Pediatric Digit Replantation: A Nationwide Analysis of Failure Rate, Complications, and Potential Factors Affecting Failure.

Purpose: Function and cosmesis may be improved by replantation following digital amputation in pediatric patients. However, accurate failure and complication rate estimates may be limited as most pertinent studies reflect single center/surgeon experience and therefore are limited by small sample sizes. The primary aim of this study was to assess the rate of failure (amputation) following pediatric digital replantation. Secondary aims include evaluating the rate of complications and associated resource utilization (intensive care unit stays, readmission rate, and hospital length of stay). Methods: Digital replantation patients were identified from 47 pediatric hospitals using the 2004 to 2020 Pediatric Health Information System nationwide database. Using applicable International Classification of Disease 9/10 and Current Procedural Terminology codes, we identified complications after replantation, including revision amputation, infection, surgical complications, medical complications, admission to intensive care unit (ICU), and length of stay. Results: Of the 348 patients who underwent replantation the mean age was 8.3 ± 5.1 years, and 27% were female. Mean hospital length of stay was 5.8 ± 4.7 (range, 1-28) days. Of the 53% of patients who required ICU admission, the mean ICU length of stay was 2.4 ± 3.3 days. Failure/amputation after replantation occurred in 71 (20.4%) patients, at a mean of 9.7 ± 27.2 days postoperatively. Surgical complications occurred in 58 (17%) patients, 30-day hospital readmissions occurred in 5.7% of patients, and 90-day readmissions occurred in 6.3% patients. Conclusion: The estimated rate of failure following pediatric digit replantation was 20%. Our data on failure and complication rates and associated resource utilization may be useful in counseling pediatric replantation patients and their families and provide an update on prior literature. Level of Evidence: IV, Prognosis.

Time to Transplant in X-Linked Adrenoleukodystrophy.

OBJECTIVES: Cerebral X-linked adrenoleukodystrophy (cALD) is an inflammatory demyelination of the brain that can lead to death unless treated by hematopoietic stem cell transplantation. Survival and improved outcomes for cerebral adrenoleukodystrophy are associated with hematopoietic stem cell transplantation at earliest evidence of disease on magnetic resonance imaging (MRI). Our goal was to determine average duration between diagnosis of cALD and hematopoietic stem cell transplantation. METHODS: This was a retrospective review of data of patients aged 18 years or younger, using a nationwide administrative health care database (Pediatric Health Information System), with an International Classification of Diseases, Tenth Revision (ICD-10) diagnosis of adrenoleukodystrophy. Time range was October 1, 2015, through June 30, 2021. We determined time to hematopoietic stem cell transplantation by duration between index brain MRI and a code for hematopoietic stem cell transplantation. RESULTS: We identified 27 patients with cerebral adrenoleukodystrophy. Total charges for the cohort was $53 million. Time to transplant averaged 97 days. For Hispanic patients, time to transplant was 117 days, compared with 80 days for White, non-Hispanic patients. Comparison of different hospitals showed significant variability in time to hematopoietic stem cell transplantation. DISCUSSION: We found that time to hematopoietic stem cell transplantation was >3 months for patients with cerebral adrenoleukodystrophy in the hospitals we evaluated. We noted differences in average time by race/ethnicity and by hospital. Our findings suggest opportunity to reduce time to transplant in cerebral adrenoleukodystrophy.

National U.S. Patient and Transplant Data for Krabbe Disease.

Krabbe disease (KD) is a leukodystrophy caused by mutations in the galactosylceramidase gene. Presymptomatic hematopoietic stem cell transplantation (HSCT) is associated with improved outcomes, but most data are from single-center studies. Our objective was to characterize national patterns of HSCT for KD including whether there were disparities in HSCT utilization and outcomes. We conducted a retrospective study of KD patients ≤ age 18 years from November 1, 2015, through December 31, 2019, using the U.S. Children's Hospital Association's Pediatric Health Information System database. We evaluated outcomes for HSCT, intensive care unit days, and mortality, comparing age, sex, race/ethnicity, rural/urban location, and median household income. We identified 91 KD patients. HSCT, performed in 32% of patients, was associated with reduced mortality, 31 vs. 68% without HSCT (p < 0.003). Trends included the fact that more males than females had HSCT (39 vs. 23%); more Asian and White patients had HSCT compared to Black or Hispanic patients (75, 33, 25, and 17%, respectively); and patients from households with the lowest-income quartile (< $25,000) had more HSCT compared to higher-income quartiles (44 vs. 33, 30, and 0%). Overall, receiving HSCT was associated with reduced mortality. We noted trends in patient groups who received HSCT. Our findings suggest that disparities in receiving HSCT could affect outcomes for KD patients.

