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.

Factors Associated With Mortality and Adverse Outcomes After Truncus Arteriosus Repair.

BACKGROUND: Truncus arteriosus repair is associated with higher morbidity and mortality compared with many other congenital heart operations. We sought to determine factors associated with mortality and adverse outcomes in infants undergoing truncus arteriosus repair. METHODS: We used the Pediatric Health Information System Database to identify infants aged < 90 days who underwent truncus arteriosus repair from 2004 to 2019. The primary outcome was hospital mortality. Secondary outcomes were prolonged postoperative length of stay (>30 days) and hospital readmission within 90 days. Multivariable logistic regression models were used to identify associated factors for adverse outcomes. RESULTS: A total of 1645 subjects were included. Hospital mortality occurred in 164 (10%). Factors independently associated with mortality included birth weight < 3 kg, admit age < 48 hours, truncal valve surgery, cardiac arrest, extracorporeal membrane oxygenation, acute kidney injury, cardiac catheterization, tracheostomy, and earlier era. Prolonged postoperative length of stay occurred in 508 patients (31%). Factors independently associated with prolonged postoperative length of stay included prematurity, DiGeorge syndrome, admit age < 48 hours, later surgical era, acute kidney injury, infection, cardiac catheterization, vocal cord paralysis, tracheostomy, and gastrostomy. Readmission within 90 days occurred in 511 of 1481 surviving patients (34%). DiGeorge syndrome, cleft lip/palate, cardiac catheterization, and extracorporeal membrane oxygenation were factors independently associated with hospital readmission. CONCLUSIONS: We identified multiple factors associated with hospital mortality and adverse outcomes in infants undergoing truncus arteriosus repair. This information is useful for quality improvement initiatives, perioperative counseling, and discharge planning.

Outcomes of Gastrostomy and Tracheostomy in Infants Undergoing Truncus Arteriosus Repair: Database Study Using the Pediatric Health Information System.

OBJECTIVES: We sought to determine the prevalence of and factors associated with gastrostomy tube placement and tracheostomy in infants undergoing truncus arteriosus repair, and associations between these procedures and outcome. DESIGN: Retrospective cohort study. SETTING: Pediatric Health Information System database. PATIENTS: Infants less than 90 days old who underwent truncus arteriosus repair from 2004 to 2019. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Multivariable logistic regression models were used to identify factors associated with gastrostomy tube and tracheostomy placement and to identify associations between these procedures and hospital mortality and prolonged postoperative length of stay (LOS; > 30 d). Of 1,645 subjects, gastrostomy tube was performed in 196 (11.9%) and tracheostomy in 56 (3.4%). Factors independently associated with gastrostomy tube placement were DiGeorge syndrome, congenital airway anomaly, admission age less than or equal to 2 days, vocal cord paralysis, cardiac catheterization, infection, and failure to thrive. Factors independently associated with tracheostomy congenital airway anomaly, truncal valve surgery, and cardiac catheterization. Gastrostomy tube was independently associated with prolonged postoperative LOS (odds ratio [OR], 12.10; 95% CI, 7.37-19.86). Hospital mortality occurred in 17 of 56 patients (30.4%) who underwent tracheostomy versus 147 of 1,589 patients (9.3%) who did not ( p < 0.001), and median postoperative LOS was 148 days in patients who underwent tracheostomy versus 18 days in those who did not ( p < 0.001). Tracheostomy was independently associated with mortality (OR, 3.11; 95% CI, 1.43-6.77) and prolonged postoperative LOS (OR, 9.85; 95% CI, 2.16-44.80). CONCLUSIONS: In infants undergoing truncus arteriosus repair, tracheostomy is associated with greater odds of mortality; while gastrostomy and tracheostomy are strongly associated with greater odds of prolonged postoperative LOS.

Optimal Fat-Modified Diet Duration for the Treatment of Postoperative Chylothorax in Children.

