Factors associated with renal oxygen extraction in mechanically ventilated children after the Norwood operation: insights from high fidelity haemodynamic data.

BACKGROUND: Maintaining the adequacy of systemic oxygen delivery is of utmost importance, particularly in critically ill children. Renal oxygen extraction can be utilised as metric of the balance between systemic oxygen delivery and oxygen consumption. The primary aim of this study was to determine what clinical factors are associated with renal oxygen extraction in children after Norwood procedure. METHODS: Mechanically ventilated children who underwent Norwood procedure from 1 September, 2022 to 1 March, 2023 were identified as these patients had data collected and stored with high fidelity by the T3 software. Data regarding haemodynamic values, fluid balance, and airway pressure were collected and analysed using Bayesian regression to determine the association of the individual metrics with renal oxygen extraction. RESULTS: A total of 27,270 datapoints were included in the final analyses. The resulting top two models explained had nearly 80% probability of being true and explained over 90% of the variance in renal oxygen extraction. The coefficients for each variable retained in the best were -1.70 for milrinone, -19.05 for epinephrine, 0.129 for mean airway pressure, -0.063 for mean arterial pressure, 0.111 for central venous pressure, 0.093 for arterial saturation, 0.006 for heart rate, -0.025 for respiratory rate, 0.366 for systemic vascular resistance, and -0.032 for systemic blood flow. CONCLUSION: Increased milrinone, epinephrine, mean arterial pressure, and systemic blood flow were associated with decreased (improved) renal oxygen extraction, while increased mean airway pressure, central venous pressure, arterial saturation, and systemic vascular resistance were associated with increased (worsened) renal oxygen extraction.

ALCAPA in Children with Complex Congenital Heart Disease: A Multicenter Study.

We sought to report characteristics and outcomes of children with complex anomalous left coronary artery from the pulmonary artery (ALCAPA) and to compare the outcomes to children with isolated ALCAPA. We performed a retrospective data analysis of children with ALCAPA who underwent cardiac surgery between 1/2009 and 3/2018 at 21 centers. Characteristics and outcomes of patients with complex ALCAPA are provided using descriptive statistics. Outcomes were compared between complex ALCAPA and isolated ALCAPA using Fisher's exact test. We reviewed 258 patients who underwent surgical repair of ALCAPA at 21 centers. We identified 10 patients (3.9%) with complex ALCAPA. Median age at initial cardiac surgery was 49 days (range: 4 days, 12.8 years). Cardiac lesions associated with ALCAPA were HLHS (n = 3); scimitar syndrome (n = 2); VSD with aortic coarctation (n = 2); VSD with right pulmonary artery discontinuity (1); DORV with mitral atresia (n = 1); and ToF (n = 1). ALCAPA was diagnosed prior to surgical intervention in 1 patient; during the initial cardiac surgery in 4 patients; in the early postoperative period via cardiac catheterization in 3 patients; and later in childhood after initial surgical repair in 2 patients. Following ALCAPA repair, patients with complex ALCAPA, as compared to patients with isolated ALCAPA, were more likely to be placed on ECMO (50% vs 12%, p = 0.002), receive CPR (30% vs 6%, p = 0.017), or suffer operative mortality (50% vs 3%, p < 0.001). Complex ALCAPA is uncommon. All but one with complex ALCAPA was not diagnosed preoperatively and postoperative morbidity and mortality were significantly greater in these complex patients compared to patients with isolated ALCAPA.

Clinical Outcomes After Tracheostomy in Children With Single Ventricle Physiology: Collaborative Research From the Pediatric Cardiac Intensive Care Society Multicenter Cohort, 2010-2021.

