Emergency Department Use and Hospital Mortality Among Heart Transplant Recipients in the United States.

BACKGROUND: Annual heart transplant (HT) volumes have increased, as have post-HT outpatient care needs. Data on HT-related emergency department (ED) visits are limited. METHODS AND RESULTS: A retrospective analysis of 177 450 HT patient ED visits from the 2009 to 2018 Nationwide Emergency Department Sample was conducted. HT recipients, primary diagnoses, and comorbidities associated with ED visits were identified via International Classification of Diseases, Ninth Revision (ICD-9) and International Classification of Diseases, Tenth Revision (ICD-10) codes. Multivariable logistic regression was used to predict outcomes of hospital admission and death. HT volumes and HT-related ED visits increased from 2009 to 2018. Infection was the most common primary diagnosis (24%), and cardiac primary diagnoses represented 10% of encounters. Hospital admissions occurred in 48% of visits, but overall mortality was low (1.6%). Length of stay was 3.1 days (interquartile range, 1.6-5.9 days), and comorbidity burden was high: 42% had hypertension, 38% had diabetes, and 31% had ≥2 comorbidities. Those aged ≥65 years had significantly higher odds of admission (odds ratio [OR], 2.14 [95% CI, 1.97-2.33]) and death (OR, 2.06 [95% CI, 1.61-2.62]). Comorbidities increased odds of admission (OR, 1.62 [95% CI, 1.51-1.75]) but not death. Renal primary diagnosis had the highest risk of admission (OR, 4.1 [95% CI, 3.6-4.6]), but cardiac primary diagnosis had the highest odds of death (OR, 11.6 [95% CI, 9.1-14.8]). CONCLUSIONS: HT-related ED visits increased from 2009 to 2018 with high admission rates but low in-hospital mortality, suggesting an opportunity to improve prehospital care. Older patients and those with cardiac primary diagnoses had the highest risk of death. The observed contrast between predictors of admission and mortality signals a need for further study to improve risk stratification and outpatient care strategies.

Central venous access in children: Placement trends over the last decade.

PURPOSE: To evaluate central venous access placement trends for radiology and non-radiology services over the last decade. MATERIALS AND METHODS: Children who had central venous access procedures included in a large administrative database of 49 pediatric institutions in the United States between 2010 and 2020 were included. Patient demographics and patient specific factors were compared between groups. The percentage of procedures performed by interventional radiology (IR) and non-radiology services were compared over time and by region. RESULTS: A total of 483,181 vascular access encounters were recorded (45.3% female; median age 2 years (IQR 0-11 years)). Approximately one quarter of vascular access encounters were IR-led, with a slight increase of 3.8% between 2010 and 2020. Children who underwent IR-placed vascular access were older (median age of 4 years compared to 1 year in non-radiology encounters). Interventional radiology-placed access was greatest in the Midwest (33.5%) with a decrease of 5.9% over the study period; in the other three regions, IR-performed encounters increased. Patient comorbidities more prevalent in the IR encounters were technology dependence (42.4% of all radiology encounters), gastrointestinal (34.9%), respiratory (20.8%), and transplant (8.1%), while those which were more prevalent in the non-radiology encounters were nephrology/urology (21.4% of all non-radiology encounters), prematurity/neonatal (17.3%), and malignancy (17.3%). CONCLUSIONS: Interventional radiology-provided vascular access services have slightly increased over the last decade without significant service-line transfer to other specialties. Underlying comorbidities in IR-led vascular access encounters vary across institutions based on referral patterns, possibly reflecting the adoption of ultrasound guidance by other pediatric subspecialties.

The Effect of Psychiatric Comorbidity on Healthcare Utilization for Youth With Newly Diagnosed Systemic Lupus Erythematosus.

