Intrapulmonary administration of recombinant activated factor VII in pediatric, adolescent, and young adult oncology and hematopoietic cell transplant patients with pulmonary hemorrhage.

Introduction: Diffuse alveolar hemorrhage (DAH) is a devastating disease process with 50-100% mortality in oncology and hematopoietic cell transplant (HCT) recipients. High concentrations of tissue factors have been demonstrated in the alveolar wall in acute respiratory distress syndrome and DAH, along with elevated levels of tissue factor pathway inhibitors. Activated recombinant factor VII (rFVIIa) activates the tissue factor pathway, successfully overcoming the tissue factor pathway inhibitor (TFPI) inhibition of activation of Factor X. Intrapulmonary administration (IP) of rFVIIa in DAH is described in small case series with successful hemostasis and minimal complications. Methods: We completed a single center retrospective descriptive study of treatment with rFVIIa and outcomes in pediatric oncology and HCT patients with pulmonary hemorrhage at a quaternary hematology/oncology hospital between 2011 and 2019. We aimed to assess the safety and survival of patients with pulmonary hemorrhage who received of IP rFVIIa. Results: We identified 31 patients with pulmonary hemorrhage requiring ICU care. Thirteen patients received intrapulmonary rFVIIa, while eighteen patients did not. Overall, 13 of 31 patients (41.9%) survived ICU discharge. ICU survival (n=6) amongst those in the IP rFVIIa group was 46.2% compared to 38.9% (n=7) in those who did not receive IP therapy (p=0.69). Hospital survival was 46.2% in the IP group and 27.8% in the non-IP group (p=0.45). There were no adverse events noted from use of IP FVIIa. Conclusions: Intrapulmonary rFVIIa can be safely administered in pediatric oncology patients with pulmonary hemorrhage and should be considered a viable treatment option for these patients.

Proinflammatory state promotes red blood cell alloimmunization in pediatric patients with sickle cell disease.

We examined risk factors for red blood cell (RBC) alloimmunization in pediatric patients with sickle cell disease, focusing on the recipients' inflammatory state at the time of transfusion and anti-inflammatory role of hydroxyurea (HU). Among 471 participants, 55 (11.70%) participants were alloimmunized and formed 59 alloantibodies and 17 autoantibodies with an alloimmunization rate of 0.36 alloantibodies per 100 units. Analysis of 27 participants in whom alloantibodies were formed with specificities showed 23.8% (30/126) of units transfused during a proinflammatory event resulting in alloantibody formation compared with 2.8% (27/952) of units transfused at steady state. Therefore, transfusion during proinflammatory events increased the risk for alloimmunization (odds ratio [OR], 4.22; 95% confidence interval [CI], 1.64-10.85; P = .003). Further analysis of all the 471 participants showed that alloimmunization of patients who received episodic transfusion, mostly during proinflammatory events, was not reduced with HU therapy (OR, 6.52; 95% CI, 0.85-49.77; P = .071), HU therapy duration (OR, 1.13; 95% CI, 0.997-1.28; P = .056), or HU dose (OR, 1.06; 95% CI, 0.96-1.16; P = .242). The analysis also identified high transfusion burden (OR, 1.02; 95% CI, 1.003-1.04; P = .020) and hemoglobin S (HbSS) and HbSß0-thalassemia genotypes (OR, 11.22, 95% CI, 1.51-83.38; P = .018) as additional risk factors for alloimmunization. In conclusion, the inflammatory state of transfusion recipients affects the risk of RBC alloimmunization, which is not modified by HU therapy. Judicious use of transfusion during proinflammatory events is critical for preventing alloimmunization.

Optimizing haematopoietic stem and progenitor cell apheresis collection from plerixafor-mobilized patients with sickle cell disease.

