5] with the duration of 56 hours [37, 90]; passive drainage cohort drained for 92 hours [64, 163]. Peritoneal catheter were more common among patients receiving pre-operative mechanical ventilation, single ventricle physiology, and higher complexity surgery. There was no association with adverse events. Serum creatinine and daily fluid balance were not clinically different on any post-operative day. Mortality was similar. CONCLUSIONS: In neonates undergoing complex cardiac surgery, peritoneal catheter use is not rare, with substantial variability among centres. Peritoneal catheters are used more commonly with higher surgical complexity. Adverse event rates, including mortality, are not different with peritoneal catheter use. Fluid overload and creatinine-based acute kidney injury rates are not different in peritoneal catheter cohorts.

Variation in hospital costs and resource utilisation after congenital heart surgery.

BACKGROUND: Children undergoing cardiac surgery have overall improving survival, though they consume substantial resources. Nationwide inpatient cost estimates and costs at longitudinal follow-up are lacking. METHODS: Retrospective cohort study of children <19 years of age admitted to Pediatric Health Information System administrative database with an International Classification of Diseases diagnosis code undergoing cardiac surgery. Patients were grouped into neonates (≤30 days of age), infants (31-365 days of age), and children (>1 year) at index procedure. Primary and secondary outcomes included hospital stay and hospital costs at index surgical admission and 1- and 5-year follow-up. RESULTS: Of the 99,670 cohort patients, neonates comprised 27% and had the highest total hospital costs, though daily hospital costs were lower. Mortality declined (5.6% in 2004 versus 2.5% in 2015, p < 0.0001) while inpatient costs rose (5% increase/year, p < 0.0001). Neonates had greater index diagnosis complexity, greater inpatient costs, required the greatest ICU resources, pharmacotherapy, and respiratory therapy. We found no relationship between hospital surgical volume, mortality, and hospital costs. Neonates had higher cumulative hospital costs at 1- and 5-year follow-up compared to infants and children. CONCLUSIONS: Inpatient hospital costs rose during the study period, driven primarily by longer stay. Neonates had greater complexity index diagnosis, required greater hospital resources, and have higher hospital costs at 1 and 5 years compared to older children. Surgical volume and in-hospital mortality were not associated with costs. Further analyses comprising merged clinical and administrative data are necessary to identify longer stay and cost drivers after paediatric cardiac surgery.

Epidemiology of Acute Kidney Injury After Neonatal Cardiac Surgery: A Report From the Multicenter Neonatal and Pediatric Heart and Renal Outcomes Network.

OBJECTIVES: Cardiac surgery-associated acute kidney injury occurs commonly following congenital heart surgery and is associated with adverse outcomes. This study represents the first multicenter study of neonatal cardiac surgery-associated acute kidney injury. We aimed to describe the epidemiology, including perioperative predictors and associated outcomes of this important complication. DESIGN: This Neonatal and Pediatric Heart and Renal Outcomes Network study is a multicenter, retrospective cohort study of consecutive neonates less than 30 days. Neonatal modification of The Kidney Disease Improving Global Outcomes criteria was used. Associations between cardiac surgery-associated acute kidney injury stage and outcomes (mortality, length of stay, and duration of mechanical ventilation) were assessed through multivariable regression. SETTING: Twenty-two hospitals participating in Pediatric Cardiac Critical Care Consortium. PATIENTS: Twenty-two-thousand forty neonates who underwent major cardiac surgery from September 2015 to January 2018. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Cardiac surgery-associated acute kidney injury occurred in 1,207 patients (53.8%); 983 of 1,657 in cardiopulmonary bypass patients (59.3%) and 224 of 583 in noncardiopulmonary bypass patients (38.4%). Seven-hundred two (31.3%) had maximum stage 1, 302 (13.5%) stage 2, 203 (9.1%) stage 3; prevalence of cardiac surgery-associated acute kidney injury peaked on postoperative day 1. Cardiac surgery-associated acute kidney injury rates varied greatly (27-86%) across institutions. Preoperative enteral feeding (odds ratio = 0.68; 0.52-0.9) and open sternum (odds ratio = 0.76; 0.61-0.96) were associated with less cardiac surgery-associated acute kidney injury; cardiopulmonary bypass was associated with increased cardiac surgery-associated acute kidney injury (odds ratio = 1.53; 1.01-2.32). Duration of cardiopulmonary bypass was not associated with cardiac surgery-associated acute kidney injury in the cardiopulmonary bypass cohort. Stage 3 cardiac surgery-associated acute kidney injury was independently associated with hospital mortality (odds ratio = 2.44; 1.3-4.61). No cardiac surgery-associated acute kidney injury stage was associated with duration of mechanical ventilation or length of stay. CONCLUSIONS: Cardiac surgery-associated acute kidney injury occurs frequently after neonatal cardiac surgery in both cardiopulmonary bypass and noncardiopulmonary bypass patients. Rates vary significantly across hospitals. Only stage 3 cardiac surgery-associated acute kidney injury is associated with mortality. Cardiac surgery-associated acute kidney injury was not associated with any other outcomes. Kidney Disease Improving Global Outcomes criteria may not precisely define a clinically meaningful renal injury phenotype in this population.