OBJECTIVES: Multicenter studies reporting outcomes following tracheostomy in children with congenital heart disease are limited, particularly in patients with single ventricle physiology. We aimed to describe clinical characteristics and outcomes in a multicenter cohort of patients with single ventricle physiology who underwent tracheostomy before Fontan operation. DESIGN: Multicenter retrospective cohort study.SETTING: Twenty-one tertiary care pediatric institutions participating in the Collaborative Research from the Pediatric Cardiac Intensive Care Society. PATIENTS: We reviewed 99 children with single ventricle physiology who underwent tracheostomy before the Fontan operation at 21 institutions participating in Collaborative Research from the Pediatric Cardiac Intensive Care Society between January 2010 and December 2020, with follow-up through December 31, 2021. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Death occurred in 51 of 99 patients (52%). Cox proportional hazard analysis was performed to determine factors associated with death after tracheostomy. Results are presented as hazard ratio (HR) with 95% CIs. Nonrespiratory indication(s) for tracheostomy (HR, 2.21; 95% CI, 1.14-4.32) and number of weeks receiving mechanical ventilation before tracheostomy (HR, 1.06; 95% CI, 1.02-1.11) were independently associated with greater hazard of death. In contrast, diagnosis of tricuspid atresia or Ebstein's anomaly was associated with less hazard of death (HR, 0.16; 95% CI, 0.04-0.69). Favorable outcome, defined as survival to Fontan operation or decannulation while awaiting Fontan operation with viable cardiopulmonary physiology, occurred in 29 of 99 patients (29%). Median duration of mechanical ventilation before tracheostomy was shorter in patients who survived to favorable outcome (6.1 vs. 12.1 wk; p < 0.001), and only one of 16 patients with neurologic indications for tracheostomy and 0 of ten patients with cardiac indications for tracheostomy survived to favorable outcome. CONCLUSIONS: For children with single ventricle physiology who undergo tracheostomy, mortality risk is high and should be carefully considered when discussing tracheostomy as an option for these children. Favorable outcomes are possible, although thoughtful attention to patient selection and tracheostomy timing are likely necessary to achieve this goal.

Preoperative Hemodynamics Impact the Benefit of Fenestration on Fontan Postoperative Length of Stay.

BACKGROUND: Utilization of Fontan fenestration varies considerably by center. OBJECTIVES: Using a multicenter Pediatric Heart Network dataset linking surgical and preoperative hemodynamic variables, the authors evaluated factors associated with use of Fontan fenestration and the impact of fenestration on post-Fontan length of stay (LOS). METHODS: Patients 2 to 6 years old at Fontan surgery from 2010 to 2020 with catheterization<1 year prior were included. Factors associated with fenestration were evaluated using multivariable logistic regression adjusting for key covariates. Restrictive cubic spline analysis was used to evaluate potential cut-points for hemodynamic variables associated with longer postoperative LOS stratified by fenestration with multivariable linear regression to evaluate the magnitude of effect. RESULTS: Fenestration was used in 465 of 702 patients (66.2%). Placement of a fenestration was associated with center (range 27%-93% use, P < 0.0001) and Fontan type (OR: 14.1 for lateral tunnel vs extracardiac conduit, P < 0.0001). No hemodynamic variable was independently associated with fenestration. In a multivariable linear model adjusting for center, a center-fenestration interaction, prematurity, preoperative mean pulmonary artery pressure (mPAP), and cardiac index, fenestration was associated with shorter hospital LOS after Fontan (P = 0.0024). The benefit was most pronounced at mPAP ≥13 mm Hg (median LOS: 9 vs 12 days, P = 0.001). CONCLUSIONS: There is wide center variability in use of Fontan fenestration that is not explained by preoperative hemodynamics. Fenestration is independently associated with shorter LOS, and those with mPAP ≥13 mm Hg at pre-Fontan catheterization benefit the most. We propose this threshold as minimal criteria for fenestration.

Postoperative Atrioventricular Block in Pediatric Patients: Impact of Congenital Cardiac Malformations and Medications.

Postoperative atrioventricular block may occur after pediatric cardiac surgery. A small proportion of those who develop atrioventricular block will require pacemaker placement. The primary aim of this study was to determine factors associated with postoperative atrioventricular block. Secondary aims included determining factors associated with pacemaker placement in those with atrioventricular block. Data from the PHIS data were utilized to identify patients under 18 years of age who underwent cardiac surgery. Those who did and did not develop atrioventricular block. Univariable analyses and regression analyses were conducted to determine factors associated with postoperative atrioventricular block. Similar analyses were conducted to determine factors associated with pacemaker placement in those with atrioventricular block. A total of 43,716 admissions were identified. Of these, 2093 (5%) developed atrioventricular block and 480 (1% of total admissions) underwent pacemaker placement. Approximately 70% of those with atrioventricular block received steroids but this was not associated with a decrease in pacemaker placement. Risk factors (congenital malformations of the heart, comorbidities, medications) associated with increased risk of atrioventricular block and pacemaker placement were identified. Postoperative atrioventricular block occurred in 5% of pediatric admissions for cardiac surgery. Of these admissions with postoperative atrioventricular block, 23% required pacemaker placement. Isoproterenol and steroids were not associated with a reduction in the likelihood of pacemaker placement.