We adjusted haematopoietic stem and progenitor cell (HSPC) apheresis collection from patients with sickle cell disease (SCD) by targeting deep buffy coat collection using medium or low collection preference (CP), and by increasing anticoagulant-citrate-dextrose-solution A dosage. In 43 HSPC collections from plerixafor-mobilized adult patients with SCD, we increased the collection efficiency to 35.79% using medium CP and 82.23% using low CP. Deep buffy coat collection increased red blood cell contamination of the HSPC product, the product haematocrit was 4.7% with medium CP and 6.4% with low CP. These adjustments were well-tolerated and allowed efficient HSPC collection from SCD patients.

Gabapentin for acute pain in sickle cell disease: A randomized double-blinded placebo-controlled phase II clinical trial.

Pain in sickle cell disease (SCD) can have a neuropathic component. This randomized phase II double-blinded placebo-controlled study evaluated the efficacy of gabapentin in reducing pain and opioid consumption (morphine-equivalent dose [MED]) during acute vaso-occlusive crisis (VOC). Of 90 patients aged 1-18 years with VOC pain, 45 were randomized to a single gabapentin dose (15 mg/kg) and 45 to placebo, in addition to standard treatment; 42 and 44 patients were evaluable in the gabapentin and placebo arms, respectively. A decrease in pain of ≥33% was reported in 68% of patients in the gabapentin arm and 60% of those in the placebo arm (one-sided p = 0.23). The median MED (mg/kg) in the gabapentin (0.12) and placebo arms (0.13) was similar (p = 0.9). However, in the subset of patients with the HbSS genotype (n = 45), the mean (SD) absolute pain score decrease by the time of discharge was significantly greater in the gabapentin arm (5.9 [3.5]) than in the placebo arm (3.6 [3.3]) (p = 0.032). Pain scores in the overall study population were not significantly reduced when gabapentin was added to standard treatment; however, gabapentin benefited individuals with the more severe genotype, HbSS, during acute VOC. Larger, prospective studies are needed to confirm these findings.

Comparison of inhospital outcomes of pediatric heart transplantation between single ventricle congenital heart disease and cardiomyopathy.

Data investigating the impact of household income and other factors on SV patient status-post-Fontan palliation after heart transplantation are lacking. We aim to evaluate factors affecting outcomes after OHT in this population. The PHIS database was interrogated for either SV or myocarditis/primary CM who were 4 years or older who underwent a single OHT. There were 1599 patients with a median age of 13.2 years (IQR: 9.3-16.1). Total hospital costs were significantly higher in the SV group ($408 000 vs $294 000, P < 0.0001), but as median household income increased, the risk of inhospital mortality, post-transplant LOS, and LOS-adjusted total hospital costs all decreased. The risk of inhospital mortality increased 6.5% per 1 year of age increase at the time of transplant. Patients in the SV group had significantly more diagnoses than those in the CM group (21 vs 15, P < 0.0001) and had longer total hospital LOSs as a result of longer post-transplant courses (25 days vs 15, P < 0.0001). Increased median household income and younger age are associated with decreased resource utilization and improved inhospital mortality in SV CHD patients who undergo OHT. In conclusion, earlier consideration of OHT in this population, coupled with improved selection criteria, may increase survival in this population.

Epidemiologic Trends of Adoption of Do-Not-Resuscitate Status After Pediatric In-Hospital Cardiac Arrest.