Higher total ultrafiltration volume during cardiopulmonary bypass-assisted infant cardiac surgery is associated with acute kidney injury and fluid overload.

BACKGROUND: Ultrafiltration (UF) is used for fluid removal during and after infant cardiopulmonary bypass (CPB) surgery to reduce fluid overload. Excessive UF may have the opposite of its intended effect, resulting in acute kidney injury (AKI), oliganuria, and fluid retention. METHODS: This is a single-center, retrospective review of infants treated with conventional and/or modified UF during CPB surgery. UF volume was indexed to weight. AKI was defined using serum creatinine "Kidney Disease Improving Global Outcome (KDIGO)" criteria. Fluid balance was defined according to: [Formula: see text]. Peak fluid overload was determined on postoperative day 3. Multivariable logistic regression adjusted for multiple covariates was used to explore associations with UF, AKI, and fluid overload. RESULTS: Five hundred thirty subjects < 1 year of age underwent CPB-assisted congenital heart surgery with UF. Sixty-four (12%) developed postoperative AKI. On multivariable regression, higher indexed total UF volume was associated with increased AKI risk (OR 1.11, 95% CI=1.04-1.19, p = 0.003). UF volume > 119.9 mL/kg did not reduce peak fluid overload. Subjects with AKI took longer to reach a negative fluid balance (2 vs. 3 days, p = 0.04). Those with more complex surgery were at highest AKI risk (STAT 3 [25-75 percentile: 3-4] in AKI group versus STAT 3 [25-75 percentile: 2-4] in non-AKI group, p = 0.05). AKI was reduced in subjects undergoing more complex surgery and treated with UF volume < 119.9 mL/kg. CONCLUSIONS: Judicious use of UF in more complex congenital cardiac surgery reduces the risk of AKI.

Hospital-Acquired Infection in Pediatric Subjects With Congenital Heart Disease Postcardiotomy Supported on Extracorporeal Membrane Oxygenation.