BACKGROUND: Dietary modification is the mainstay of treatment for postoperative chylothorax in children. However, optimal fat-modified diet (FMD) duration to prevent recurrence is unknown. Our aim was to determine the association between FMD duration and chylothorax recurrence. METHODS: Retrospective cohort study conducted across 6 pediatric cardiac intensive care units within the United States. Patients aged <18 years who developed chylothorax within 30 days after cardiac surgery between January 2020 and April 2022 were included. Patients with a Fontan palliation, who died, or were lost to follow-up or within 30 days of resuming a regular diet were excluded. FMD duration was defined as the first day of a FMD when chest tube output was <10 mL/kg/d without increasing until the resumption of a regular diet. Patients were classified into 3 groups (<3 weeks, 3-5 weeks, >5 weeks) based on FMD duration. RESULTS: A total of 105 patients were included: <3 weeks (n = 61) 3-5 weeks (n = 18), and >5 weeks (n = 26). Demographic, surgical, and hospitalization characteristics were not different across groups. In the >5 weeks group, chest tube duration was longer compared with the <3 weeks and 3-5 weeks groups (median, 17.5 days [interquartile range, 9-31] vs 10 and 10.5 days; P = .04). There was no recurrence of chylothorax within 30 days once chylothorax was resolving regardless of FMD duration. CONCLUSIONS: FMD duration was not associated with recurrence of chylothorax, suggesting that FMD duration can safely be shortened to at least <3 weeks from time of resolving chylothorax.

Contemporary Multicenter Outcomes for Truncus Arteriosus With Interrupted Aortic Arch.

BACKGROUND: Truncus arteriosus with interrupted aortic arch (TA-IAA) is a rare congenital heart defect with historically poor outcomes. Contemporary multicenter data are limited. METHODS: A retrospective cohort study of children who underwent repair of TA-IAA between 2009 and 2016 at 12 tertiary care referral centers within the United States was performed. Major adverse cardiac events (MACE) were defined as postoperative extracorporeal membrane oxygenation, cardiopulmonary resuscitation, or operative mortality. TA-IAA patients were compared with TA patients who underwent repair during the study period from the same institutions. RESULTS: We reviewed 35 patients with TA-IAA. MACE occurred in 12 patients (34%). Improvement over time was observed during the study period with 11 events (92%) occurring in the first half of the study period (P = .03). Factors associated with MACE included moderate or severe truncal valve insufficiency (P < .01), concomitant truncal valve repair (P = .04), and longer cardiopulmonary bypass duration (P = .02). In comparison with 216 patients who underwent TA repair, patients with TA-IAA had a higher rate of MACE, but this finding was not statistically significant (34% vs 20%, respectively; P = .07). Additionally no differences between TA-IAA and TA groups were observed for unplanned reoperations (14% vs 22%, respectively; P = .3), hospital length of stay (24 vs 23 days, P = .65), or late deaths (7% vs 7%, P = 1.00). CONCLUSIONS: In this contemporary, multicenter cohort the rate of MACE after repair of TA-IAA was high but improved during the study period. Early childhood outcomes of patients with TA-IAA were similar to those with TA.

Procedural Outcomes of Pulmonary Atresia With Intact Ventricular Septum in Neonates: A Multicenter Study.

BACKGROUND: Multicenter contemporary data describing short-term outcomes after initial interventions of neonates with pulmonary atresia with intact ventricular septum (PA-IVS) are limited. This multicenter study describes characteristics and outcomes of PA-IVS neonates after their initial catheter or surgical intervention and identifies factors associated with major adverse cardiac events (MACE). METHODS: Neonates with PA-IVS who underwent surgical or catheter intervention between 2009 and 2019 in 19 centers were reviewed. Risk factors for MACE, defined as cardiopulmonary resuscitation, mechanical circulatory support, stroke, or in-hospital mortality, were analyzed using multivariable logistic regression models. RESULTS: We reviewed 279 neonates: 79 (28%) underwent right ventricular decompression, 151 (54%) underwent systemic-to-pulmonary shunt or ductal stent placement only, 36 (13%) underwent right ventricular decompression with shunt or ductal stent placement, and 11 (4%) underwent transplantation. MACE occurred in 57 patients (20%): 26 (9%) received mechanical circulatory support, 37 (13%) received cardiopulmonary resuscitation, stroke occurred in 16 (6%), and 23 (8%) died. The presence of 2 major coronary artery stenoses (adjusted odds ratio, 4.99; 95% CI, 1.16-21.39) and lower weight at first intervention (adjusted odds ratio, 1.52; 95% CI, 1.01-2.27) were significantly associated with MACE. Coronary ischemia was the most frequent presumed mechanism of death (n = 10). CONCLUSIONS: In a multicenter cohort, 1 in 5 neonates with PA-IVS experienced MACE after their initial intervention. Patients with 2 major coronary artery stenoses or lower weight at the time of the initial procedure were most likely to experience MACE and warrant vigilance during preintervention planning and postintervention management.