OBJECTIVES: To evaluate the prevalence of do-not-resuscitate status, assess the epidemiologic trends of do-not-resuscitate status, and assess the factors associated with do-not-resuscitate status in children after in-hospital cardiac arrest using large, multi-institutional data. DESIGN: Generalized estimating equations logistic regression model was used to evaluate the trends of do-not-resuscitate status and evaluate the factors associated with do-not-resuscitate status after cardiac arrest. SETTING: American Heart Association's Get With the Guidelines-Resuscitation Registry. PATIENTS: Children (< 18 yr old) with an index in-hospital cardiac arrest and greater than or equal to 1 minute of documented chest compressions were included (2006-2015). Patients with no return of spontaneous circulation after cardiac arrest were excluded. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: In total, 8,062 patients qualified for inclusion. Of these, 1,160 patients (14.4%) adopted do-not-resuscitate status after cardiac arrest. We found low rates of survival to hospital discharge among children with do-not-resuscitate status (do-not-resuscitate vs no do-not-resuscitate: 6.0% vs 69.7%). Our study found that rates of do-not-resuscitate status after cardiac arrest are highest in children with Hispanic ethnicity (16.4%), white race (15.0%), and treatment at institutions with larger PICUs (> 50 PICU beds: 17.8%) and at institutions located in North Central (17.6%) and South Atlantic/Puerto Rico (17.1%) regions of the United States. Do-not-resuscitate status was more common among patients with more preexisting conditions, longer duration of cardiac arrest, greater than 1 cardiac arrest, and among patients requiring extracorporeal cardiopulmonary resuscitation. We also found that trends of do-not-resuscitate status after cardiac arrest in children are decreasing in recent years (2013-2015: 13.8%), compared with previous years (2006-2009: 16.0%). CONCLUSIONS: Patient-, hospital-, and regional-level factors are associated with do-not-resuscitate status after pediatric cardiac arrest. As cardiac arrest might be a signal of terminal chronic illness, a timely discussion of do-not-resuscitate status after cardiac arrest might help families prioritize quality of end-of-life care.

Impact of Regional Cerebral Perfusion on Outcomes Among Neonates Undergoing Norwood Operation.

OBJECTIVES: To evaluate the impact of regional cerebral perfusion (RCP) during heart operation on outcomes in neonates undergoing Norwood operation. METHODS: We performed a retrospective cohort study using data from the Single Ventricle Reconstruction trial data set. The adjusted effect of RCP use on each outcome was studied using a penalized logistic regression model with bootstrap validation. RESULTS: Of 549 patients included in the study, 252 patients (45.9%) received RCP during their heart operation. In univariate comparisons, the majority of the baseline characteristics and preoperative risk factors were similar in the RCP and No RCP group. The total cardiopulmonary bypass (CPB) time and the total cross-clamp (CC) time were longer in the RCP group (RCP vs No RCP, median CPB time: 161 minutes vs 109 minutes; median CC time: 63 minutes vs 43 minutes). In adjusted models, the use of RCP was not associated with decreased mortality and/or need for heart transplant at hospital discharge (odds ratio [OR]: 0.73; 95% confidence interval [CI]: 0.43-1.25) or prolonged mechanical ventilation (OR: 1.20, 95% CI: 0.62-2.28) or prolonged hospital length of stay (OR: 1.30, 95% CI: 0.73-2.30). We demonstrated that use of RCP was associated with longer CPB times, increased use of ultrafiltration, and higher probability of open chest after Norwood operation. CONCLUSIONS: This study did not demonstrate any impact of RCP on in-hospital mortality and/or heart transplantation, prolonged mechanical ventilation, and prolonged hospital length of stay among neonates undergoing Norwood operation.

Creation of an empiric tool to predict ECMO deployment in pediatric respiratory or cardiac failure.

PURPOSE: To create a real-time prediction tool to predict probability of ECMO deployment in children with cardiac or pulmonary failure. MATERIALS AND METHODS: Patients ≤18 years old admitted to an ICU that participated in the Virtual Pediatric Systems database (2009-2015) were included. Logistic regression models using adaptive lasso methodology were used to identify independent factors associated with ECMO use. RESULTS: A total of 538,202 ICU patients from 140 ICUs qualified for inclusion. ECMO was deployed in 3484 patients (0.6%) with a mortality of 1450 patients (41.6%). The factors associated with increased probability of ECMO use included: younger age, pulmonary hypertension, congenital heart disease, high-complexity cardiac surgery, cardiomyopathy, acute lung injury, shock, renal failure, cardiac arrest, use of nitric oxide, use of either conventional mechanical ventilation or high frequency oscillatory ventilation, and higher annual ECMO center volume. The area under the receiver operating curve for this model was 0.90 (95% CI: 0.85-0.93). This tool can be accessed at https://soipredictiontool.shinyapps.io/ECMORisk/. CONCLUSIONS: Here, we present a tool to predict ECMO deployment among critically ill children; this tool will help create real-time risk stratification among critically ill children, and it will help with benchmarking, family counseling, and research.