Changes in Provider Prescribing Behavior for Infants with Single Ventricle Physiology After Evidence-Based Publications.

BACKGROUND: The impact of published evidence on clinical practice has been understudied in pediatric cardiology. OBJECTIVE: We sought to assess changes in prescribing behavior for angiotensin-converting enzyme inhibitor (ACEI) and digoxin at discharge after initial palliation of infants with single ventricle (SV) physiology following the publication of two large studies: The Pediatric Heart Network Infant Single Ventricle (PHN-ISV) trial showing no benefit with routine ACEI use and the National Pediatric Cardiology Quality Improvement Collaborative (NPC-QIC) analysis showing an association between digoxin and survival. METHODS: ICD-9-10 codes identified SV infants from the Pediatric Health Information System (1/2004 to 1/2018) and charge codes identified medications at discharge. Generalized estimating equations implementing segmented logistic regressions modeled medication use, before and after (with a 3-month washout period) the relevant publication (ACEI 7/1/2010; digoxin 4/1/2016). A subgroup analysis was performed for hypoplastic left heart syndrome (HLHS). RESULTS: ACEI use (37 centers, n = 4700) at discharge did not change over time during the pre-publication period. After publication of the PHN-ISV trial, ACEI use decreased (OR: 0.61, CI 0.44-0.84, p = 0.003). Digoxin use (43 centers, n = 4778) decreased by 1% monthly before publication. After the NPC-QIC publication, digoxin use increased (OR: 2.07, CI 1.05-4.08, p = 0.04) with an ongoing increase of 9% per month. Results were similar for the HLHS subgroup. CONCLUSIONS: Prescribing behavior changed congruently after the publication of evidence-based studies, with decreased ACEI use and increased digoxin use at discharge following initial palliation of SV infants. Our findings suggest scientific findings were rapidly implemented into clinical practice.

Celiac Disease in Children: An Association With Drug-Resistant Epilepsy.

BACKGROUND: Neurological manifestations are commonly reported in patients with celiac disease (CD). We aimed to characterize epilepsy features in a pediatric population with CD and the effect of a gluten-free diet (GFD) on seizure burden. METHODS: A retrospective chart review was performed on pediatric patients treated at the University of Utah and Primary Children's Hospital in Salt Lake City, Utah, with both epilepsy and CD and compared with a control group with epilepsy only. RESULTS: We identified 56 patients with epilepsy and biopsy-confirmed CD (n = 36, 64%) or elevated tissue transglutaminase antibodies (tTG-Ab) without biopsy-confirmed CD (n = 20, 36%). Age- and gender-matched controls were selected from patients with epilepsy only (n = 168). Patients with biopsy-proven CD or positive tTG-Ab had high percentage of drug-resistant epilepsy (DRE) compared with the control group (P < 0.05). Age at seizure onset preceded the diagnosis of CD on average by 5.9 years for patients with DRE (P < 0.01) compared with 2.2 years for those with drug-responsive epilepsy. Adhering to a GFD reduced seizure frequency or resulted in weaning dosage or weaning off of one or more antiseizure medications in a majority of patients with DRE. CONCLUSIONS: DRE was more prevalent in pediatric patients with biopsy-confirmed CD and positive tTG-Ab compared with the control group (which included childhood epilepsy syndromes), but comparable with the prevalence of DRE in the general population. Adherence to a GFD in combination with antiseizure medications appears to reduce seizure burden for those with CD and DRE.

Early iron supplementation and iron sufficiency at one month of age in NICU patients at-risk for iron deficiency.