OBJECTIVE: To examine the effect of psychiatric diagnoses on healthcare use in youth with systemic lupus erythematosus (SLE) during their first year of SLE care. METHODS: We conducted a retrospective cohort study using claims from 2000 to 2013 from Clinformatics Data Mart (OptumInsight). Youth aged 10 years to 24 years with an incident diagnosis of SLE (≥ 3 International Classification of Diseases, 9th revision, codes for SLE 710.0, > 30 days apart) were categorized as having: (1) a preceding psychiatric diagnosis in the year before SLE diagnosis, (2) an incident psychiatric diagnosis in the year after SLE diagnosis, or (3) no psychiatric diagnosis. We compared ambulatory, emergency, and inpatient visits in the year after SLE diagnosis, stratified by nonpsychiatric and psychiatric visits. We examined the effect of childhood-onset vs adult-onset SLE by testing for an interaction between age and psychiatric exposure on outcome. RESULTS: We identified 650 youth with an incident diagnosis of SLE, of which 122 (19%) had a preceding psychiatric diagnosis and 105 (16%) had an incident psychiatric diagnosis. Compared with those without a psychiatric diagnosis, youth with SLE and a preceding or incident psychiatric diagnosis had more healthcare use across both ambulatory and emergency settings for both nonpsychiatric and psychiatric-related care. These associations were minimally affected by age at time of SLE diagnosis. CONCLUSION: Psychiatric comorbidity is common among youth with newly diagnosed SLE and is associated with greater healthcare use. Interventions to address preceding and incident psychiatric comorbidity may decrease healthcare burden for youth with SLE.

Impact of Device Miniaturization on Insertable Cardiac Monitor Use in the Pediatric Population: An Analysis of the MarketScan Commercial and Medicaid Databases.

Background Insertable cardiac monitors (ICMs) are effective in the detection of paroxysmal arrhythmias. In 2014, the first miniaturized ICM was introduced with a less invasive implant technique. The impact of this technology on ICM use in pediatric patients has not been evaluated. We hypothesized an increase in annual pediatric ICM implants starting in 2014 attributable to device miniaturization. Methods and Results A retrospective observational study was conducted using administrative claims from MarketScan Medicaid and commercial insurance claims databases. Use of ICM between January 2013 and December 2018 was measured (normalized to the total enrolled population ≤18 years) and compared with balancing measures (Holter ambulatory monitors, cardiac event monitors, encounters with syncope diagnosis, implantation of implantable cardioverter-defibrillator/pacemaker). Secondary analyses included evaluations of subsequent interventions and complications. The study cohort included 33 532 185 individual subjects, of which 769 (0.002%) underwent ICM implantation. Subjects who underwent ICM implantation were 52% male sex, with a median age of 16 years (interquartile range, 10-17 years). A history of syncope was present in 71%, palpitations in 43%, and congenital heart disease in 28%. Following release of the miniaturized ICM, use of ICMs increased from 5 procedures per million enrollees in 2013 to 11 per million between 2015 and 2018 (P<0.001), while balancing measures remained static. Of 394 subjects with ≥1 year of follow-up after implantation, interventions included catheter ablation in 24 (6%), pacemaker implantation in 15 (4%), and implantable cardioverter-defibrillator implantation in 7 (2%). Conclusions Introduction of the miniaturized ICM was followed by a rapid increase in pediatric use. The effects on outcomes and value deserve further attention.

Sex Differences in Left Ventricular Assist Device-related Emergency Department Encounters in the United States.

BACKGROUND: There is a paucity of data regarding sex differences in the profiles and outcomes of ambulatory patients on left ventricular assist device (LVAD) support who present to the emergency department (ED). METHODS AND RESULTS: We performed a retrospective analysis of 57,200 LVAD-related ED patient encounters from the 2010 to 2018 Nationwide Emergency Department Sample. International Classification of Diseases Clinical Modification, Ninth Revision and Tenth Revision, codes identified patients aged 18 years or older with LVADs and associated primary and comorbidity diagnoses. Clinical characteristics and outcomes were stratified by sex and compared. Multivariable logistic regression was used to evaluate predictors of hospital admission and death. Female patient encounters comprised 27.2% of ED visits and occurred at younger ages and more frequently with obesity and depression (all P < .01). There were no sex differences in presentation for device complication, stroke, infection, or heart failure (all P > .05); however, female patient encounters were more often respiratory- and genitourinary or gynecological related (both P < .01). After adjustment for age group, diabetes, depression, and hypertension, male patient encounters had a 38% increased odds of hospital admission (95% confidence interval 1.20-1.58), but there was no sex difference in the adjusted odds of death (odds ratio 1.11, 95% confidence interval 0.86-1.45). CONCLUSIONS: Patient encounters of females on LVAD support have significantly different comorbidities and outcomes compared with males. Further inquiry into these sex differences is imperative to improve long-term outcomes.

Practice Variation in Use of Neuroimaging Among Infants With Concern for Abuse Treated in Children's Hospitals.