Development of consensus recommendations for the management of post-operative chylothorax in paediatric CHD.

OBJECTIVE: A standardised multi-site approach to manage paediatric post-operative chylothorax does not exist and leads to unnecessary practice variation. The Chylothorax Work Group utilised the Pediatric Critical Care Consortium infrastructure to address this gap. METHODS: Over 60 multi-disciplinary providers representing 22 centres convened virtually as a quality initiative to develop an algorithm to manage paediatric post-operative chylothorax. Agreement was objectively quantified for each recommendation in the algorithm by utilising an anonymous survey. "Consensus" was defined as ≥ 80% of responses as "agree" or "strongly agree" to a recommendation. In order to determine if the algorithm recommendations would be correctly interpreted in the clinical environment, we developed ex vivo simulations and surveyed patients who developed the algorithm and patients who did not. RESULTS: The algorithm is intended for all children (<18 years of age) within 30 days of cardiac surgery. It contains rationale for 11 central chylothorax management recommendations; diagnostic criteria and evaluation, trial of fat-modified diet, stratification by volume of daily output, timing of first-line medical therapy for "low" and "high" volume patients, and timing and duration of fat-modified diet. All recommendations achieved "consensus" (agreement >80%) by the workgroup (range 81-100%). Ex vivo simulations demonstrated good understanding by developers (range 94-100%) and non-developers (73%-100%). CONCLUSIONS: The quality improvement effort represents the first multi-site algorithm for the management of paediatric post-operative chylothorax. The algorithm includes transparent and objective measures of agreement and understanding. Agreement to the algorithm recommendations was >80%, and overall understanding was 94%.

Pulmonary Atresia with Intact Ventricular Septum: Midterm Outcomes from a Multicenter Cohort.

Contemporary multicenter data regarding midterm outcomes for neonates with pulmonary atresia with intact ventricular septum are lacking. We sought to describe outcomes in a contemporary multicenter cohort, determine factors associated with end-states, and evaluate the effect of right ventricular coronary dependency and coronary atresia on transplant-free survival. Neonates treated during 2009-2019 in 19 United States centers were reviewed. Competing risks analysis was performed to determine cumulative risk of each end-state, and multivariable regression analyses were performed to identify factors associated with each end-state and transplant-free survival. We reviewed 295 patients. Median tricuspid valve Z-score was - 3.06 (25%, 75%: - 4.00, - 1.52). Final end-state was biventricular repair for 45 patients (15.2%), one-and-a half ventricle for 16 (5.4%), Fontan for 75 (25.4%), cardiac transplantation for 29 (9.8%), and death for 54 (18.3%). Seventy-six patients (25.7%) remained in mixed circulation. Cumulative risk estimate of death was 10.9%, 16.1%, 16.9%, and 18.8% at 1, 6 months, 1 year, and 5 years, respectively. Tricuspid valve Z-score was inversely, and coronary atresia positively associated with death or transplantation [odds ratio (OR) = 0.46, (95% confidence interval (CI) = 0.29-0.75, p < 0.001) and OR = 3.75 (95% CI 1.46-9.61, p = 0.011), respectively]. Right ventricular coronary dependency and left coronary atresia had a significant effect on transplant-free survival (log-rank p < 0.001). In a contemporary multicenter cohort of patients with PAIVS, consisting predominantly of patients with moderate-to-severe right ventricular hypoplasia, we observed favorable survival outcomes. Right ventricular coronary dependency and left, but not right, coronary atresia significantly worsens transplant-free survival.

Preoperative in-hospital mortality in neonates with critical CHD.