In order to reduce iron deficiency in neonates at-risk for iron deficiency, we implemented a guideline to increase the consistency of early iron supplementation in infants of diabetic mothers, small for gestational age neonates and very low birthweight premature neonates. Three years following implementation we performed a retrospective analysis in order to assess adherence to the guideline and to compare timing of early iron supplementation and reticulocyte-hemoglobin (RET-He) values at one month of life in at-risk infants. Adherence with early iron supplementation guidelines was 73.4% (399/543) with 51% (275/543) having RET-He values obtained at one month. Despite good adherence, 16% (44/275) had RET-He <25 pg (5th percentile for gestational age). No infants receiving red blood cell transfusion (0/20) had RET-He <25 pg vs. 26.1% (40/153) of those treated with darbepoetin (p < 0.001). There was no evidence of increased feeding intolerance (episodes of emesis/day) with early iron supplementation.

Influence of Geographic Access on Surgical Center Readmissions After Index Congenital Heart Surgery.

OBJECTIVE: To assess the impact of geographic access to surgical center on readmission risk and burden in children after congenital heart surgery. STUDY DESIGN: Children <6 years old at discharge after congenital heart surgery (Risk Adjustment for Congenital Heart Surgery-1 score 2-6) were identified using Pediatric Health Information System data (46 hospitals, 2004-2015). Residential distance from the surgery center, calculated using ZIP code centroids, was categorized as <15, 15-29, 30-59, 60-119, and ≥120 miles. Rurality was defined using rural-urban commuting area codes. Geographic risk factors for unplanned readmissions to the surgical center and associated burden (total hospital length of stay [LOS], costs, and complications) were analyzed using multivariable regression. RESULTS: Among 59 696 eligible children, 19 355 (32%) had ≥1 unplanned readmission. The median LOS was 9 days (IQR 22) across the entire cohort. In those readmitted, median total costs were $31 559 (IQR $90 176). Distance from the center was inversely related but rurality was positively related to readmission risk. Among those readmitted, increased distance was associated with longer LOS, more complications, and greater costs. Compared with urban patients, highly rural patients were more likely to have an unplanned readmission but had fewer average readmission days. CONCLUSIONS: Geographic measures of access differentially affect readmission to the surgery center. Increased distance from the center was associated with fewer unplanned readmissions but more complications. Among those readmitted, the most isolated patients had the greatest readmission costs. Understanding the contribution of geographic access will aid in developing strategies to improve care delivery to this population.

Dronabinol Prescribing and Exposure Among Children and Young Adults Diagnosed with Cancer.

Purpose: The therapeutic utility of Cannabis in cancer is a topic of intense interest. Dronabinol is synthetic Δ9-tetrahydrocannabinol (THC), the primary psychoactive component of Cannabis sativa, and is approved for treating refractory chemotherapy-induced nausea and vomiting. Little is known about dronabinol prescribing in children and young adults, and no published concentration data are available. This study evaluated national level dronabinol use and assessed concentrations of THC and its primary metabolites in patients with cancer <27 years of age prescribed dronabinol. Methods: Observational review of records from the Pediatric Health Information System (PHIS) and a regional network of hospitals in the Intermountain West, including a tertiary care children's hospital, Primary Children's Hospital (PCH), for inpatients <27 years of age prescribed dronabinol. Prospective blood samples were collected from children with cancer at PCH. Results: Across PHIS institutions, overall dronabinol prescribing aligned with the pharmacy records for those with cancer (p < 0.0001), and of these, 10.4% received dronabinol as inpatients. Blood collected within 72 hours of dronabinol administration was available from 10 children with a median age of 12.5 (range 6-17) years. Quantifiable concentrations were found in 4 (13%), 6 (20%), and 1 (3%) samples assayed for THC, 11-nor-9-carboxy-Δ9-tetrahydrocannabinol (COOH-THC), and 11-hydroxy-Δ9-tetrahydrocannabinol (OH-THC), respectively. THC concentrations ranged between 0.100 and 0.128 ng/mL and were not associated with dose. Conclusion: Dronabinol prescribing appears exclusive to patients diagnosed with cancer, and its use has increased steadily in the past decade. In a small sample of children administered dronabinol, THC and metabolite concentrations were consistently low or undetectable.

Primary Care Autism Screening and Later Autism Diagnosis.