Utility of Follow-Up Annual Echocardiograms in Patients With Complete Transposition of the Great Arteries After Arterial Switch Operations.

The arterial switch operation (ASO) in complete transposition of the great arteries (TGA) has increased long-term survival. Annual follow-up echocardiograms are recommended, but evidence-based guidelines do not exist. We sought to assess how often a patient with TGA after ASO who had no symptoms or change in physical exam underwent an intervention based solely on echocardiographic changes. We retrospectively reviewed all records from patients with TGA and a history of ASO followed at our institution between November 1983 and January 2015. Changes in echocardiograms resulting in hospital admission, significant medication change, interventional catheterization, or surgical procedure were identified through the surgical and cardiac catheterization laboratory databases and patient charts. These changes were referred to as an actionable change (AC). Interventions were defined as being driven by either clinical (change in physical exam, patient and/or parental concerns) or echocardiographic findings. A total of 1,792 echocardiograms from 149 patients were reviewed. Median number of echocardiograms per patient was 12 (1 to 34). Of the 1,792 echocardiograms, 20 (1.12%) were associated with AC. The most common intervention for an AC was cardiac catheterization (13 of 20, 65%). Most AC (15 of 20, 75%) occurred in the first decade after ASO. AC occurred in 83% (5 of 6) of those with a history of both ASO and arch repair. Annual echocardiograms in patients with TGA after ASO are rarely useful and are unnecessary. In conclusion, decreasing surveillance of asymptomatic patients to biennial follow-up echocardiograms in asymptomatic patients without physical examination changes is safe and would decrease medical expenses.

Long-Term Neurodevelopment of Low-Birthweight, Preterm Infants with Patent Ductus Arteriosus.

OBJECTIVE: To evaluate whether the presence of patent ductus arteriosus (PDA) in preterm infants worsens long-term neurodevelopmental outcomes. STUDY DESIGN: This was a secondary observational analysis of data from 1090 preterm low-birthweight infants in the Infant Health and Development Program (IHDP), a multicenter longitudinal cohort study of outcomes assessed from 3 to 18 years of age. Multivariable analysis was adjusted for IHDP treatment group (intervention or follow-up), birth weight, maternal race, maternal education, infant sex, maternal preconception weight, Home Observation Measurement of the Environment (HOME) total score at 12 months, neonatal health index, and gestational age. RESULTS: Of the 1090 patients (49% male) included in the analysis, 135 had a PDA. Mean birth weight (1322 g vs 1871 g; P < .0001) and gestational age (30.2 weeks vs 33.4 weeks, P < .0001) were lower and mean ventilator days (11.8 vs 1.3; P < .0001), vasopressor use (12.6% vs 1.2%; P < .0001), and congestive heart failure (8.9% vs 0.1%; P < .0001) were higher in the PDA group. There were no differences between the PDA and no-PDA groups in maternal education level and HOME total score at age 12 months. Multivariable analysis demonstrated no between-group differences in cognitive development or behavioral competence at age 3, 8, and 18 years. CONCLUSIONS: The presence of a PDA in moderately preterm, low-birthweight infants does not impact long-term neurodevelopmental outcomes.

Magnitude and Pace of Improvement in Performance of Hospitals Treating Mechanically Ventilated Children in the United States: Analysis From Two National Databases.