Estimation of the inferior caval vein saturation using high-fidelity non-invasive haemodynamic values and validation of modelled estimates.

OBJECTIVES: Monitoring venous saturation allows identification of inadequate systemic oxygen delivery. The aim was to develop a model using non-invasive haemodynamic variables to estimate the inferior caval vein saturation and to determine its prognostic utility. METHODS: This is a single-centre, retrospective study. A Bayesian Pearson's correlation was conducted to model the inferior caval vein saturation. Next, a Bayesian linear regression was conducted for data from all the patients and from only those with parallel circulation. Venous saturation estimations were developed. The correlation of these estimates to the actual inferior caval vein saturation was assessed. The resulting models were then applied to two validation cohorts: biventricular circulation (arterial switch operation) and parallel circulation (Norwood operation). RESULTS: One hundred and thirteen datasets were collected across 15 patients. Of which, 65% had parallel circulation. In all patients, the measured and estimated inferior caval vein saturations had a moderate and significant correlation with a coefficient of 0.64. In patients with parallel circulation, the measured and estimated inferior caval vein saturation had a moderate and significant correlation with a coefficient of 0.61. In the biventricular circulation cohort, the estimated inferior caval vein saturation had an area under the curve of 0.71 with an optimal cut-off of 49. In the parallel circulation cohort, the estimated interior caval vein saturation had an area under the curve of 0.83 with an optimal cut-off of 24%. CONCLUSION: The inferior caval vein saturation can be estimated utilising non-invasive haemodynamic data. This estimate has correlation with measured inferior caval vein saturations and offers prognostic utility.

Correlation of hepatic venous saturation and mixed venous saturation: pooled analyses.

INTRODUCTION: In critical care, monitoring adequate tissue oxygenation is essential. Mixed venous oxygen saturation has traditionally been considered the gold standard for measuring cardiac output, which represents systemic oxygen delivery. Studies have shown that hepatic vein saturation is correlated with mixed venous oxygen saturation and mortality. The primary aim of this study was to determine the correlation between hepatic vein saturation and mixed venous saturation, and the impact of clinical characteristics on this correlation. EVIDENCE ACQUISITION: A systematic review of the literature was performed to identify manuscripts. They must have included patients who received simultaneous mixed venous saturations and hepatic vein saturations, and the data for both must have been explicitly shared. Data were pooled from these studies to analyze the correlation between mixed venous saturation and the corresponding hepatic vein saturation. EVIDENCE SYNTHESIS: A total of 13 studies with 333 patients were included in the final analyses. The average age across these studies was 60.3±5.2. The pooled correlation between the mixed venous saturation and hepatic vein saturation was 0.88, demonstrating a strong correlation between the two. The average mixed venous saturation was 73.3±5.0 while the average hepatic vein saturation was 59.5±11.1. CONCLUSIONS: In these pooled analyses, hepatic vein saturation has a strong correlation with mixed venous saturation. This correlation is not significantly impacted by patient age, weight, or clinical setting. Nonetheless, further prospective studies are needed for confirmation.

Association Between Congenital Heart Disease and Stroke: Insights from a National Database.

To delineate prevalence of stroke in the pediatric intensive care unit and to determine risk factors for stroke and association of stroke with mortality in patients with congenital heart disease. Retrospective cohort study. Patients admitted to pediatric intensive care units in the USA participating in the Pediatric Health Information System database from 2016 to 2021. Patients were categorized as those who experienced ischemic or hemorrhagic stroke and those with congenital heart disease. We performed univariate and multivariate logistic regressions to determine risk factors associated with stroke and then developed a predictive model for stroke development in patients with congenital heart disease. Of 426,029 admissions analyzed, 4237 (0.9%) patients experienced stroke and 1197 (1.4%) of 80,927 patients with congenital heart disease developed stroke (odds ratio 1.15, 95% confidence interval 1.06-1.24). Patients with congenital heart disease, younger age, extracorporeal membrane oxygenation, mechanical ventilation, and cardiac arrest were most strongly associated with increased risk of stroke. Stroke increased odds of mortality for patients with congenital heart disease (odds ratio 2.49, 95% confidence interval 2.08-2.98). A risk score greater than 0 was associated with a 33.3% risk of stroke for patients with congenital heart disease (negative predictive value of 99%, sensitivity 69%, specificity 63%). Children with congenital heart disease are at increased risk for developing stroke, which is associated with increased mortality. Early identification of the most vulnerable patients may enable providers to implement preventative measures or rapid treatment strategies to prevent neurologic morbidity.