OBJECTIVES: To describe the proportion of children screened by the Modified Checklist for Autism in Toddlers (M-CHAT), identify characteristics associated with screen completion, and examine associations between autism spectrum disorder (ASD) screening and later ASD diagnosis. METHODS: We examined data from children attending 18- and 24-month visits between 2013 and 2016 from 20 clinics within a health care system for evidence of screening with the M-CHAT and subsequent coding of ASD diagnosis at age >4.75 years. We interviewed providers for information about usual methods of M-CHAT scoring and ASD referral. RESULTS: Of 36 233 toddlers, 73% were screened and 1.4% were later diagnosed with ASD. Hispanic children were less likely to be screened (adjusted prevalence ratio [APR]: 0.95, 95% confidence interval [CI]: 0.92-0.98), and family physicians were less likely to screen (APR: 0.12, 95% CI: 0.09-0.15). Compared with unscreened children, screen-positive children were more likely to be diagnosed with ASD (APR: 10.3, 95% CI: 7.6-14.1) and were diagnosed younger (38.5 vs 48.5 months, P < .001). The M-CHAT's sensitivity for ASD diagnosis was 33.1%, and the positive predictive value was 17.8%. Providers routinely omitted the M-CHAT follow-up interview and had uneven referral patterns. CONCLUSIONS: A majority of children were screened for ASD, but disparities exist among those screened. Benefits for screen-positive children are improved detection and younger age of diagnosis. Performance of the M-CHAT can be improved in real-world health care settings by administering screens with fidelity and facilitating timely ASD evaluations for screen-positive children. Providers should continue to monitor for signs of ASD in screen-negative children.

Changing trends in IVIG use in pediatric patients: A retrospective review of practices in a network of major USA pediatric hospitals.

The use of immunoglobulins is gradually increasing. Intravenous immunoglobulins (IVIG) are used as replacement therapy for primary and secondary immune deficiencies, and as an anti-inflammatory and immunomodulatory medication for the treatment of neurologic, dermatologic, and rheumatologic diseases. The objective of this study was to analyze trends in the IVIG use in pediatric patients hospitalized to 47 US-based children's hospitals from 2007 to 2014. IVIG was used for the treatment of >2300 primary diagnoses in 53,648 unique patients. The number of IVIG admissions increased by 30.2% during the study period, while the mean rate of IVIG admissions/100,000 admissions increased only 5.8%. Most patients receiving IVIG were children and adolescents. IVIG was frequently used off-label or for the treatment of FDA-approved indications in children under two years of age and BMT patients <20 years of age. Primary immune deficiencies represented only 1.2% of all IVIG admissions. Pediatric patients with mucocutaneous lymph node syndrome (Kawasaki disease, KD) and idiopathic thrombocytopenic purpura (ITP) were two primary consumers of the IVIG. Another top-ranked indications were acute infectious polyneuritis (Guillain-Barré syndrome, GBS) and prophylaxis of infections in patients receiving antineoplastic chemotherapy. IVIG usage is a dynamic process guided by emerging evidence and FDA approval for new indications. IVIG was mostly prescribed for treatment of diseases with pathologic immune responses to foreign of self-antigens. These indications usually, require higher amounts of IVIG per admission. More studies are needed to understand whether IVIG treatments of off-label indications are effective and cost-efficient.

Current Epidemiology of Vocal Cord Dysfunction After Congenital Heart Surgery in Young Infants.

OBJECTIVES: Surgery of the aortic arch poses risk of recurrent laryngeal nerve injury due to the anatomic proximity and can manifest as vocal cord dysfunction after surgery. We assessed risk factors for vocal cord dysfunction and calculated surgical procedure associated rates in young infants after congenital heart surgery. DESIGN: Cross section analysis. SETTING: Forty-four children's hospitals reporting administrative data to Pediatric Health Information System. PARTICIPANTS: Cardiac surgical patients less than or equal to 90 days old and discharged between January 2004 and June 2014. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Overall, 2,319 of 46,567 subjects (5%) had vocal cord dysfunction, increasing from 4% to 7% over the study period. Of those with vocal cord dysfunction, 75% had unilateral partial paralysis. Vocal cord dysfunction was significantly more common in newborn infants (74%), those with aortic arch procedures (77%) and with greater surgical complexity. Rates of vocal cord dysfunction ranged from 0.7% to 22.4% across surgical procedure groups. Vocal cord dysfunction was significantly associated with greater use of: prolonged mechanical ventilation (53% vs 40%), diaphragmatic plication (3% vs 1%), feeding tube use (32% vs 8%), surgical airways (4% vs 2%), and prolonged length of stay (44 vs 21 d). Vocal cord dysfunction testing increased significantly over the study (6-14 %), and vocal cord dysfunction diagnosis increased almost two-fold (odds ratio, 1.9; 95% CI, 1.7-2.1) comparing the last to first study quarters with the increase in vocal cord dysfunction diagnosis occurring predominately in surgeries to the aortic arch supported by cardiopulmonary bypass. However, aortic procedures without cardiopulmonary bypass and nonaortic arch procedures were common surgeries accounting for 27% and 23% of vocal cord dysfunction cases despite low overall vocal cord dysfunction rates (3.7% and 2.6%). CONCLUSIONS: Vocal cord dysfunction complicated all cardiac surgical procedures among infants including those without aortic arch involvement. Increased efforts to determine appropriate indications for prevention, screening and treatment of vocal cord dysfunction among young infants after congenital heart surgery are needed.