Importance: Infants who appear neurologically well and have fractures concerning for abuse are at increased risk for clinically occult head injuries. Evidence of excess variation in neuroimaging practices when abuse is suspected may indicate opportunity for quality and safety improvement. Objective: To quantify neuroimaging practice variation across children's hospitals among infants with fractures evaluated for abuse, with the hypothesis that hospitals would vary substantially in neuroimaging practices. As a secondary objective, factors associated with neuroimaging use were identified, with the hypothesis that age and factors associated with potential biases (ie, payer type and race or ethnicity) would be associated with neuroimaging use. Design, Setting, and Participants: This cross-sectional study included infants with a femur or humerus fracture or both undergoing abuse evaluation at 44 select US children's hospitals in the Pediatric Health Information System (PHIS) from January 1, 2016, through March 30, 2020, including emergency department, observational, and inpatient encounters. Included infants were aged younger than 12 months with a femur or humerus fracture or both without overt signs or symptoms of head injury for whom a skeletal survey was performed. To focus on infants at increased risk for clinically occult head injuries, infants with billing codes suggestive of overt neurologic signs or symptoms were excluded. Multivariable logistic regression was used to investigate demographic, clinical, and temporal factors associated with use of neuroimaging. Marginal standardization was used to report adjusted percentages of infants undergoing neuroimaging by hospital and payer type. Data were analyzed from March 2021 through January 2022. Exposures: Covariates included age, sex, race and ethnicity, payer type, fracture type, presentation year, and hospital. Main Outcomes and Measures: Use of neuroimaging by CT or MRI. Results: Of 2585 infants with humerus or femur fracture or both undergoing evaluations for possible child abuse, there were 1408 (54.5%) male infants, 1726 infants (66.8%) who were publicly insured, and 1549 infants (59.9%) who underwent neuroimaging. The median (IQR) age was 6.1 (3.2-8.3) months. There were 748 (28.9%) Black non-Hispanic infants, 426 (16.5%) Hispanic infants, 1148 (44.4%) White non-Hispanic infants. In multivariable analyses, younger age (eg, odds ratio [OR] for ages <3 months vs ages 9 to <12 months, 13.2; 95% CI, 9.54-18.2; P < .001), male sex (OR, 1.47; 95% CI, 1.22-1.78; P < .001), payer type (OR for public vs private insurance, 1.48; 95% CI, 1.18-1.85; P = .003), fracture type (OR for femur and humerus fracture vs isolated femur fracture, 5.36; 95% CI, 2.11-13.6; P = .002), and hospital (adjusted range in use of neuroimaging, 37.4% [95% CI 21.4%-53.5%] to 83.6% [95% CI 69.6%-97.5%]; P < .001) were associated with increased use of neuroimaging, but race and ethnicity were not. Publicly insured infants were more likely to undergo neuroimaging (62.0%; 95% CI, 60.0%-64.1%) than privately insured infants (55.1%; 95% CI, 51.8%-58.4%) (P = .001). Conclusions and Relevance: This study found that hospitals varied in neuroimaging practices among infants with concern for abuse. Apparent disparities in practice associated with insurance type suggest opportunities for quality, safety, and equity improvement.

Health Care Use of Cardiac Specialty Care in Children With Muscular Dystrophy in the United States.

Background Duchenne and Becker muscular dystrophy are progressive disorders associated with cardiac mortality. Guidelines recommend routine surveillance; we assess cardiac resource use and identify gaps in care delivery. Methods and Results Male patients, aged 1 to 18 years, with Duchenne and Becker muscular dystrophy between January 2013 and December 2017 were identified in the IBM MarketScan Research Database. The cohort was divided into <10 and 10 to 18 years of age. The primary outcome was rate of annual health care resource per person year. Resource use was assessed for place of service, cardiac testing, and medications. Adjusted incidence rate ratios (IRRs) were estimated using a Poisson regression model. Medication use was measured by proportion of days covered. There were 1386 patients with a median follow-up time of 3.0 years (interquartile range, 1.9-4.7 years). Patients in the 10 to 18 years group had only 0.40 (95% CI, 0.35-0.45) cardiology visits per person year and 0.66 (95% CI, 0.62-0.70) echocardiography/magnetic resonance imaging per person year. Older patients had higher rates of inpatient admissions (IRR, 1.46; 95% CI, 1.03-2.09), outpatient cardiology visits (IRR, 2.0; 95% CI, 1.66-2.40), cardiac imaging (IRR, 1.59; 95% CI, 1.40-1.80), and Holter monitoring (IRR, 3.33; 95% CI, 2.35-4.73). A proportion of days covered >80% for angiotensin-converting enzyme inhibitors/angiotensin receptor blockers was observed in 13.6% (419/3083) of total person years among patients in the 10 to 18 years group. Conclusions Children 10 to 18 years of age have higher rates of cardiac resource use compared with those <10 years of age. However, rates in both age groups fall short of guidelines. Opportunities exist to identify barriers to resource use and optimize cardiac care for patients with Duchenne and Becker muscular dystrophy.