Point-of-Care-Ultrasound in Pediatrics: A Review and Update.

Point-of-Care-Ultrasound (POCUS) has encountered a tremendous expansion in patient care. POCUS has taken a central role during invasive procedures. POCUS has expanded to most subspecialties from adult to pediatric and neonatal health care. POCUS in pediatrics has also become part of specific critical situations such as myocardial function assessment during cardiac arrest, extracorporeal membrane oxygenation deployment and neurological evaluation. In this review we will go over the most important historical aspects of POCUS. We will also review important aspects of POCUS in the intensive care unit, cardiologist evaluation and in the emergency department among others.

Parental holding of infants improves haemodynamics in the cardiac ICU.

We performed a single-centre, retrospective study to assess physiologic changes of infants in the cardiac ICU while being held by their parent. Continuous data streaming of vital signs were collected for infants included in the study from January 2021 to March 2022. Demographic and clinical characteristics were collected from the electronic medical record. The physiologic streaming data were analysed using mixed-effects models to account for repeated measures and quantify the effect of parental holding. Comparison analysis was also performed controlling for intubation, pre-operative versus post-operative status, and whether the holding was skin-to-skin or not. Ninety-five patients with complete physiologic data were included in the study. There were no immediate adverse events associated with holding. Heart rate decreased during the response time compared to its baseline value (p = 0.01), and this decrease was more pronounced for the non-intubated and pre-operative patients. The near-infrared spectroscopy-based venous saturation increased overall (p = 0.02) in patients while being held. We conclude that parental holding of infants in the cardiac ICU can be safely accomplished, and the haemodynamic and oximetric profile during the holding is favourable compared to the infants' baseline prior to holding.

The Inadequate Oxygen Delivery Index and Its Correlation with Venous Saturation in the Pediatric Cardiac Intensive Care Unit.

Continuous monitoring software, T3, has an integrated index called the inadequate oxygen delivery index 50% (IDO2-50) which displays a probability that the mixed venous saturation is below a user-selected threshold of 30-50%. The primary aim of this study was to determine the correlation of the IDO2-50 with a measured venous saturation. The secondary aim of this study was to characterize the hemodynamic factors that contributed to the IDO2-50. This single-center, retrospective study aimed to characterize the correlation between IDO2-50 and inferior vena cava (IVC) saturation. A Bayesian Pearson correlation was conducted to assess the correlation between the collected variables of interest, with a particular interest in the correlation between the IDO2-50 and the IVC saturation. Receiver operator curve (ROC) analysis to assess the ability of the IDO2-50 to identify when the venous saturation was less than 50%. Bayesian linear regression was done with the IDO2-50 (dependent variable) and other independent variables. A total of 113 datasets were collected across 15 unique patients. IDO2-50 had moderate correlation with the IVC saturation (correlation coefficient - 0.569). The IDO2-50 had a weak but significant correlation with cerebral near-infrared spectroscopy (NIRS) values, a weak but significant correlation with heart rate, and a moderate and significant correlation with arterial saturation. ROC analysis demonstrated that the IDO2-50 had a good ability to identify a venous saturation below 50%, with an area under the curve of 0.797, cutoff point of 24.5 with a sensitivity of 81%, specificity of 66%, positive predictive value of 44%, and negative predictive value of 91%. Bayesian linear regression analysis yielded the following model: 237.82 + (1.18 × age in months) - (3.31 × arterial saturation) - (1.92 × cerebral NIRS) + (0.84 × heart rate). The IDO2 index has moderate correlation with IVC saturation. It has good sensitive and negative predictive value. Cerebral NIRS does appear to correlate better with the underlying venous saturation than the IDO2 index.

A Program of Assessment Model for Point-of-Care Ultrasound Training for Pediatric Critical Care Providers: A Comprehensive Approach to Enhance Competency-Based Point-of-Care Ultrasound Training.

Point-of-care ultrasound (POCUS) is increasingly accepted in pediatric critical care medicine as a tool for guiding the evaluation and treatment of patients. POCUS is a complex skill that requires user competency to ensure accuracy, reliability, and patient safety. A robust competency-based medical education (CBME) program ensures user competency and mitigates patient safety concerns. A programmatic assessment model provides a longitudinal, holistic, and multimodal approach to teaching, assessing, and evaluating learners. The authors propose a fit-for-purpose and modifiable CBME model that is adaptable for different institutions' resources and needs for any intended competency level. This educational model drives and supports learning, ensures competency attainment, and creates a clear pathway for POCUS education while enhancing patient care and safety.