OBJECTIVES: To use two national databases to quantify the pace and magnitude of improvement in hospital performance (as measured by mortality) across hospitals caring for critically ill children in the United States. DESIGN: We used empirical Bayes shrinkage estimators to obtain shrinkage estimators of observed/expected mortality ratios for each hospital assuming a Gamma Poisson posterior distribution. Revised mortality rates for each hospital were obtained from the shrunken incidence ratios. SETTING: Pediatric Health Information System participating hospital and Kids' Inpatient Database participating hospital. PATIENTS: Patients less than or equal to 18 years old who received invasive mechanical ventilation during their hospital stay at a Pediatric Health Information System participating hospital (2005-2015) or a Kids' Inpatient Database participating hospital (1997-2012) were included. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: A total of 486,838 patients from 48 Pediatric Health Information System hospitals and 798,840 patients from 947 Kids' Inpatient Database hospitals were included. For the Pediatric Health Information System hospitals, the median shrunken adjusted mortality decreased from 11.66% in 2005 to 7.11% in 2015; for the Kids' Inpatient Database hospitals, it decreased from 5.79% in 1997 to 3.86% in 2012. By 2015, more than 95% of the Pediatric Health Information System hospitals performed better than or as well as the best 25% of the hospitals in 2005. By 2012, 33.7% of Kids' Inpatient Database hospitals performed better than or as well as the best 25% of the hospitals in 1997. CONCLUSIONS: This study provides insight into the magnitude of improvement in patient mortality in hospitals caring for critically ill children in the United States. This study quantifies hospital performance in pediatric critical care over time, and it provides benchmarks against which individual hospitals can assess their own performance. In future pediatric epidemiologic studies, we should identify outcomes other than mortality to quantify improvement in hospital performance.

Incidence of Immediate Extubation After Pediatric Cardiac Surgery and Predictors for Reintubation.

OBJECTIVES: The objectives were to assess the incidence of immediate tracheal extubation in the operating room after pediatric cardiac surgery and to investigate predictors for subsequent reintubation. METHODS: This is a single institutional retrospective study including all patients who had a cardiac operation with cardiopulmonary bypass from 2011 to 2016. Patients who required preoperative ventilator support, postoperative open chest, or mechanical support were excluded. Predictors for reintubation after immediate extubation were analyzed only for patients with stage II palliation for single ventricle physiology. RESULTS: Nine hundred nine qualifying operations were identified. Immediate extubation was performed in 590 (64.9%) operations. A multivariable logistic regression model showed that the identities of anesthesiologist ( P = .0003), year of the operation performed ( P < .001), cardiopulmonary bypass time ( P < .001), and type of operations ( P < .001) were significantly associated with immediate extubation. Reintubation was significantly less frequent in patients with immediate extubation compared to those without (6.1% vs 15.0%; P < .001). A subgroup analysis for stage II palliation showed that reintubation after immediate extubation was significant for younger age (0.42 vs 0.54 years, P = .044), lower Po2/Fio2 and Po2 at the last blood gas analysis (66 vs 98 mm Hg, P = .032 and 39 vs 47 mm Hg, P = .008), and higher inotropic score (2 vs 0, P = .034). A multivariable logistic regression model showed that only inotropic score was significantly associated with reintubation ( P = .018). CONCLUSIONS: Immediate extubation in the operating room after pediatric cardiac surgery can be performed in most patients. Inotropic score is a predictor for reintubation in stage II palliation.

Reclassifying by highest complexity operation rather than first operation influences mortality after pediatric heart surgery.