OBJECTIVE: To determine prevalence of and risk factors for infection in pediatric subjects with congenital heart disease status postcardiotomy supported on extracorporeal membrane oxygenation, as well as outcomes of these subjects. DESIGN: Retrospective cohort from the Extracorporeal Life Support Organization. SETTING: U.S. and international medical centers providing care to children with congenital heart disease status postcardiotomy. PATIENTS: Critically ill pediatric subjects less than 8 years old admitted to medical centers between January 1, 2013, and December 31, 2015, who underwent cardiac surgery for congenital heart disease and required extracorporeal membrane oxygenation support within the first 14 postoperative days. Subjects were excluded if they underwent orthotopic heart transplantation, required preoperative extracorporeal membrane oxygenation, and had more than one postoperative extracorporeal membrane oxygenation run. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: A total of 1,314 extracorporeal membrane oxygenation subject encounters in the Extracorporeal Life Support Organization registry met inclusion criteria. Neonates comprised 53% (n = 696) of the cohort, whereas infants made up 33% (n = 435). Of the 994 subjects with Society of Thoracic Surgeons-European Association for Cardio-Thoracic Surgery categorizable surgery, 33% (n = 325) were in Society of Thoracic Surgeons-European Association for Cardio-Thoracic Surgery category 4 and 23% (n = 231) in Society of Thoracic Surgeons-European Association for Cardio-Thoracic Surgery category 5. While on extracorporeal membrane oxygenation, 229 subjects (17%) acquired one or more extracorporeal membrane oxygenation-related infections, which represents an occurrence rate of 67 infections per 1,000 extracorporeal membrane oxygenation days. Gram-negative (62%) and Gram-positive (42%) infections occurred most commonly. Forty percent had positive blood cultures. Infants and children were at higher infection risk compared with neonatal subjects; subjects undergoing less complex surgery had higher infection rates. Unadjusted survival to hospital discharge was lower in infected subjects compared with noninfected subjects (43% vs 51%; p = 0.01). After adjusting for confounders via propensity matching, we identified no significant mortality difference between infected and noninfected subjects. CONCLUSIONS: Neonatal and pediatric subjects in this study have a high rate of acquired infection. Infants and children were at higher infection risk compared with neonatal subjects. There was not, however, a significant association between extracorporeal membrane oxygenation-related infection and survival to hospital discharge after propensity matching.

Pulse Oximetry Screening Has Not Changed Timing of Diagnosis or Mortality of Critical Congenital Heart Disease.

This study evaluates the effectiveness of mandatory pulse oximetry screening. The objective is to evaluate whether mandatory pulse oximetry testing had decreased the late critical congenital heart disease (CCHD) diagnosis rate and reduced mortality in neonatal subjects. This was a single center, retrospective cohort study comparing the timing of diagnosis of CCHD between neonates undergoing cardiac surgery in 2009-2010, prior to mandatory pulse oximetry screening, and neonates in 2015-2016, after mandatory pulse oximetry screening was instituted. Follow-up was for 1 year. We defined CCHD as lesions requiring surgical correction within 30 days of life. Exclusions included: pacemaker insertions, vascular ring divisions, closure of patent ductus arteriosus, arterial cutdown, or extracorporeal membrane oxygenation cannulation without structural heart disease as the sole procedure, or if subjects were born at home. Infants diagnosed prior to discharge from birth hospital were defined as early postnatal; late postnatal subjects were diagnosed after birth hospital discharge. In-hospital mortality and 1-year mortality were measured. A total of 527 neonates were included; 251 (47.6%) comprised the pre-mandatory pulse oximetry screening cohort (2009-2010). Only 3.6% of the 2009-2010 cohort and 4.3% of the 2015-2016 cohort were diagnosed late (p = 0.66). One-year mortality decreased during the study period (17.2% in 2009-2010 vs 10.5% in 2015-2016, p = 0.03). There were no deaths in the late CCHD diagnosis groups. Mandatory pulse oximetry screening legislation has not changed the late postnatal diagnosis rate at our institution. Mortality for neonatal CCHD has declined, but this decline is not attributable to mandatory pulse oximetry screening.

Neonatal and Paediatric Heart and Renal Outcomes Network: design of a multi-centre retrospective cohort study.

BACKGROUND: Cardiac surgery-associated acute kidney injury is common. In order to improve our understanding of acute kidney injury, we formed the multi-centre Neonatal and Pediatric Heart and Renal Outcomes Network. Our main goals are to describe neonatal kidney injury epidemiology, evaluate variability in diagnosis and management, identify risk factors, investigate the impact of fluid overload, and explore associations with outcomes. METHODS: The Neonatal and Pediatric Heart and Renal Outcomes Network collaborative includes representatives from paediatric cardiac critical care, cardiology, nephrology, and cardiac surgery. The collaborative sites and infrastructure are part of the Pediatric Cardiac Critical Care Consortium. An acute kidney injury module was developed and merged into the existing infrastructure. A total of twenty-two participating centres provided data on 100-150 consecutive neonates who underwent cardiac surgery within the first 30 post-natal days. Additional acute kidney injury variables were abstracted by chart review and merged with the corresponding record in the quality improvement database. Exclusion criteria included >1 operation in the 7-day study period, pre-operative renal replacement therapy, pre-operative serum creatinine >1.5 mg/dl, and need for extracorporeal support in the operating room or within 24 hours after the index operation. RESULTS: A total of 2240 neonatal patients were enrolled across 22 centres. The incidence of acute kidney injury was 54% (stage 1 = 31%, stage 2 = 13%, and stage 3 = 9%). CONCLUSIONS: Neonatal and Pediatric Heart and Renal Outcomes Network represents the largest multi-centre study of neonatal kidney injury. This new network will enhance our understanding of kidney injury and its complications.