Predicting inpatient mortality in pediatric traumatic brain injury: insights from a national database.

PURPOSE: The purpose of this study was to determine factors significantly associated with mortality and length of stay (LOS) in admissions to the pediatric intensive care unit (PICU) for traumatic brain injury (TBI). METHODS: A cross-sectional, retrospective cohort study that identified PICU admissions with TBI from forty-nine hospitals in the USA using the Pediatric Health Information System database from 2016 to 2021. Univariable analyses comparing those who did and did not experience mortality were performed. The following regression analyses were conducted: logistic regression with mortality as dependent variable; linear regression with LOS as the dependent variable; logistic regression with mortality as the dependent variable but only included patients with cerebral edema; and linear regression with LOS as the dependent variable but only included patients who survived. From the regression analysis for mortality in all TBI patients was utilized to develop a mortality risk score. RESULTS: A total of 3041 admissions were included. Those with inpatient mortality (18.5%) tended to be significantly younger (54 vs. 92 months, p < 0.01), have < 9 pediatric Glasgow Coma Scale on admission (100% vs. 52.9%, p < 0.01) and more likely to experience acute renal, hepatic and respiratory failure, acidosis, central diabetes insipidus, hyperkalemia, and hypocalcemia. Regression analysis identified that pediatric Glasgow Coma Scale, alkalosis and cardiac arrest significantly increased risks of mortality. The TBI mortality risk score had an area under the curve of 0.89 to identify those with mortality; a score of 6 ≤ was associated with 88% mortality. CONCLUSION: Patients admitted to the PICU with TBI have 18.5% risk of inpatient mortality with most occurring the first 48 h and these are characterized with greater multisystem organ dysfunction, received medical and mechanical support. TBI mortality risk score suggested is a practical tool to identify patients with an increase likelihood to die.

Pediatric Swine Model of Methicillin-Resistant Staphylococcus aureus Sepsis-Induced Coagulopathy, Disseminated Microvascular Thrombosis, and Organ Injuries.

Sepsis-induced coagulopathy leading to disseminated microvascular thrombosis is associated with high mortality and has no existing therapy. Despite the high prevalence of Gram-positive bacterial sepsis, especially methicillin-resistant Staphylococcus aureus (MRSA), there is a paucity of published Gram-positive pediatric sepsis models. Large animal models replicating sepsis-induced coagulopathy are needed to test new therapeutics before human clinical trials. HYPOTHESIS: Our objective is to develop a pediatric sepsis-induced coagulopathy swine model that last 70 hours. METHODS AND MODELS: Ten 3 weeks old piglets, implanted with telemetry devices for continuous hemodynamic monitoring, were IV injected with MRSA (n = 6) (USA300, Texas Children's Hospital 1516 strain) at 1 × 109 colony forming units/kg or saline (n = 4). Fluid resuscitation was given for heart rate greater than 50% or mean arterial blood pressure less than 30% from baseline. Acetaminophen and dextrose were provided as indicated. Point-of-care complete blood count, prothrombin time (PT), activated thromboplastin time, d-dimer, fibrinogen, and specialized coagulation assays were performed at pre- and post-injection, at 0, 24, 48, 60, and 70 hours. Piglets were euthanized and necropsies performed. RESULTS: Compared with the saline treated piglets (control), the septic piglets within 24 hours had significantly lower neurologic and respiratory scores. Over time, PT, d-dimer, and fibrinogen increased, while platelet counts and activities of factors V, VII, protein C, antithrombin, and a disintegrin and metalloproteinase with thrombospondin-1 motifs (13th member of the family) (ADAMTS-13) decreased significantly in septic piglets compared with control. Histopathologic examination showed minor focal organ injuries including microvascular thrombi and necrosis in the kidney and liver of septic piglets. INTERPRETATIONS AND CONCLUSIONS: We established a 70-hour swine model of MRSA sepsis-induced coagulopathy with signs of consumptive coagulopathy, disseminated microvascular thrombosis, and early organ injuries with histological minor focal organ injuries. This model is clinically relevant to pediatric sepsis and can be used to study dysregulated host immune response and coagulopathy to infection, identify potential early biomarkers, and to test new therapeutics.