OBJECTIVE: To evaluate the effect on mortality of reclassifying patients undergoing pediatric heart reoperations of varying complexity by operation of highest complexity instead of by first operation. METHODS: Data from the Virtual Pediatric Systems Database on children aged < 18 years who underwent heart surgery (with or without cardiopulmonary bypass) were included (2009-2015). Only patients who underwent reoperations during the same hospitalization were included. Patients were classified based on the first cardiovascular operation (the index operation), and on the complexity of the operation (the operation with the highest Society of Thoracic Surgeons-European Association for Cardio-Thoracic Surgery [STAT] mortality category of each hospital admission) performed. RESULTS: Of 51,047 patients (73 centers), 22,393 met inclusion criteria. Using index operation as the classifying operation, the number of patients classified in the STAT 1 category increased by approximately 2.5 times compared with the highest-complexity operation (index, 7,077 and highest complexity, 2,654). In contrast, when the highest-complexity classification was used, we noted an increase in the number of patients in other STAT categories. We also noted higher mortality in all STAT categories when patients were classified by index operation instead of by highest complexity (index vs highest STAT category 1, 0.6% vs 0.2%; category 2, 2.4% vs 0.8%; category 3, 3.1% vs 2.1%; category 4, 5.8% vs 5.6%; and category 5, 16.7% vs 16.5%). CONCLUSIONS: This study demonstrates differences in the reported number of patients and reported mortality in each STAT category among children undergoing various heart reoperations during the same hospitalization by classifying patients based on index operation compared with the operation of highest complexity.

Comparison of the Effect of Cycloplegia on Astigmatism Measurements in a Pediatric Amblyopic Population: A Prospective Study.

PURPOSE: To study the effect of cycloplegia on astigmatism measurements in pediatric patients with amblyopia. METHODS: This was a prospective comparative clinical study. Participants 4 to 17 years old were recruited from the patient population at the Arkansas Children's Hospital eye clinic after informed consent was obtained. Autorefractor measurements were used to obtain values of refractive error in amblyopic and non-amblyopic patients before and after cycloplegia. The groups were subdivided into myopia and hyperopia and with and without underlying amblyopia. The refractive error was expressed as sphere, cylinder, axis of astigmatism, and spherical equivalent. The treatment effect was summarized as the mean difference (95% confidence interval) for each outcome. RESULTS: No statistically significant difference was found on the axis and power of astigmatism before and after cycloplegia in the patients with amblyopia (P = .28 and .99, respectively). CONCLUSIONS: Non-cycloplegic autorefraction measurements may be considered safe for refining astigmatism power and axis in pediatric patients with amblyopia. [J Pediatr Ophthalmol Strabismus. 2018;55(5):293-298.].

Short-Axis Diastolic Ventricular Area Ratio as a New Index in Screening Patients with Repaired Tetralogy of Fallot.

Right ventricular (RV) end-diastolic volume measured by cardiovascular magnetic resonance imaging (CMR) is a criterion for pulmonary valve replacement in patients with tetralogy of Fallot (TOF). We sought to determine if the ratio of echocardiographic, short-axis RV-to-left ventricular (LV) end-diastolic areas (EDA) could be used to predict RV volume on CMR. We retrospectively reviewed the echocardiograms of all patients with repaired TOF who underwent CMR at our institution from 2011 to 2015 and also had an echocardiogram within 6 months of the CMR. The short-axis RV and LV EDAs were measured and the ratio of the two was calculated. Results were compared with CMR RV end-diastolic volume index (RVEDVi) and RV:LV end-diastolic volume ratio. The sensitivity and specificity values predicting RV volumes > 150 ml/m2 were calculated. Fifty-eight studies met inclusion criteria. There were 47 studies with RVEDVi < 150 ml/m2 and 11 with RVEDVi > 150 ml/m2. RV:LV EDA and CMR RV:LV end-diastolic volume ratio correlated strongly (r = 0.76, p < 0.0001). An RV:LV EDA ≥ 1.57 had a 90% sensitivity to predict RVEDVi > 150 ml/m2 (area under the curve = 0.74, 95% CI 1.5-27.9; p = 0.012). An RV:LV EDA ≥ 1.88 had an 81% specificity to detect RV volume index > 150 ml/m2. Short-axis RV:LV EDA correlates well with an increased RVEDVi as measured by CMR. This new and simple measure can be used to predict optimal timing for CMR in anticipation of pulmonary valve replacement in repaired TOF.

Impact of intensive care unit attending physician training background on outcomes in children undergoing heart operations.