Acute Kidney Injury After Pediatric Cardiac Surgery: A Secondary Analysis of the Safe Pediatric Euglycemia After Cardiac Surgery Trial.

OBJECTIVES: To understand the effect of tight glycemic control on cardiac surgery-associated acute kidney injury. DESIGN: Secondary analysis of data from the Safe Pediatric Euglycemia after Cardiac Surgery trial of tight glycemic control versus standard care. SETTING: Pediatric cardiac ICUs at University of Michigan, C.S. Mott Children's Hospital, and Boston Children's Hospital. PATIENTS: Children 0-36 months old undergoing congenital cardiac surgery. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Cardiac surgery-associated acute kidney injury was assigned using the Acute Kidney Injury Network criteria with the modification that a greater than 0.1 mg/dL increase in serum creatinine was required to assign cardiac surgery-associated acute kidney injury. We explored associations between cardiac surgery-associated acute kidney injury and tight glycemic control and clinical outcomes. Of 799 patients studied, cardiac surgery-associated acute kidney injury occurred in 289 patients (36%), most of whom had stage II or III disease (72%). Cardiac surgery-associated acute kidney injury rates were similar between treatment groups (36% vs 36%; p = 0.99). Multivariable modeling showed that patients with cardiac surgery-associated acute kidney injury were younger (p = 0.002), underwent more complex surgery (p = 0.005), and had longer cardiopulmonary bypass times (p = 0.002). Cardiac surgery-associated acute kidney injury was associated with longer mechanical ventilation and ICU and hospital stays and increased mortality. Patients at University of Michigan had higher rates of cardiac surgery-associated acute kidney injury compared with Boston Children's Hospital patients (66% vs 15%; p < 0.001), but University of Michigan patients with cardiac surgery-associated acute kidney injury had shorter time to extubation and ICU and hospital stays compared with Boston Children's Hospital patients. CONCLUSIONS: Tight glycemic control did not reduce the cardiac surgery-associated acute kidney injury rate in this trial cohort. We observed significant differences in cardiac surgery-associated acute kidney injury rates between the two study sites, and there was a differential effect of cardiac surgery-associated acute kidney injury on clinical outcomes by site. These findings warrant further investigation to identify causal variation in perioperative practices that affect cardiac surgery-associated acute kidney injury epidemiology.

An evaluation of the outcomes associated with peritoneal catheter use in neonates undergoing cardiac surgery: A multicenter study.

Objective: The study objective was to determine if intraoperative peritoneal catheter placement is associated with improved outcomes in neonates undergoing high-risk cardiac surgery with cardiopulmonary bypass. Methods: This propensity score-matched retrospective study used data from 22 academic pediatric cardiac intensive care units. Consecutive neonates undergoing Society of Thoracic Surgeons-European Association for Cardio-Thoracic Surgery category 3 to 5 cardiac surgery with cardiopulmonary bypass at centers participating in the NEonatal and Pediatric Heart Renal Outcomes Network collaborative were studied to determine the association of the use of an intraoperative placed peritoneal catheter for dialysis or passive drainage with clinical outcomes, including the duration of mechanical ventilation. Results: Among 1490 eligible neonates in the NEonatal and Pediatric Heart Renal Outcomes Network dataset, a propensity-matched analysis was used to compare 395 patients with peritoneal catheter placement with 628 patients without peritoneal catheter placement. Time to extubation and most clinical outcomes were similar. Postoperative length of stay was 5 days longer in the peritoneal catheter placement cohort (17 vs 22 days, P = .001). There was a 50% higher incidence of moderate to severe acute kidney injury in the no-peritoneal catheter cohort (12% vs 18%, P = .02). Subgroup analyses between specific treatments and in highest risk patients yielded similar associations. Conclusions: This study does not demonstrate improved outcomes among neonates with placement of a peritoneal catheter during cardiac surgery. Outcomes were similar apart from longer hospital stay in the peritoneal catheter cohort. The no-peritoneal catheter cohort had a 50% higher incidence of moderate to severe acute kidney injury (12% vs 18%). This analysis does not support indiscriminate peritoneal catheter use, although it may support the utility for postoperative fluid removal among neonates at risk for acute kidney injury. A multicenter controlled trial may better elucidate peritoneal catheter effects.