OBJECTIVE: Data regarding preoperative mortality in neonates with critical CHD are sparse and would aid patient care and family counselling. The objective of this study was to utilise a multicentre administrative dataset to report the rate of and identify risk factors for preoperative in-hospital mortality in neonates with critical CHD across US centres. STUDY DESIGN: The Pediatric Health Information System database was utilised to search for newborns ≤30 days old, born 1 January 2009 to 30 June 2018, with an ICD-9/10 code for d-transposition of the great arteries, truncus arteriosus, interrupted aortic arch, or hypoplastic left heart syndrome. Preoperative in-hospital mortality was defined as patients who died prior to discharge without an ICD code for cardiac surgery or interventional catheterisation. RESULTS: Overall preoperative mortality rate was at least 5.4% (690/12,739) and varied across diagnoses (d-TGA 2.9%, TA 8.3%, IAA 5.5%, and HLHS 7.3%) and centres (0-20.5%). In multivariable analysis, risk factors associated with preoperative mortality included preterm delivery (<37 weeks) (OR 2.3, 95% CI: 1.8-2.9; p < 0.01), low birth weight (<2.5 kg) (OR 3.8, 95% CI: 3.0-4.7; p < 0.01), and genetic abnormality (OR 1.6, 95% CI: 1.2-2.2; p < 0.01). Centre average surgical volume was not a significant risk factor. CONCLUSION: Approximately 1 in 20 neonates with critical CHD suffered preoperative in-hospital mortality, and rates varied across diagnoses and centres. Better understanding of the factors that drive the variation (e.g. patient factors, preoperative care models, surgical timing) could help identify patient care improvement opportunities and inform conversations with families.

Calcium Chloride Infusions are Not Associated with Improved Outcomes in Neonates Undergoing Cardiac Operations.

Neonatal cardiac performance is dependent on calcium delivery to the myocardium. Little is known about the use and impact of calcium chloride infusions in neonates who undergo cardiac surgery. We hypothesized that the use of calcium chloride infusions would decrease the doses required of traditional inotropic and vasoactive medications by supporting cardiac output in this patient population. We performed a single-institution, retrospective, cohort study. All neonates (≤ 30 days old) undergoing cardiac surgery from 06/01/2015 through 12/31/2018 were included. Patients were divided into two groups: those who received postoperative calcium chloride infusions (calcium group) and those who did not (control group). The primary outcome was the occurrence of a maximum Vasoactive Inotropic Score (VIS) > 15 in the first 24 h following surgery. One hundred and thirty-five patients met inclusion criteria. Sixty-six patients received postoperative calcium infusions and 69 patients did not. Gestational age, weight at surgery, age at surgery, surgical complexity and cardiopulmonary bypass times were similar between groups. Forty-two (70%) patients receiving calcium had a postoperative maximum VIS > 15 compared with 38 (55%) patients not on a calcium infusion (p = 0.08). There were no differences in postoperative length of ventilation, time to enteral feeding, hospital LOS, or operative mortality between groups. Calcium chloride infusions in neonates who underwent cardiac surgery did not decrease exposure to other inotropic and vasoactive agents in the first 24 post-operative hours or improve patient outcomes.

Relationship Between Gestational Age and Outcomes After Congenital Heart Surgery.

BACKGROUND: Previous studies suggest that birth before 39 weeks' gestational age (GA) is associated with higher perioperative mortality and morbidity after congenital heart surgery. The optimal approach to timing of cardiac operation in premature infants remains unclear. We investigated the impact of GA at birth and corrected GA at surgery on postoperative outcomes using the Pediatric Cardiac Critical Care Consortium (PC4) database. METHODS: Infants undergoing selected index cardiac operations before the end of the neonatal period were included (n = 2298). GA at birth and corrected GA at the time of the index cardiac operation were used as categorical predictors and fitted as a cubic spline to assess nonlinear relationships. The primary outcome was hospital mortality. Multivariable logistic regression models assessed the association between predictors and outcomes while adjusting for confounders. RESULTS: Late-preterm (34-36 weeks) birth was associated with increased odds of mortality compared with full-term (39-40 weeks) birth, while early-term (37-38 weeks) birth was not associated with increased mortality. Corrected GA at surgery of 34 to 37 weeks compared with 40 to 44 weeks was associated with increased mortality. When analyzing corrected GA at surgery as a continuous predictor of outcome, odds of survival improve as patients approach 39 weeks corrected GA. CONCLUSIONS: Contrary to previous literature, we did not find an association between early-term birth and hospital mortality at PC4 hospitals. Our analysis of the relationship between corrected GA and mortality suggests that operating closer to full-term corrected GA may improve survival.

Multicenter Analysis of Truncal Valve Management and Outcomes in Children with Truncus Arteriosus.