The Design of a Data Management System for a Multicenter Palliative Care Cohort Study.

CONTEXT: Prospective cohort studies of individuals with serious illness and their family members, such as children receiving palliative care and their parents, pose challenges regarding data management. OBJECTIVE: To describe the design and lessons learned regarding the data management system for the Pediatric Palliative Care Research Network's Shared Data and Research (SHARE) project, a multicenter prospective cohort study of children receiving pediatric palliative care (PPC) and their parents, and to describe important attributes of this system, with specific considerations for the design of future studies. METHODS: The SHARE study consists of 643 PPC patients and up to two of their parents who enrolled from April 2017 to December 2020 at seven children's hospitals across the United States. Data regarding demographics, patient symptoms, goals of care, and other characteristics were collected directly from parents or patients at 6 timepoints over a 24-month follow-up period and stored electronically in a centralized location. Using medical record numbers, primary collected data was linked to administrative hospitalization data containing diagnostic and procedure codes and other data elements. Important attributes of the data infrastructure include linkage of primary and administrative data; centralized availability of multilingual questionnaires; electronic data collection and storage system; time-stamping of instrument completion; and a separate but connected study administrative database used to track enrollment. CONCLUSIONS: Investigators planning future multicenter prospective cohort studies can consider attributes of the data infrastructure we describe when designing their data management system.

Novel Risk Model to Predict Emergency Department Associated Mortality for Patients Supported With a Ventricular Assist Device: The Emergency Department-Ventricular Assist Device Risk Score.

Background The past decade has seen tremendous growth in patients with ambulatory ventricular assist devices. We sought to identify patients that present to the emergency department (ED) at the highest risk of death. Methods and Results This retrospective analysis of ED encounters from the Nationwide Emergency Department Sample includes 2010 to 2017. Using a random sampling of patient encounters, 80% were assigned to development and 20% to validation cohorts. A risk model was derived from independent predictors of mortality. Each patient encounter was assigned to 1 of 3 groups based on risk score. A total of 44 042 ED ventricular assist device patient encounters were included. The majority of patients were male (73.6%), <65 years old (60.1%), and 29% presented with bleeding, stroke, or device complication. Independent predictors of mortality during the ED visit or subsequent admission included age ≥65 years (odds ratio [OR], 1.8; 95% CI, 1.3-4.6), primary diagnoses (stroke [OR, 19.4; 95% CI, 13.1-28.8], device complication [OR, 10.1; 95% CI, 6.5-16.7], cardiac [OR, 4.0; 95% CI, 2.7-6.1], infection [OR, 5.8; 95% CI, 3.5-8.9]), and blood transfusion (OR, 2.6; 95% CI, 1.8-4.0), whereas history of hypertension was protective (OR, 0.69; 95% CI, 0.5-0.9). The risk score predicted mortality areas under the curve of 0.78 and 0.71 for development and validation. Encounters in the highest risk score strata had a 16-fold higher mortality compared with the lowest risk group (15.8% versus 1.0%). Conclusions We present a novel risk score and its validation for predicting mortality of patients with ED ventricular assist devices, a high-risk, and growing, population.

Impact of Age on Emergency Resource Utilization and Outcomes in Pediatric and Young Adult Patients Supported with a Ventricular Assist Device.

There are minimal data describing outcomes in ambulatory pediatric and young adult ventricular assist device (VAD)-supported patient populations. We performed a retrospective analysis of encounter-level data from 2006 to 2017 Nationwide Emergency Department Sample (NEDS) to compare emergency department (ED) resource utilization and outcomes for pediatric (≤18 years, n = 494) to young adult (19-29 years, n = 2,074) VAD-supported patient encounters. Pediatric encounters were more likely to have a history of congenital heart disease (11.3% vs. 4.8%). However, Pediatric encounters had lower admission/transfer rates (37.8% vs. 57.8%) and median charges ($3,334 (IQR $1,473-$19,818) vs. $13,673 ($3,331-$45,884)) (all p < 0.05). Multivariable logistic regression modeling revealed that age itself was not a predictor of admission, instead high acuity primary diagnoses and medical complexity were: (adjusted odds ratio; 95% confidence intervals): cardiac (3.0; 1.6-5.4), infection (3.4; 1.7-6.5), bleeding (3.9; 1.7-8.8), device complication (7.2; 2.7-18.9), and ≥1 chronic comorbidity (4.1; 2.5-6.7). In this largest study to date describing ED resource use and outcomes for pediatric and young adult VAD-supported patients, we found that, rather than age, high acuity presentations and comorbidities were primary drivers of clinical outcomes. Thus, reducing morbidity in this population should target comorbidities and early recognition of VAD-related complications.