Hemodynamic and Echocardiographic Predictors of Mortality in Pediatric Patients on Venoarterial Extracorporeal Membrane Oxygenation: A Multicenter Investigation.

BACKGROUND: Venoarterial extracorporeal membrane oxygenation (ECMO) supports patients with advanced cardiac dysfunction; however, mortality occurs in a significant subset of patients. The authors performed a multicenter, prospective study to determine hemodynamic and echocardiographic predictors of mortality in children placed on ECMO for cardiac support. METHODS: Over 8 years, six heart centers prospectively assessed echocardiographic and hemodynamic variables on full and minimum ECMO flow. Sixty-three patients were enrolled, ranging in age from 1 day to 16 years. Hemodynamic measurements included heart rate, vasoactive inotropic score, arteriovenous oxygen difference, pulse pressure, and lactate. Echocardiographic variables included shortening fraction, ejection fraction (EF), right ventricular fractional area change, outflow tract Doppler-derived stroke distance (velocity-time integral [VTI]), and degree of atrioventricular valve regurgitation. Patients were stratified into two groups: those who were able to wean within 48 hours of assessment and survived without ventricular assist devices or orthotopic heart transplantation (successful wean group) and those with unsuccessful weaning. For each patient, variables were compared between full and minimum ECMO flow for each group. RESULTS: Thirty-eight patients (60%) formed the unsuccessful group (two with ventricular assist devices, four with orthotopic heart transplantation, 24 deaths), and 25 constituted the successful wean group. At minimum flow, higher EF (53 ± 16% vs 40 ± 20%, P = .0094), less mitral regurgitation (0.8 ± 0.9 vs 1.4 ± 0.9, P = .0329), and lower central venous pressure (12.0 ± 3.9 vs 14.7 ± 5.4 mm Hg), along with higher VTI (9.0 ± 2.9 vs 6.8 ± 3.7 cm, P = .0154), correlated successful weaning. A longer duration of ECMO (8 vs 5 days, P < .0002) was associated with unsuccessful weaning. Multivariate logistic regression predicted minimum-flow EF and VTI to independently predict successful weaning with cutoff values by receiver operating characteristic analysis of EF > 41% (area under the curve, 0.712; P = .0005) and VTI > 7.9 cm (area under the curve, 0.729; P = .0010). CONCLUSIONS: Diminished VTI or EF during ECMO weaning predicts the need for orthotopic heart transplantation or ventricular assist device support or death in children on ECMO for cardiac dysfunction. Increased postwean central venous pressure or mitral regurgitation along with a prolonged ECMO course also predicted these adverse outcomes. These measurements should be used to help discriminate which patients will require alternative methods of circulatory support for survival.

Fluid Accumulation After Neonatal Congenital Cardiac Operation: Clinical Implications and Outcomes.