Truncal valve management in patients with truncus arteriosus is a clinical challenge, and indications for truncal valve intervention have not been defined. We sought to evaluate truncal valve dysfunction and primary valve intervention in patients with truncus arteriosus and determine risk factors for later truncal valve intervention. We conducted a retrospective cohort study of children who underwent truncus arteriosus repair at 15 centers between 2009 and 2016. Multivariable competing risk analysis was performed to determine risk factors for later truncal valve intervention. We reviewed 252 patients. Forty-two patients (17%) underwent truncal valve intervention during their initial surgery. Postoperative extracorporeal support, CPR, and operative mortality for patients who underwent truncal valve interventions were statistically similar to the rest of the cohort. Truncal valve interventions were performed in 5 of 64 patients with mild insufficiency; 5 of 16 patients with mild-to-moderate insufficiency; 17 of 35 patients with moderate insufficiency; 5 of 9 patients with moderate-to-severe insufficiency; and all 10 patients with severe insufficiency. Twenty patients (8%) underwent later truncal valve intervention, five of whom had no truncal valve intervention during initial surgical repair. Multivariable analysis revealed truncal valve intervention during initial repair (HR 11.5; 95% CI 2.5, 53.2) and moderate or greater truncal insufficiency prior to initial repair (HR 4.0; 95% CI 1.1, 14.5) to be independently associated with later truncal valve intervention. In conclusion, in a multicenter cohort of children with truncus arteriosus, 17% had truncal valve intervention during initial surgical repair. For patients in whom variable truncal valve insufficiency is present and primary intervention was not performed, late interventions were uncommon. Conservative surgical approach to truncal valve management may be justifiable.

Corticosteroid Therapy in Neonates Undergoing Cardiopulmonary Bypass: Randomized Controlled Trial.

BACKGROUND: The efficacy of intraoperative corticosteroids to improve outcomes following congenital cardiac operations remains controversial. OBJECTIVES: The purpose of this study was to determine whether intraoperative methylprednisolone improves post-operative recovery in neonates undergoing cardiac surgery. METHODS: Neonates undergoing cardiac surgery with cardiopulmonary bypass at 2 centers were enrolled in a double-blind randomized controlled trial of methylprednisolone (30 mg/kg) or placebo after the induction of anesthesia. The primary outcome was a previously validated morbidity-mortality composite that included any of the following events following surgery before discharge: death, mechanical circulatory support, cardiac arrest, hepatic injury, renal injury, or rising lactate level (>5 mmol/l). RESULTS: Of the 190 subjects enrolled, 176 (n = 81 methylprednisolone, n = 95 placebo) were included in this analysis. A total of 27 (33%) subjects in the methylprednisolone group and 40 (42%) in the placebo group reached the primary study endpoint (odds ratio [OR]: 0.63; 95% confidence interval [CI]: 0.31 to 1.3; p = 0.21). Methylprednisolone was associated with reductions in vasoactive inotropic requirements and in the incidence of the composite endpoint in subjects undergoing palliative operations (OR: 0.38; 95% CI: 0.15 to 0.99; p = 0.048). There was a significant interaction between treatment effect and center. In this analysis, methylprednisolone was protective at 1 center, with an OR: 0.35 (95% CI: 0.15 to 0.84; p = 0.02), and not so at the other center, with OR: 5.13 (95% CI: 0.85 to 30.90; p = 0.07). CONCLUSIONS: Intraoperative methylprednisolone failed to show an overall significant benefit on the incidence of the composite primary study endpoint. There was, however, a benefit in patients undergoing palliative procedures and a significant interaction between treatment effect and center, suggesting that there may be center or patient characteristics that make prophylactic methylprednisolone beneficial.

Utilization of an Abiomed Impella Device as a Rescue Therapy for Acute Ventricular Failure in a Fontan Patient.

Progressive ventricular dysfunction is not uncommon in patients with univentricular hearts as they age. In the acute setting vasoactive support can be employed, but is not always sufficient and patients occasionally require mechanical support. We report the successful implantation and subsequent challenges of a percutaneous Abiomed Impella ventricular assist device as a rescue therapy for a 15-year old-patient with Fontan circulation and severe ventricular dysfunction after cardiac arrest.

Plasma Neutrophil Gelatinase-Associated Lipocalin Is Associated With Acute Kidney Injury and Clinical Outcomes in Neonates Undergoing Cardiopulmonary Bypass.