Prevalent pharmacotherapy of US Fontan survivors: A study utilizing data from the MarketScan Commercial and Medicaid claims databases.

BACKGROUND: Survivors of Fontan palliation are at life-long risk of thrombosis, arrhythmia, and circulatory failure. To our knowledge, no studies have evaluated current United States pharmaceutical prescription practice in this population. METHODS: A retrospective observational study evaluating the prevalent use of prescription medications in children and adolescents with hypoplastic left heart syndrome or tricuspid atresia after Fontan completion (identified using ICD9/10 codes) was performed using data contained in the MarketScan Commercial and Medicaid databases for the years 2013 through 2018. Cardiac pharmaceuticals were divided by class. Anticoagulant agents other than platelet inhibitors, which are not uniformly a prescription medication, were also studied. Associations between increasing age and the likelihood of a filled prescription for each class of drug were evaluated. Annualized retail costs of pharmaceutical regimens were calculated. RESULTS: A cohort of 4,056 subjects (median age 12 years [interquartile range: 8-16], 61% male, 60% commercial insurance) was identified. Of the cohort, 50% received no prescription medications. Angiotensin converting enzyme inhibitors/angiotensin receptor blockers (ACEi/ARB) (38%), diuretics (15%), and mineralocorticoid receptor antagonists (8%) were prescribed with the highest frequency. Pulmonary vasodilators were received by 6% of subjects. Older age was associated with increased likelihood of filled prescriptions for anticoagulants (P = .008), antiarrhythmic agents, digoxin, ACEi/ARB, and beta blockers (each P < .0001), but also lower likelihood of filled prescriptions for pulmonary vasodilators, conventional diuretics (both P < .0001), and mineralocorticoid receptor antagonists (P = .02). CONCLUSIONS: Pharmaceuticals typically used to treat heart failure and pulmonary hypertension are the most commonly prescribed medications following Fontan palliation. While the likelihood of treatment with a particular class of medication is associated with the age of the patient, determining the optimal regimen for individual patients and the population at large is an important knowledge gap for future research.

PRagMatic Pediatric Trial of Balanced vs nOrmaL Saline FlUid in Sepsis: study protocol for the PRoMPT BOLUS randomized interventional trial.

BACKGROUND/AIMS: Despite evidence that preferential use of balanced/buffered fluids may improve outcomes compared with chloride-rich 0.9% saline, saline remains the most commonly used fluid for children with septic shock. We aim to determine if resuscitation with balanced/buffered fluids as part of usual care will improve outcomes, in part through reduced kidney injury and without an increase in adverse effects, compared to 0.9% saline for children with septic shock. METHODS: The Pragmatic Pediatric Trial of Balanced versus Normal Saline Fluid in Sepsis (PRoMPT BOLUS) study is an international, open-label pragmatic interventional trial being conducted at > 40 sites in the USA, Canada, and Australia/New Zealand starting on August 25, 2020, and continuing for 5 years. Children > 6 months to < 18 years treated for suspected septic shock with abnormal perfusion in an emergency department will be randomized to receive either balanced/buffered crystalloids (intervention) or 0.9% saline (control) for initial resuscitation and maintenance fluids for up to 48 h. Eligible patients are enrolled and randomized using serially numbered, opaque envelopes concurrent with clinical care. Given the life-threatening nature of septic shock and narrow therapeutic window to start fluid resuscitation, patients may be enrolled under "exception from informed consent" in the USA or "deferred consent" in Canada and Australia/New Zealand. Other than fluid type, all decisions about timing, volume, and rate of fluid administration remain at the discretion of the treating clinicians. For pragmatic reasons, clinicians will not be blinded to study fluid type. Anticipated enrollment is 8800 patients. The primary outcome will be major adverse kidney events within 30 days (MAKE30), a composite of death, renal replacement therapy, and persistent kidney dysfunction. Additional effectiveness, safety, and biologic outcomes will also be analyzed. DISCUSSION: PRoMPT BOLUS will provide high-quality evidence for the comparative effectiveness of buffered/balanced crystalloids versus 0.9% saline for the initial fluid management of children with suspected septic shock in emergency settings. TRIAL REGISTRATION: PRoMPT BOLUS was first registered at ClinicalTrials.gov ( NCT04102371 ) on September 25, 2019. Enrollment started on August 25, 2020.