BACKGROUND: The existing training pathways to become a pediatric cardiac intensivist are very variable with physicians coming from varied training backgrounds of pediatric critical care, pediatric cardiology, neonatology, or pediatric anesthesia. AIM: To evaluate the impact of cardiac Intensive Care Unit (ICU) attending physician training background on outcomes in children undergoing heart operations. SETTING AND DESIGN: Patients in the age group from 1 day to 18 years undergoing heart operation at a Pediatric Health Information System database participating hospital were included (2010-2015). PATIENTS AND METHODS: Based on the training background of majority of attending physicians in an ICU, the participating ICUs were divided into three groups: critical care medicine (CCM), cardiology, and indeterminate. STATISTICAL ANALYSIS: Multivariable logistic regression models were fitted to evaluate the association of ICU physician training background with study outcomes. RESULTS: A total of 54,935 patients from 42 ICUs were included. Of these, 31,815 patients (58%) were treated in the CCM group (26 ICUs), 19,340 patients (35%) were treated in the cardiology group (12 ICUs), and 3780 patients (7%) were treated in the indeterminate group (4 ICUs). In adjusted models, no specific group based on ICU attending physician training background was associated with lower mortality (CCM vs. cardiology, odds ratio: 0.75, 95% confidence interval: 0.48-1.18), or lower incidence of cardiac arrest, or prolonged hospital length of stay, or prolonged mechanical ventilation. CONCLUSIONS: This large observational study did not demonstrate any impact of ICU attending training background on outcomes in children undergoing heart operations.

An Empirically Derived Pediatric Cardiac Inotrope Score Associated With Pediatric Heart Surgery.

We aimed to empirically derive an inotrope score to predict real-time outcomes using the doses of inotropes after pediatric cardiac surgery. The outcomes evaluated included in-hospital mortality, prolonged hospital length of stay, and composite poor outcome (mortality or prolonged hospital length of stay). The study population included patients <18 years of age undergoing heart operations (with or without cardiopulmonary bypass) of varying complexity. To create this novel pediatric cardiac inotrope score (PCIS), we collected the data on the highest doses of 4 commonly used inotropes (epinephrine, norepinephrine, dopamine, and milrinone) in the first 24 hours after heart operation. We employed a hierarchical framework by representing discrete probability models with continuous latent variables that depended on the dosage of drugs for a particular patient. We used Bayesian conditional probit regression to model the effects of the inotropes on the mean of the latent variables. We then used Markov chain Monte Carlo simulations for simulating posterior samples to create a score function for each of the study outcomes. The training dataset utilized 1030 patients to make the scientific model. An online calculator for the tool can be accessed at https://soipredictiontool.shinyapps.io/InotropeScoreApp. The newly proposed empiric PCIS demonstrated a high degree of discrimination for predicting study outcomes in children undergoing heart operations. The newly proposed empiric PCIS provides a novel measure to predict real-time outcomes using the doses of inotropes among children undergoing heart operations of varying complexity.

Is Magnet® recognition associated with improved outcomes among critically ill children treated at freestanding children's hospitals?

PURPOSE: With increasing emphasis on high-quality care, we designed this study to evaluate the relationship between Magnet® recognition and patient outcomes in pediatric critical care. MATERIALS AND METHODS: Post hoc analysis of data from an existing administrative national database. We used inverse probability of treatment weighting and multivariate models to compare outcomes between two study groups after adjusting for confounding variables. RESULTS: A total of 823,634 pediatric patients from 41 centers were included. Of these, 454,616 patients (55.2%) were treated in 23 Magnet hospitals. The majority of baseline characteristics did not vary significantly among the two study groups. In adjusted models, there was no difference in mortality between the two groups (Magnet vs. non-Magnet; odds ratio: 0.92, 95% confidence interval: 0.77-1.11). When stratified by various subgroups, such as cardiac, non-cardiac, ECMO, cardiac arrest, respiratory failure, use of nitric oxide, genetic abnormality etc., Magnet status of the hospital did not confer a survival advantage. In a sensitivity analysis on patients from crossover hospitals only, attainment of magnet status was associated with increased hospital charges. CONCLUSIONS: This large observational study calls into question the utility of the Magnet Recognition Program among children with critical illness, at least among the freestanding children's hospitals.