OBJECTIVES: Acute kidney injury is a frequent complication following neonatal cardiac surgery and is associated with significant morbidity and mortality. The objectives of this study were to determine if plasma neutrophil gelatinase-associated lipocalin levels were associated with acute kidney injury and clinical outcomes in neonates with congenital heart disease undergoing cardiopulmonary bypass. DESIGN: Retrospective single-center observational study. SETTING: A pediatric cardiac ICU within a tertiary-care academic hospital. PATIENTS: Patients age less than 30 days undergoing cardiac surgery requiring cardiopulmonary bypass. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Plasma neutrophil gelatinase-associated lipocalin peaked at 12 hours postcardiopulmonary bypass and more than doubled compared with preoperative levels. Higher preoperative and 24-hour postoperative neutrophil gelatinase-associated lipocalin levels were associated with acute kidney injury (r = 0.30, r = 0.49), longer duration of mechanical ventilation (r = 0.40, r = 0.51), ICU (r = 0.32, r = 0.33) and hospital lengths of stay (r = 0.28, r = 0.32), and total hospital charges (r = 0.35, r = 0.30; all p values < 0.05). CONCLUSIONS: Both preoperative and 24-hour postoperative plasma neutrophil gelatinase-associated lipocalin levels are associated with acute kidney injury and worse clinical outcomes in neonates undergoing cardiac surgery. Plasma neutrophil gelatinase-associated lipocalin levels may have a role in risk stratification for predicting postoperative renal dysfunction as well as providing a potential clinical trajectory in the postoperative period.

Characteristics and operative outcomes for children undergoing repair of truncus arteriosus: A contemporary multicenter analysis.

OBJECTIVE: We sought to describe characteristics and operative outcomes of children who underwent repair of truncus arteriosus and identify risk factors for the occurrence of major adverse cardiac events (MACE) in the immediate postoperative period in a contemporary multicenter cohort. METHODS: We conducted a retrospective review of children who underwent repair of truncus arteriosus between 2009 and 2016 at 15 centers within the United States. Patients with associated interrupted or obstructed aortic arch were excluded. MACE was defined as the need for postoperative extracorporeal membrane oxygenation, cardiopulmonary resuscitation, or operative mortality. Risk factors for MACE were identified using multivariable logistic regression analysis and reported as odds ratios (ORs) with 95% confidence intervals (CIs). RESULTS: We reviewed 216 patients. MACE occurred in 44 patients (20%) and did not vary significantly over time. Twenty-two patients (10%) received postoperative extracorporeal membrane oxygenation, 26 (12%) received cardiopulmonary resuscitation, and 15 (7%) suffered operative mortality. With multivariable logistic regression analysis (which included adjustment for center effect), factors independently associated with MACE were failure to diagnose truncus arteriosus before discharge from the nursery (OR, 3.1; 95% CI, 1.3-7.4), cardiopulmonary bypass duration >150 minutes (OR, 3.5; 95% CI, 1.5-8.5), and right ventricle-to-pulmonary artery conduit diameter >50 mm/m2 (OR, 4.7; 95% CI, 2.0-11.1). CONCLUSIONS: In a contemporary multicenter analysis, 20% of children who underwent repair of truncus arteriosus experienced MACE. Early diagnosis, shorter duration of cardiopulmonary bypass, and use of smaller diameter right ventricle-to-pulmonary artery conduits represent potentially modifiable factors that could decrease morbidity and mortality in this fragile patient population.

Multicenter Analysis of Early Childhood Outcomes After Repair of Truncus Arteriosus.

BACKGROUND: Literature describing morbidity and mortality after truncus arteriosus repair is predominated by single-center reports. We created and analyzed a multicenter dataset to identify risk factors for late mortality and right ventricle-to-pulmonary artery (RV-PA) conduit reintervention for this patient population. METHODS: We retrospectively collected data on children who underwent repair of truncus arteriosus without concomitant arch obstruction at 15 centers between 2009 and 2016. Cox regression survival analysis was conducted to determine risk factors for late mortality, defined as death occurring after hospital discharge and greater than 30 days after operation. Probability of any RV-PA conduit reintervention was analyzed over time using Fine-Gray modeling. RESULTS: We reviewed 216 patients with median follow-up of 2.9 years (range, 0.1 to 8.8). Operative mortality occurred in 15 patients (7%). Of the 201 survivors there were 14 (7%) late deaths. DiGeorge syndrome (hazard ratio [HR], 5.4; 95% confidence interval [CI], 1.6 to 17.8) and need for postoperative tracheostomy (HR, 5.9; 95% CI, 1.8 to 19.4) were identified as independent risk factors for late mortality. At least one RV-PA conduit catheterization or surgical reintervention was performed in 109 patients (median time to reintervention, 23 months; range, 0.3 to 93). Risk factors for reintervention included use of pulmonary or aortic homografts versus Contegra (Medtronic, Inc, Minneapolis, MN) bovine jugular vein conduits (HR, 1.9; 95% CI, 1.2 to 3.1) and smaller conduit size (HR per mm/m2, 1.05; 95% CI, 1.03 to 1.08). CONCLUSIONS: In a multicenter dataset DiGeorge syndrome and need for tracheostomy postoperatively were found to be independent risk factors for late mortality after repair of truncus arteriosus, whereas risk of conduit reintervention was independently influenced by both initial conduit type and size.