BACKGROUND: This study was conducted to determine the association between fluid balance metrics and mortality and other postoperative outcomes after neonatal cardiac operation in a contemporary multicenter cohort. METHODS: This was an observational cohort study across 22 hospitals in neonates (≤30 days) undergoing cardiac operation. We explored overall percentage fluid overload, postoperative day 1 percentage fluid overload, peak percentage fluid overload, and time to first negative daily fluid balance. The primary outcome was in-hospital mortality. Secondary outcomes included postoperative duration of mechanical ventilation and intensive care unit (ICU) and hospital length of stay. Multivariable logistic or negative binomial regression was used to determine independent associations between fluid overload variables and each outcome. RESULTS: The cohort included 2223 patients. In-hospital mortality was 3.9% (n = 87). Overall median peak percentage fluid overload was 4.9% (interquartile range, 0.4%-10.5%). Peak percentage fluid overload and postoperative day 1 percentage fluid overload were not associated with primary or secondary outcomes. Hospital resource utilization increased on each successive day of not achieving a first negative daily fluid balance and was characterized by longer duration of mechanical ventilation (incidence rate ratio, 1.11; 95% CI, 1.08-1.14), ICU length of stay (incidence rate ratio, 1.08; 95% CI, 1.03-1.12), and hospital length of stay (incidence rate ratio, 1.09; 95% CI, 1.05-1.13). CONCLUSIONS: Time to first negative daily fluid balance, but not percentage fluid overload, is associated with improved postoperative outcomes in neonates after cardiac operation. Specific treatments to achieve an early negative fluid balance may decrease postoperative care durations.

Noncompaction of the left ventricular myocardium in a boy with a novel chromosome 8p23.1 deletion.

Interstitial deletion of chromosome 8p23.1 has been reported in patients with congenital heart defects, including atrial and ventricular septal defects, pulmonary stenosis, and complex cyanotic heart defects. GATA4, a zinc-finger transcription factor gene, has been localized to this region. GATA4 interacts with additional transcription factors in the embryogenesis of the primitive heart tube. Mutations in GATA4 are thought to be responsible for the congenital heart defects reported in association with this chromosomal deletion, and several familial point mutations leading to amino acid substitutions have also been identified. Left ventricular noncompaction (LVNC) is a clinically heterogeneous disorder characterized by LV myocardial trabeculations and intertrabecular recesses that communicate with the LV cavity. Patients may be asymptomatic or may present with evidence of severely depressed LV systolic and diastolic function. The LV may be dilated or hypertrophied, and clinical expression may be undulating. Several genetic causes of LVNC have been reported, with variable modes of inheritance, including autosomal dominant and X-linked inheritance, but relatively few responsible genes have been identified. A 12-year-old boy with a history of acute lymphoblastic leukemia, dysmorphic features, and LVNC with preserved LV systolic function was referred to the Cardiovascular Genetics Clinic at our institution. The patient was asymptomatic in terms of cardiovascular function. Chromosome microarray testing revealed an interstitial deletion in the region of 8p23.1 containing GATA4. LVNC has not been reported previously in association with this chromosome deletion. Further investigation into the role of GATA4 in patients with LVNC is warranted.

Development of Care Curves Following the Stage 1 Palliation: A Comparison of Intensive Care Among 5 Centers.

Background Comparison of care among centers is currently limited to major end points, such as mortality, length of stay, or complication rates. Creating "care curves" and comparing individual elements of care over time may highlight modifiable differences in intensive care among centers. Methods and Results We performed an observational retrospective study at 5 centers in the United States to describe key elements of postoperative care following the stage 1 palliation. A consecutive sample of 502 infants undergoing stage 1 palliation between January 2009 and December 2018 were included. All electronic health record entries relating to mandatory mechanical ventilator rate, opioid administration, and fluid intake/outputs between postoperative days (POD) 0 to 28 were extracted from each institution's data warehouse. During the study period, 502 patients underwent stage 1 palliation among the 5 centers. Patients were weaned to a median mandatory mechanical ventilator rate of 10 breaths/minute by POD 4 at Center 5 but not until POD 7 to 8 at Centers 1 and 2. Opioid administration peaked on POD 2 with extreme variance (median 6.9 versus 1.6 mg/kg per day at Center 3 versus Center 2). Daily fluid balance trends were variable: on POD 3 Center 1 had a median fluid balance of -51 mL/kg per day, ranging between -34 to 19 mL/kg per day among remaining centers. Intercenter differences persist after adjusting for patient and surgical characteristics (P<0.001 for each end point). Conclusions It is possible to detail and compare individual elements of care over time that represent modifiable differences among centers, which persist even after adjusting for patient factors. Care curves may be used to guide collaborative quality improvement initiatives.