Fluid Overload and AKI After the Norwood Operation: The Correlation and Characterization of Routine Clinical Markers.

The purpose of this study was to determine the correlation of different methods of assessing fluid overload and determine which metrics are associated with development of acute kidney injury (AKI) in the period immediately following Norwood palliation. This was a retrospective single-center study of Norwood patients from January 2011 through January 2021. AKI was defined using the Kidney Disease Improving Global Outcomes (KDIGO). Patients were separated into two groups: those with AKI and those without. A logistic regression analysis was conducted with AKI at any point in the study period as the dependent variable and clinical and laboratory data as independent variables. Analysis was conducted as a stepwise regression. The coefficients from the logistic regression were then used to develop a cumulative AKI risk score. Spearman correlations were conducted to analyze the correlation of fluid markers. 116 patients were included, and 49 (42.4%) developed AKI. The duration of open chest, duration of mechanical ventilation, need for dialysis, need for extracorporeal membrane oxygenation, and inpatient mortality were associated with AKI (p ≤ 0.05). Stepwise logistic regression demonstrated the following significant independent associations AKI: age at Norwood in days (p < 0.01), blood urea nitrogen (p < 0.01), central venous pressure (p = 0.04), and renal oxygen extraction ratio (p < 0.01). The area under the receiver operating characteristic curve for the logistic regression was 0.74. The fluid markers had weak R-value. Urea, central venous pressure, and renal oxygen extraction ratio are associated with AKI after the Norwood operation. Common clinical metrics used to assess fluid overload are poorly correlated with each other for postoperative Norwood patients.

Echocardiographic Strain to Predict Need for Transplant or Mortality in Fontan Patients with Hypoplastic Left Heart Syndrome.

Despite recent advances, hypoplastic left heart syndrome (HLHS) patients subsequent to the Fontan still have significant morbidity and mortality. Some require heart transplant due to systemic ventricular dysfunction. Limited data exist on timing for transplant referral. This study aims to correlate systemic ventricular strain by echocardiography to transplant-free survival. HLHS patients who had Fontan palliation at our institution were included. Patients were divided into: 1) Required transplant or experienced mortality (composite end point); 2) Did not require transplant or survived. For those who experienced the composite endpoint, the last echocardiogram prior to the composite outcome was used, while for those who did not experience the composite endpoint the last echocardiogram obtained was used. Several qualitative and quantitative parameters were analyzed with focus on strain parameters. Ninety-five patients with HLHS Fontan palliation were identified. Sixty-six had adequate images and eight (12%) experienced transplant or mortality. These patients had greater myocardial performance index by flow Doppler (0.72 versus 0.53, p = 0.01), higher systolic/diastolic duration ratio (1.51 versus 1.13, p = 0.02), lower fractional area change (17.65 versus 33.99, p < 0.01), lower global longitudinal strain (GLS) (-8.63 versus - 17.99, p < 0.01), lower global longitudinal strain rate (GLSR) (- 0.51 versus - 0.93, p < 0.01), lower global circumferential strain (GCS) (-6.68 versus -18.25, p < 0.01), and lower (GCSR) global circumferential strain rate (-0.45 versus -1.01, p < 0.01). ROC analysis demonstrated predictive value for GLS - 7.6 (71% sensitive, 97% specific, AUC 81%), GLSR -0.58 (71% sensitive, 88% specific, AUC 82%), GCS - 10.0 (86% sensitive, 91% specific, AUC 82%), and GCSR -0.85 (100% sensitive, 71% specific, AUC 90%). GLS and GCS can help predict transplant-free survival in patients with hypoplastic left heart syndrome having undergone Fontan palliation. Higher strain values (closer to zero) may be a helpful tool in determining when transplant evaluation is warranted in these patients.

Elevated bile acids are associated with left ventricular structural changes in biliary atresia.