Out-of-pocket medical expenses in severe CHD.

IntroductionFamilies of children born with CHD face added stress owing to uncertainty about the magnitude of the financial burden for medical costs they will face. This study seeks to assess the family responsibility for healthcare bills during the first 12 months of life for commercially insured children undergoing surgery for severe CHD. METHODS: The MarketScan ® database from Truven was used to identify commercially insured infants in 39 states from 2010 to 2012 with an ICD-9 diagnosis code for transposition of the great arteries, tetralogy of Fallot, or truncus arteriosus, as well as the corresponding procedure code for complete repair. Data extraction identified payment responsibilities of the patients' families in the form of co-payments, deductibles, and co-insurance during the 1st year of life. RESULTS: There were 481 infants identified who met the criteria. Average family responsibility for healthcare bills during the 1st year of life was $2928, with no difference between the three groups. The range of out-of-pocket costs was $50-$18,167. Initial hospitalisation and outpatient care accounted for the majority of these responsibilities. CONCLUSIONS: Families of commercially insured children with severe CHD requiring corrective surgery face an average of ~$3000 in out-of-pocket costs for healthcare bills during the first 12 months of their child's life, although the amount varied considerably. This information provides a framework to alleviate some of the uncertainty surrounding healthcare financial responsibilities, and further examination of the origination of these expenditures may be useful in informing future healthcare policy discussion.

Nutrition Considerations in the Pediatric Cardiac Intensive Care Unit Patient.

Adequate caloric intake plays a vital role in the course of illness and the recovery of critically ill patients. Nutritional status and nutrient delivery during critical illness have been linked to clinical outcomes such as mortality, incidence of infection, and length of stay. However, feeding practices with critically ill pediatric patients after cardiac surgery are variable. The Pediatric Cardiac Intensive Care Society sought to provide an expert review on provision of nutrition to pediatric cardiac intensive care patients, including caloric requirements, practical considerations for providing nutrition, safety of enteral nutrition in controversial populations, feeding considerations with chylothorax, and the benefits of feeding beyond nutrition. This article addresses these areas of concern and controversy.

Clinical epidemiology and centre variation in chylothorax rates after cardiac surgery in children: a report from the Pediatric Cardiac Critical Care Consortium.

Introduction Chylothorax after paediatric cardiac surgery incurs significant morbidity; however, a detailed understanding that does not rely on single-centre or administrative data is lacking. We described the present clinical epidemiology of postoperative chylothorax and evaluated variation in rates among centres with a multicentre cohort of patients treated in cardiac ICU. METHODS: This was a retrospective cohort study using prospectively collected clinical data from the Pediatric Cardiac Critical Care Consortium registry. All postoperative paediatric cardiac surgical patients admitted from October, 2013 to September, 2015 were included. Risk factors for chylothorax and association with outcomes were evaluated using multivariable logistic or linear regression models, as appropriate, accounting for within-centre clustering using generalised estimating equations. RESULTS: A total of 4864 surgical hospitalisations from 15 centres were included. Chylothorax occurred in 3.8% (n=185) of hospitalisations. Case-mix-adjusted chylothorax rates varied from 1.5 to 7.6% and were not associated with centre volume. Independent risk factors for chylothorax included age <1 year, non-Caucasian race, single-ventricle physiology, extracardiac anomalies, longer cardiopulmonary bypass time, and thrombosis associated with an upper-extremity central venous line (all p<0.05). Chylothorax was associated with significantly longer duration of postoperative mechanical ventilation, cardiac ICU and hospital length of stay, and higher in-hospital mortality (all p<0.001). CONCLUSIONS: Chylothorax after cardiac surgery in children is associated with significant morbidity and mortality. A five-fold variation in chylothorax rates was observed across centres. Future investigations should identify centres most adept at preventing and managing chylothorax and disseminate best practices.