Predictors of extracorporeal membrane oxygenation support after surgery for adult congenital heart disease in children's hospitals.

OBJECTIVE: Adult congenital heart disease (ACHD) patients who undergo cardiac surgery are at risk for poor outcomes, including extracorporeal membrane oxygenation support (ECMO) and death. Prior studies have demonstrated risk factors for mortality, but have not fully examined risk factors for ECMO or death without ECMO (DWE). We sought to identify risk factors for ECMO and DWE in adults undergoing congenital heart surgery in tertiary care children's hospitals. DESIGN: All adults (≥18 years) undergoing congenital heart surgery in the Pediatric Health Information System (PHIS) database between 2003 and 2014 were included. Patients were classified into three groups: ECMO-free survival, requiring ECMO, and DWE. Univariate analyses were performed, and multinomial logistic regression models were constructed examining ECMO and DWE as independent outcomes. SETTING: Tertiary care children's hospitals. RESULTS: A total of 4665 adult patients underwent ACHD surgery in 39 children's hospitals with 51 (1.1%) patients requiring ECMO and 64 (1.4%) patients experiencing DWE. Of the 51 ECMO patients, 34 (67%) died. Increasing patient age, surgical complexity, diagnosis of single ventricle heart disease, preoperative hospitalization, and the presence of noncardiac complex chronic conditions (CCC) were risk factors for both outcomes. Additionally, low and medium hospital ACHD surgical volume was associated with an increased risk of DWE in comparison with ECMO. CONCLUSIONS: There are overlapping but separate risk factors for ECMO support and DWE among adults undergoing congenital heart surgery in pediatric hospitals.

Mortality and Resource Use Following Cardiac Interventions in Children with Trisomy 13 and Trisomy 18 and Congenital Heart Disease.

We sought to evaluate the mortality, risk factors for mortality, and resource utilization following cardiac interventions in trisomy 13 (T13) and 18 (T18) children. All T13 and T18 children who underwent a cardiac intervention from January 1999 to March 2015 were identified from the Pediatric Health Information System database. Data collected included demographics, type of congenital heart disease (CHD), cardiac interventions, comorbidities, length of stay (LOS), hospital charges, and deaths (within 30 days). Logistic regression analysis was used to determine factors associated with mortality. There were 49 (47% females) T13 and 140 (67% females) T18 subjects. The two cohorts were similar in distribution for race, geographic region, insurance type, and median household income. The most common CHD in both groups was a shunt lesion followed by conotruncal defects. Compared to T18, the T13 cohort had higher mortality (29% vs. 12%), tracheostomies (12% vs. 4%), gastrostomies (18% vs. 6%), and overall resource use (P < 0.05 for all). White race (OR 0.23, 95% CI 0.06-0.81) in T13 and older age (in weeks) at surgery in T18 (OR 0.75, 95% CI 0.64-0.86) were associated with lower mortality. A select group of T13 and T18 CHD patients can undergo successful cardiac interventions, albeit with a higher mortality and resource use. T13 patients have higher mortality and resource use compared to T18. In T13 and T18 patients, interventions for CHD may be an acceptable and ethical option following a careful individualized selection and counseling by a team of experts.

The Impact of Pediatric Palliative Care Involvement in the Care of Critically Ill Patients without Complex Chronic Conditions.