The Effect of Epinephrine Dosing Intervals on Outcomes from Pediatric In-Hospital Cardiac Arrest.

Rationale: Animal studies of cardiac arrest suggest that shorter epinephrine dosing intervals than currently recommended (every 3-5 min) may be beneficial in select circumstances. Objectives: To evaluate the association between epinephrine dosing intervals and pediatric cardiac arrest outcomes. Methods: Single-center retrospective cohort study of children (<18 years of age) who received ⩾1 minute of cardiopulmonary resuscitation and ⩾2 doses of epinephrine for an index in-hospital cardiac arrest. Exposure was epinephrine dosing interval ⩽2 minutes (frequent epinephrine) versus >2 minutes. The primary outcome was survival to hospital discharge with a favorable neurobehavioral outcome (Pediatric Cerebral Performance Category score 1-2 or unchanged). Logistic regression evaluated the association between dosing interval and outcomes; additional analyses explored duration of cardiopulmonary resuscitation (CPR) as a mediator. In a subgroup, the effect of dosing interval on diastolic blood pressure was investigated. Measurements and Main Results: Between January 2011 and December 2018, 125 patients met inclusion/exclusion criteria; 33 (26%) received frequent epinephrine. Frequent epinephrine was associated with increased odds of survival with favorable neurobehavioral outcome (adjusted odds ratio, 2.56; 95% confidence interval, 1.07-6.14; P = 0.036), with 66% of the association mediated by CPR duration. Delta diastolic blood pressure was greater after the second dose of epinephrine among patients who received frequent epinephrine (median [interquartile range], 6.3 [4.1 to 16.9] vs. 0.13 [-2.3 to 1.9] mm Hg; P = 0.034). Conclusions: In patients who received at least two doses of epinephrine, dosing intervals ⩽2 minutes were associated with improved neurobehavioral outcomes compared with dosing intervals >2 minutes. Mediation analysis suggests that improved outcomes are largely due to frequent epinephrine shortening duration of CPR.

Effect of first-line biologic initiation on glucocorticoid exposure in children hospitalized with new-onset systemic juvenile idiopathic arthritis: emulation of a pragmatic trial using observational data.

BACKGROUND: Glucocorticoid exposure is a significant driver of morbidity in children with systemic juvenile idiopathic arthritis (sJIA). We determined the effect of early initiation of biologic therapy (IL-1 or IL-6 inhibition) on glucocorticoid exposure in hospitalized patients with new-onset sJIA. METHODS: We emulated a pragmatic sequence of trials ("pseudo-trials") of biologic initiation in children (≤ 18 years) hospitalized with new-onset sJIA utilizing retrospective data from an administrative database from 52 tertiary care children's hospitals from 2008 to 2019. Eligibility window, treatment assignment and start of follow-up between biologic and non-biologic study arms were aligned for each pseudo-trial. Patients in the source population could meet eligibility criteria at several timepoints. Mixed-effects logistic regression was used to determine the effect of biologic initiation on in-hospital glucocorticoid exposure. RESULTS: Four hundred sixty-eight children met eligibility criteria, of which 19% received biologic therapy without preceding or concomitant initiation of immunomodulatory medications. This proportion significantly increased over time during the study period (p <  0.01). 1451 trial subjects were included across 4 pseudo-trials with 71 assigned to the biologic arm and 1380 assigned to the non-biologic arm. After adjustment, there was a trend toward decreased odds of glucocorticoid initiation in the biologic arm compared to the non-biologic arm (OR 0.39, 95% CI [0.13, 1.15]). CONCLUSION: Biologic initiation in children hospitalized with new-onset sJIA significantly increased over time and may be associated with reduced glucocorticoid exposure. The increasing use of first-line biologic therapy may lead to clinically relevant reductions in treatment-related adverse effects of glucocorticoid-reliant therapeutic approaches.

Mental health disorders and emergency resource use and outcomes in ventricular assist device supported patients.