Bronchoscopic and histologic findings during lymphatic intervention for plastic bronchitis.

BACKGROUND: Percutaneous lymphatic intervention (PCL) is a promising new therapy for plastic bronchitis (PB). We characterized bronchoalveolar lavage (BAL) and cast morphology in surgically repaired congenital heart disease (CHD) patients with PB during PCL. We quantified respiratory and bronchoscopic characteristics and correlated them with post-intervention respiratory outcomes. METHODS: We retrospectively reviewed patients with PB and surgically repaired CHD undergoing PCL and bronchoscopy at our institution. Pre-intervention characteristics, bronchoscopy notes, BAL cell counts, virology, and cultures were collected. A pathologist blinded to clinical data reviewed cast specimens. Respiratory outcomes were evaluated through standardized telephone questionnaire. RESULTS: Sixty-two patients were included with a median follow-up of 20 months. No patients experienced airway bleeding, obstruction, or prolonged intubation related to bronchoscopy. Of BAL infectious studies, the positive results were 4 (8%) fungal, 6 (11%) bacterial, and 6 (14%) viral. Median BAL count per 100 cells for neutrophils, lymphocytes, and eosinophils were 13, 10, and 0, respectively. Of 23 bronchial casts analyzed, all contained lymphocytes, and 19 (83%) were proteinaceous, with 14 containing neutrophils and/or eosinophils. Median BAL neutrophil count was greater in patients with proteinaceous neutrophilic or eosinophilic casts compared to casts without neutrophils or lymphocytes (P = 0.030). Post-intervention, there was a significant reduction in respiratory medications and support and casting frequency. CONCLUSIONS: The predominance of neutrophilic proteinaceous casts and high percentage of positive BAL infectious studies support short-term fibrinolytic and anti-infective therapies in PB in select patients. Flexible bronchoscopy enables safe assessment of cast burden. PCL effectively treats PB and reduces respiratory therapies.

The Epidemiology of Healthcare-associated Infections in Pediatric Cardiac Intensive Care Units.

BACKGROUND: Healthcare-associated infections (HAIs) represent serious complications for patients within pediatric cardiac intensive care units (CICU). HAIs are associated with increased morbidity, mortality and resource utilization. There are few studies describing the epidemiology of HAIs across the entire spectrum of patients (surgical and nonsurgical) receiving care in dedicated pediatric CICUs. METHODS: Retrospective analyses of 22,839 CICU encounters from October 2013 to September 2016 across 22 North American CICUs contributing data to the Pediatric Cardiac Critical Care Consortium clinical registry. RESULTS: HAIs occurred in 2.4% of CICU encounters at a rate of 3.3 HAIs/1000 CICU days, with 73% of HAIs occurring in children <1 year. Eighty encounters (14%) had ≥2 HAIs. Aggregate rates for the 4 primary HAIs are as follows: central line-associated blood stream infection, 1.1/1000 line days; catheter-associated urinary tract infections, 1.5/1000 catheter days; ventilator-associated pneumonia, 1.9/1000 ventilator days; surgical site infections, 0.81/100 operations. Surgical and nonsurgical patients had similar HAIs rates/1000 CICU days. Incidence was twice as high in surgical encounters and increased with surgical complexity; postoperative infection occurred in 2.8% of encounters. Prematurity, younger age, presence of congenital anomaly, Society of Thoracic Surgeons-European Association for Cardio-Thoracic Surgery Congenital Heart Surgery Mortality Categories (STAT) 4-5 surgery, admission with an active medical condition, open sternum and extracorporeal membrane oxygenation were independently associated with HAIs. In univariable analysis, HAI was associated with longer hospital length of stay and durations of urinary catheter, central venous catheter and ventilation. Mortality was 24.4% in patients with HAIs versus 3.4% in those without, P < 0.0001. CONCLUSIONS: We provide comprehensive multicenter benchmark data regarding rates of HAIs within dedicated pediatric CICUs. We confirm that although rare, HAIs of all types are associated with significant resource utilization and mortality.