Impact of Bicuspid Aortic Valve Morphology on Aortic Valve Disease and Aortic Dilation in Pediatric Patients.

Bicuspid aortic valve (BAV) is the most common congenital heart defect. BAV is associated with aortic stenosis and insufficiency, and aortic dilation in adult groups, but data in pediatric groups are limited. We sought to assess the impact of BAV morphology on aortic valve disease and aortic dilation in pediatric patients. We performed a retrospective review of all echocardiograms in patients with isolated BAV who were followed at our institution from July 2002 to July 2012. BAV morphology, aortic valve stenosis and/or insufficiency, and aortic dimensions were measured manually. Comparisons were made between right-left cusp fusion (RL) and right-noncoronary cusp fusion (RN) BAV morphologies. Generalized least square models were fit to analyze the impact of specific variables on aortic dilation. There were 1075 echocardiograms in 366 patients (72% male) with isolated BAV. Aortic valve insufficiency and stenosis were more common in RN (p < 0.001 for both). The median aortic sinus Z score was higher in the RL (0.47; IQR - 0.31 to 1.44) than in the RN group (0.02; - 0.83 to 0.82) (p < 0.001). There was no difference in median ascending aorta Z score between groups. Patients with the highest weights had larger aortas (p < 0.001), but the absolute difference between the highest and lowest weight groups was small (1.5 mm). The impact of BAV morphology on aortic valve disease and aortic dilation in pediatric patients presages that seen in adults. Patient body weight does not make significant clinical impacts on aortic diameters, suggesting that Z scores for aortic diameters should be based on ideal body weights.

Development and Validation of an Empiric Tool to Predict Favorable Neurologic Outcomes Among PICU Patients.

OBJECTIVES: To create a novel tool to predict favorable neurologic outcomes during ICU stay among children with critical illness. DESIGN: Logistic regression models using adaptive lasso methodology were used to identify independent factors associated with favorable neurologic outcomes. A mixed effects logistic regression model was used to create the final prediction model including all predictors selected from the lasso model. Model validation was performed using a 10-fold internal cross-validation approach. SETTING: Virtual Pediatric Systems (VPS, LLC, Los Angeles, CA) database. PATIENTS: Patients less than 18 years old admitted to one of the participating ICUs in the Virtual Pediatric Systems database were included (2009-2015). INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: A total of 160,570 patients from 90 hospitals qualified for inclusion. Of these, 1,675 patients (1.04%) were associated with a decline in Pediatric Cerebral Performance Category scale by at least 2 between ICU admission and ICU discharge (unfavorable neurologic outcome). The independent factors associated with unfavorable neurologic outcome included higher weight at ICU admission, higher Pediatric Index of Morality-2 score at ICU admission, cardiac arrest, stroke, seizures, head/nonhead trauma, use of conventional mechanical ventilation and high-frequency oscillatory ventilation, prolonged hospital length of ICU stay, and prolonged use of mechanical ventilation. The presence of chromosomal anomaly, cardiac surgery, and utilization of nitric oxide were associated with favorable neurologic outcome. The final online prediction tool can be accessed at https://soipredictiontool.shinyapps.io/GNOScore/. Our model predicted 139,688 patients with favorable neurologic outcomes in an internal validation sample when the observed number of patients with favorable neurologic outcomes was among 139,591 patients. The area under the receiver operating curve for the validation model was 0.90. CONCLUSIONS: This proposed prediction tool encompasses 20 risk factors into one probability to predict favorable neurologic outcome during ICU stay among children with critical illness. Future studies should seek external validation and improved discrimination of this prediction tool.