There are limited data describing the prevalence of mental health disorders (MHDOs) in patients with ventricular assist devices (VADs), or associations between MHDOs and resource use or outcomes. We used the Nationwide Emergency Department Sample administrative database to analyze 44,041 ED encounters for VAD-supported adults from 2010 to 2017, to assess the relationship between MHDOs and outcomes in this population. MHDO diagnoses were present for 23% of encounters, and were associated with higher charges and rates of admission, but lower mortality.

Venous Flow Variation Predicts Preoperative Pulmonary Venous Obstruction in Children with Total Anomalous Pulmonary Venous Connection.

BACKGROUND: Identifying preoperative pulmonary venous obstruction in total anomalous pulmonary venous connection is important to guide treatment planning and risk prognostication. No standardized echocardiographic definition of obstruction exists in the literature. Definitions based on absolute velocities are affected by technical limitations and variations in pulmonary venous return. The authors developed a metric to quantify pulmonary venous blood flow variation: pulmonary venous variability index (PVVI). The aim of this study was to demonstrate its accuracy in defining obstruction. METHODS: All patients with total anomalous pulmonary venous connection at a single institution were identified. Echocardiograms were reviewed, and maximum (Vmax), mean (Vmean), and minimum (Vmin) velocities along the pulmonary venous pathway were measured. PVVI was defined as (Vmax - Vmin)/Vmean. These metrics were compared with pressures measured on cardiac catheterization. Echocardiographic measures were then compared between patients with and without clinical preoperative obstruction (defined as a need for preoperative intubation, catheter-based intervention, or surgery within 1 day of diagnosis), as well as pulmonary edema by chest radiography and markers of lactic acidosis. One hundred thirty-seven patients were included, with 22 having catheterization pressure recordings. RESULTS: Vmax and Vmean were not different between patients with catheter gradients ≥ 4 and < 4 mm Hg, while PVVI was significantly lower and Vmin higher in those with gradients ≥ 4 mm Hg. The composite outcome of preoperative obstruction occurred in 51 patients (37%). Absolute velocities were not different between patients with and without clinical obstruction, while PVVI was significantly lower in patients with obstruction. All metrics except Vmax were associated with pulmonary edema; none were associated with blood gas metrics. CONCLUSIONS: The authors developed a novel quantitative metric of pulmonary venous flow, which was superior to traditional echocardiographic metrics. Decreased PVVI was highly associated with elevated gradients measured by catheterization and clinical preoperative obstruction. These results should aid risk assessment and diagnosis preoperatively in patients with total anomalous pulmonary venous connection.

An Increasing Burden of Disease: Emergency Department Visits Among Patients With Ventricular Assist Devices From 2010 to 2017.

Background With a growing population of patients supported by ventricular assist devices (VADs) and the improvement in survival of this patient population, understanding the healthcare system burden is critical to improving outcomes. Thus, we sought to examine national estimates of VAD-related emergency department (ED) visits and characterize their demographic, clinical, and outcomes profile. Additionally, we tested the hypotheses that resource use increased and mortality improved over time. Methods and Results This retrospective database analysis uses encounter-level data from the 2010 to 2017 Nationwide Emergency Department Sample. The primary outcome was mortality. From 2010 to 2017, >880 million ED visits were evaluated, with 44 042 VAD-related ED visits identified. The annual mean visits were 5505 (SD 4258), but increased 16-fold from 2010 to 2017 (824 versus 13 155). VAD-related ED visits frequently resulted in admission (72%) and/or death (3.0%). Median inflation-adjusted charges were $25 679 (interquartile range, $7450, $63 119) per encounter. The most common primary diagnoses were cardiac (22%), and almost 30% of encounters were because of bleeding, stroke, or device complications. From 2010 to 2017, admission and mortality decreased from 82% to 71% and 3.4% to 2.4%, respectively (P for trends <0.001, both). Conclusions We present the first study using national-level data to characterize the growing ED resource use and financial burden of patients supported by VAD. During the past decade, admission and mortality rates decreased but remain substantial; in 2017 ≈1 in every 40 VAD ED encounters resulted in death, making it critical that clinical decision-making be optimized for patients with VAD to maximize good outcomes.

Immunomodulatory Medication Use in Newly Diagnosed Youth With Systemic Lupus Erythematosus.