Background: The impact of pediatric palliative care (PPC) is well established for children with chronic complex diseases. However, PPC likely also benefits previously healthy children with acute life-threatening conditions. Objective: To determine the incidence and impact of PPC for previously healthy patients who died in a pediatric hospital. Design: Retrospective chart review of all pediatric deaths over four years. Setting/Subjects: Patients were 0 to 25 years old, died during an inpatient stay at an academic pediatric hospital ≥48 hours after admission, and had no complex chronic conditions (CCCs) before admission. Measurements: One hundred sixty-seven patients met the eligibility criteria. Most died in intensive care settings (n = 149, 89%), and few (n = 34, 20%) received PPC consultations or services. Results: Patients who received PPC services were more likely to receive a multidisciplinary care conference than did patients without PPC support (70.5% vs. 39.9%; p = 0.001), which also occurred earlier for patients who received PPC services (seven days vs. two days before death; p = 0.04). Most patients had documented end-of-life planning in their medical records; however, this occurred earlier for patients who received PPC consultation (9.5 days before death) than for those who did not (two days before death; p < 0.0001). Patients receiving PPC support (67.7%) were also more likely to have a do-not-resuscitate/intubate order before death than those who did not (39.9%; p = 0.004). Conclusions: Pediatric patients without known CCCs who subsequently die as inpatients benefit from PPC in terms of goals of care discussions and documentation of end-of-life care preferences.

Scope and Burden of Non-Standard of Care Hematopoietic Stem Cell Transplantation in Pediatric Leukodystrophy Patients.

Inherited leukodystrophies are a group of diseases affecting central nervous system myelin that lead to death or significant health problems. Although for most leukodystrophies there are no curative treatments, for a handful of diseases hematopoietic stem cell transplantation (HSCT; bone marrow transplant) can stop disease progression, and if initiated in a timely fashion, prevent many or all neurologic and other systems involvement. However, HSCT is a complex procedure with significant morbidity and mortality risks. The study goal was to determine whether HSCT was being more widely used outside of those leukodystrophies for which HSCT is typically employed. The authors conducted a 2-year retrospective review of HSCT performed across the United States in 51 children's hospitals that are part of the Pediatric Health Information System. The authors screened for 10th revision of the International Statistical Classification of Diseases and Related Health Problems (ICD-10) codes for leukodystrophies in which HSCT is "nonstandard," including sphingolipidoses, Fabry disease, Gaucher disease, and Niemann-Pick disease, and excluded patients who had ICD-10 codes for leukodystrophies that are HSCT candidates, specifically X-linked adrenoleukodystrophy, metachromatic leukodystrophy, Krabbe disease, and Hurler disease. The authors identified 91 patients (from a total cohort of 937) with one of the nonstandard leukodystrophies who had HSCT. HSCT was performed at 20 of the hospitals, with the majority performed at only 6 hospitals. Average costs ($786 846) per patient were more than 6 times higher than patients who did not have HSCT. The data show that an unexpectedly large number of leukodystrophy patients are receiving transplants for conditions in which HSCT is not typically used, and which are associated with high medical costs.

Racial variations in extracorporeal membrane oxygenation use following congenital heart surgery.

OBJECTIVES: Previous studies demonstrate racial and ethnic disparities among children undergoing congenital heart surgery. Extracorporeal membrane oxygenation (ECMO) is used to support critically ill children after congenital heart surgery and improve survival. Thus, racial or ethnic variations in postoperative ECMO use following congenital heart surgery may be associated with racial/ethnic disparities in hospital survival. METHODS: All children in the Pediatric Health Information Systems dataset undergoing congenital heart surgery from 2004 to 2015 were examined. Multivariable, multinomial regression models examining hospital survival without ECMO use, survival after ECMO, death after ECMO, and death without ECMO support were constructed. RESULTS: Of 130,860 congenital cardiac surgery patients, 95.4% survived to hospital discharge without requiring ECMO support, whereas 1.3% survived after ECMO support, 1.3% died after ECMO support, and 1.9% died without receiving ECMO support. After adjustment for other covariates, black patients (odds ratio, 1.22; 95% confidence interval [CI], 1.05-1.42) and patients of other race (odds ratio, 1.36; 95% CI, 1.17-1.58) were at increased odds of mortality compared with white patients. In multivariable multinomial models, black patients had increased risk of death without ECMO support (relative risk, 1.31; 95% CI, 1.11-1.56). Patients of other race (relative risk, 1.37; 95% CI, 1.10-1.69) and governmental insurance (relative risk, 1.24; 95% CI, 1.12-1.37) were also at increased risk of death without ECMO. CONCLUSIONS: Black children and children of other race are at increased odds of mortality after congenital heart surgery. These disparities can be traced to variations in ECMO utilization across racial/ethnic groups.