BACKGROUND: In children with biliary atresia (BA), pathologic structural changes within the heart, which define cirrhotic cardiomyopathy, are associated with adverse perioperative outcomes. Despite their clinical relevance, little is known about the pathogenesis and triggers of pathologic remodeling. Bile acid excess causes cardiomyopathy in experimental cirrhosis, but its role in BA is poorly understood. METHODS: Echocardiographic parameters of left ventricular (LV) geometry [LV mass (LVM), LVM indexed to height, left atrial volume indexed to BSA (LAVI), and LV internal diameter (LVID)] were correlated with circulating serum bile acid concentrations in 40 children (52% female) with BA listed for transplantation. A receiver-operating characteristic curve was generated to determine optimal threshold values of bile acids to detect pathologic changes in LV geometry using Youden index. Paraffin-embedded human heart tissue was separately analyzed by immunohistochemistry for the presence of bile acid-sensing Takeda G-protein-coupled membrane receptor type 5. RESULTS: In the cohort, 52% (21/40) of children had abnormal LV geometry; the optimal bile acid concentration to detect this abnormality with 70% sensitivity and 64% specificity was 152 µmol/L (C-statistics=0.68). Children with bile acid concentrations >152 µmol/L had ∼8-fold increased odds of detecting abnormalities in LVM, LVM index, left atrial volume index, and LV internal diameter. Serum bile acids positively correlated with LVM, LVM index, and LV internal diameter. Separately, Takeda G-protein-coupled membrane receptor type 5 protein was detected in myocardial vasculature and cardiomyocytes on immunohistochemistry. CONCLUSION: This association highlights the unique role of bile acids as one of the targetable potential triggers for myocardial structural changes in BA.

The use of neutrophil-lymphocyte ratio for the prediction of refractory disease and coronary artery lesions in patients with Kawasaki disease.

BACKGROUND: Kawasaki disease is a vasculitis that can lead to cardiac complications, including coronary artery disease and cardiogenic shock. Various scoring systems have been developed to determine those that will be refractory to routine intravenous immunoglobulin therapy or develop coronary artery disease. The objective of this study was to determine if the neutrophil-lymphocyte ratio could predict refractory disease and coronary artery lesions in patients with Kawasaki disease. METHODS: A systematic review of the literature was performed to identify manuscripts describing comparisons of neutrophil-lymphocyte ratio between those who had refractory disease and those who did not, and between those who developed coronary artery lesions and those who did not. Mean difference was compared between groups. Areas under the curve were utilised to determine the pooled area under the curve. RESULTS: 12 studies with 5593 patients were included in the final analyses of neutrophil-lymphocyte ratio for the prediction of refractory disease. Neutrophil-lymphocyte ratio before therapy was higher in refractory disease with a mean difference of 2.55 (p < 0.01) and pooled area under the curve of 0.724. Neutrophil-lymphocyte ratio after therapy was higher in refractory disease with a mean difference of 1.42 (p < 0.01) and pooled area under the curve for of 0.803. Five studies with 1690 patients were included in the final analyses of neutrophil-lymphocyte ratio for the prediction of coronary artery lesions. Neutrophil-lymphocyte ratio before therapy was higher in coronary artery lesions with a mean difference of 0.65 (p < 0.01). CONCLUSION: The use of neutrophil-lymphocyte ratio may help physicians in the identification of patients at risk of refractory disease and coronary artery lesions in patients with Kawasaki disease.

Staged vs Complete Repair in Tetralogy of Fallot With Pulmonary Atresia.

BACKGROUND: We sought to compare outcomes for infants with tetralogy of Fallot with pulmonary atresia (TOF/PA) and confluent pulmonary arteries who underwent staged or primary complete surgical repair. METHODS: This retrospective study included infants undergoing initial surgical intervention between 0 and 60 days of age with TOF/PA without aortopulmonary collaterals from 2009 to 2018 at 20 centers. The primary outcome was days alive and out of the hospital in the first year of life (DAOH365). Secondary outcomes were mortality at 1 year of age and a composite major complication outcome. Multivariable modeling with generalized estimating equations were used to compare outcomes between groups. RESULTS: Of 221 subjects, 142 underwent staged repair and 79 underwent primary complete repair. There was no significant difference in median DAOH365 between the staged and primary repair groups (317 days [interquartile range, 278-336] vs 338 days [interquartile range, 314-348], respectively; adjusted P = .13). Nine staged repair patients (7%) died in the first year of life vs 5 primary repair patients (6%; adjusted odds ratio, 1.00; 95% CI, 0.25-3.95). At least 1 major complication occurred in 37% of patients who underwent staged repair vs 41% of patients who underwent primary complete repair (P = .75), largely driven by the need for unplanned cardiac reinterventions. CONCLUSIONS: For infants with TOF/PA with confluent pulmonary arteries, a surgical strategy of staged or primary complete repair resulted in statistically similar DAOH365, early mortality, and morbidity.