OBJECTIVE: To examine glucocorticoid-sparing immunomodulatory medication use in youth with systemic lupus erythematosus (SLE) during their first year of care. METHODS: We conducted a retrospective cohort study using administrative claims for 2000 to 2013 from Clinformatics DataMart for youth ages 10-24 years with an incident diagnosis of SLE (≥3 International Classification of Diseases, Ninth Revision codes for SLE [710.0], each >30 days apart). We determined the proportion of subjects filling a prescription for immunomodulatory medications within 12 months of the first SLE code (index date). We used multivariable regression to examine associations between demographic/disease factors and time to prescription fill in the first year, and also between prescription fill at any time after the index date. RESULTS: We identified 532 youth with an incident SLE diagnosis, of which 413 (78%) had a glucocorticoid-sparing immunomodulatory prescription fill in the first year. Prescriptions for hydroxychloroquine and immunosuppressants were filled in the first year by 366 youth (69%) and by 182 (34%), respectively. Those with adult-onset (versus childhood-onset) disease were less likely to fill an immunomodulatory medication by 12 months. No other statistically significant associations were found, although there was increasing likelihood of immunomodulatory medication fills with each subsequent calendar year. CONCLUSION: Among youth with newly diagnosed SLE, hydroxychloroquine use is prevalent although not universal, and prescription immunosuppressant use is notably low during the first year of care. Further research is needed to identify factors contributing to suboptimal immunomodulatory medication use during the first year of care.

Variation in Treatment of Children Hospitalized With New-Onset Systemic Juvenile Idiopathic Arthritis in the US.

OBJECTIVE: Increasing evidence supports the conclusion that early initiation of biologics may dramatically improve disease course and reduce glucocorticoid exposure for children with systemic juvenile idiopathic arthritis (JIA). The present study was undertaken to characterize variation in the use of first-line biologic and glucocorticoid therapy and to identify drivers of variation in children hospitalized with new-onset systemic JIA. METHODS: We conducted a retrospective cohort study of children hospitalized with new-onset systemic JIA from 2008 to 2019 utilizing a comparative pediatric database from 52 tertiary care children's hospitals. Subjects and treatment receipt were identified using International Classification of Diseases, Ninth Revision (ICD-9) and ICD-10 discharge diagnosis codes, pharmacy billing data, and clinical transaction classification codes. Mixed-effects logistic regression was used to identify patient- and hospital-level factors associated with receipt of glucocorticoids and biologics. RESULTS: In total, 534 children with new-onset systemic JIA hospitalized during the study period met inclusion criteria. Twenty-nine percent received biologics, and 58% received glucocorticoids. Biologic use increased over time (P < 0.001), methotrexate use decreased (P < 0.01), and glucocorticoid use remained unchanged. Biologics and glucocorticoid use varied significantly between hospitals. High annual hospital volume, intensive care unit stay, and later discharge year were significantly associated with biologic exposure. Medium-high and high annual hospital volume were significantly associated with less glucocorticoid exposure. CONCLUSION: Despite increasing evidence demonstrating improved outcomes with first-line treatment with biologics, we found significant treatment variation across hospitals with many children not receiving biologics and a persistent high rate of glucocorticoid exposure. These results underscore the need for comparative efficacy studies and improved treatment standardization.

Mid-gestational fetal placental blood flow is diminished in the fetus with congenital heart disease.

OBJECTIVE: Data suggest fetuses with congenital heart disease (CHD) have placental abnormalities. Their abnormal placental vasculature may affect fetal placental blood flow, which has not previously been explored. METHOD: We performed a retrospective cross-sectional study comparing umbilical venous volume flow (UVVF) of single ventricle, D-transposition of the great arteries, and tetralogy of Fallot fetuses with fetuses without CHD. UVVF and combined cardiac output (CCO) were calculated from fetal echocardiography and compared using t tests, χ2 and Fisher's exact tests. RESULTS: Mean gestational age and fetal weight were greater in CHD fetuses (26.5 weeks, 1119.4 g; n = 81, P < .001) compared to controls (23.1 weeks, 675 g; n = 170, P < .001). UVVF/fetal weight was nevertheless decreased among cases (99.8 vs 115.3 mL/min/kg, P < .001). Subgroup analysis of 20- to 25-week fetuses demonstrated no significant differences in case and control baseline characteristics. In CHD fetuses (n = 31) compared to controls (n = 144), absolute UVVF (50.8 vs 62.1 mL/min, P = .006), and UVVF/fetal weight (98.8 vs 118.5 mL/min/kg, P < .001) were decreased. Findings were similar in single ventricle (n = 24) and hypoplastic left heart syndrome (n = 14). CONCLUSION: Mid-gestational placental blood flow in CHD fetuses is decreased compared to controls. Further study is needed to explore the relationship between UVVF and placental pathology, and impact on outcomes.