Duration of Mechanical Ventilation and Perioperative Care Quality After Neonatal Cardiac Operations.

BACKGROUND: This study was conducted to determine whether the duration of mechanical ventilation (DOMV) could be used to benchmark the overall quality of care after neonatal congenital heart operations. METHODS: Children aged younger than 30 days undergoing cardiac operations were reviewed. Technical adequacy was assessed using the Technical Performance Score (TPS), a previously validated tool for determining the adequacy of a palliative or corrective surgical procedure that uses echocardiography criteria and need for unplanned reintervention to determine technical adequacy. Preoperative risk factors and postoperative complications were determined using The Society of Thoracic Surgeons Congenital Heart Surgery Database definitions. Surgical complexity was assessed using The Society of Thoracic Surgeons-European Association of Cardio-Thoracic Surgery (STAT) Mortality Categories. We explored associations between preoperative risk factors, STAT category, TPS, and postoperative complications with DOMV. RESULTS: Of 601 patients studied, 49 were not included in the multivariable analysis due to a STAT nonclassifiable operation or unmeasured TPS, or both. Multiple risk factors were associated with longer DOMV, including weight (p = 0.005), The Society of Thoracic Surgeons Congenital Heart Surgery Database preoperative factors (p = 0.005), STAT mortality category (p < 0.001), TPS (p < 0.001), and The Society of Thoracic Surgeons Congenital Heart Surgery Database-defined complications (p < 0.001). Multivariable regression showed that preoperative factors increased DOMV by 1.23 days (p = 0.01), class 3 TPS by 2.16 days (p < 0.001), and postoperative complications by 2.03 days (p < 0.001), with adjusted R2 = 0.42. CONCLUSIONS: Neonates with major residual lesions and postoperative complications have prolonged DOMV. DOMV may reflect the quality of care after congenital heart operations.

Lack of Furosemide Responsiveness Predicts Acute Kidney Injury in Infants After Cardiac Surgery.

BACKGROUND: This was a retrospective study to determine whether lack of furosemide responsiveness (LFR) predicts acute kidney injury (AKI) after cardiopulmonary bypass surgery in infants. METHODS: Infants (less than 1 year of age) undergoing cardiopulmonary bypass surgery, receiving routine postoperative furosemide (0.8 to 1.2 mg/kg per dose between 8 and 24 hours after surgery) were included. Urine output was measured 2 and 6 hours after furosemide dose. Lack of furosemide responsiveness was defined a priori as urine output less than 1 mL · kg-1 · h-1 after furosemide. Serum creatinine was corrected for fluid balance. Acute kidney injury was determined using changes in uncorrected and corrected serum creatinine. The predictive utility of LFR was assessed using receiver-operating characteristics curve analysis. RESULTS: We analyzed 568 infants who underwent cardiopulmonary bypass. Eighty-one (14.3%) had AKI using uncorrected serum creatinine; AKI occurred in 41 (7.2%) after correcting for fluid overload. Patients with AKI had a lower response to furosemide (median urine output 2 hours: 1.2 versus 3.4 mL · kg-1 · h-1, p = 0.01; median urine output 6 hours: 1.3 versus 2.9 mL · kg-1 · h-1, p = 0.01). After creatinine correction, LFR predicts AKI development (area under receiver-operating characteristics curve of 0.74 at 2 hours and 0.77 at 6 hours). After adjusting for surgical complexity using The Society of Thoracic Surgeons/European Association for Cardiothoracic Surgery mortality categories, the area under the receiver-operating characteristics curve was 0.74 at 2 hours and 0.81 at 6 hours. Patients with urine output greater than 1 mL · kg-1 · h-1 were unlikely to have AKI (negative predictive value, 97%). CONCLUSIONS: After correcting serum creatinine for fluid balance and adjusting for surgical complexity, LFR performs fairly at 2 hours, whereas at 6 hours, LFR is a good AKI predictor. Prospective studies are needed to validate whether diuretic responsiveness predicts AKI.