Admission to dedicated pediatric cardiac intensive care units is associated with decreased resource use in neonatal cardiac surgery.

OBJECTIVE: Neonates undergoing congenital heart surgery require highly specialized, resource-intensive care. Location of care and degree of specialization can vary between and within institutions. Using a multi-institutional cohort, we sought to determine whether location of admission is associated with an increase in health care costs, resource use and mortality. METHODS: We retrospectively analyzed admission for neonates (<30 days) undergoing congenital heart surgery between 2004 and 2013 by using the Pediatric Health Information Systems database (44 children's hospitals). Multivariate generalized estimating equations adjusted for center- and patient-specific risk factors and stratified by age at admission were performed to examine the association of admission intensive care unit (ICU) with total hospital costs, mortality, and length of stay. RESULTS: Of 19,984 neonates (60% male) identified, 39% were initially admitted to a cardiac ICU (CICU), 48% to a neonatal ICU (NICU), and 13% to a pediatric ICU. In adjusted models, admission to a CICU versus NICU was associated with a $20,440 reduction in total hospital cost for infants aged 2 to 7 days at admission (P = .007) and a $23,700 reduction in total cost for infants aged 8 to 14 days at admission (P = .01). Initial admission to a CICU or pediatric ICU versus NICU at <15 days of age was associated with shorter hospital and ICU length of stay and fewer days of mechanical ventilation. There was no difference in adjusted mortality by admission location. CONCLUSIONS: Admission to an ICU specializing in cardiac care is associated with significantly decreased hospital costs and more efficient resource use for neonates requiring cardiac surgery.

Variation in Adolescent Idiopathic Scoliosis Surgery: Implications for Improving Healthcare Value.

OBJECTIVES: To investigate the variation in care and cost of spinal fusion for adolescent idiopathic scoliosis (AIS), and to identify opportunities for improving healthcare value. STUDY DESIGN: Retrospective cohort study from the Pediatric Health Information Systems database, including children 11-18 years of age with AIS who underwent spinal fusion surgery between 2004 and 2015. Multivariable regression was used to evaluate the relationships between hospital cost, patient outcomes, and resource use. RESULTS: There were 16 992 cases of AIS surgery identified. There was marked variation across hospitals in rates of intensive care unit admission (0.5%-99.2%), blood transfusions (0%-100%), surgical complications (1.8%-32.3%), and total hospital costs ($31 278-$90 379). Hospital cost was 32% higher at hospitals that most frequently admitted patients to the intensive care unit (P = .009), and 8% higher for each additional 25 operative cases per hospital (P = .003). Hospital duration of stay was shorter for patients admitted to hospitals with highest intensive care unit admission rates and higher surgical volumes. There was no association between cost and duration of stay, 30-day readmission, or surgical complications. The largest contribution to hospital charges was supplies (55%). Review of a single hospital's detailed cost accounting system also found supplies to be the greatest single contributor to cost, the majority of which were for spinal implants, accounting for 39% of total hospital costs. CONCLUSIONS: The greatest contribution to AIS surgery cost was supplies, the majority of which is likely attributed to spinal implant costs. Opportunities for improving healthcare value should focus on controlling costs of spinal instrumentation, and improving quality of care with standardized treatment protocols.

Resource Use and Morbidities in Pediatric Cardiac Surgery Patients with Genetic Conditions.

OBJECTIVE: To evaluate and describe resource use and perioperative morbidities among those patients with genetic conditions undergoing cardiac surgery. STUDY DESIGN: Using the Pediatric Health Information System database, we identified patients ≤18 years old with cardiac surgery classified by Risk Adjustment for Congenital Heart Surgery (RACHS) during 2003-2014. A total of 95 253 patients met study criteria and included no genetic conditions (84.6%), trisomy 21 (9.9%), trisomy 13 or 18 (0.2%), 22q11 deletion (0.8%), Turner syndrome (0.4%), and "other" genetic conditions (4.2%). We compared perioperative complications and procedures in each genetic condition with patients without genetic conditions using regression analysis. RESULTS: All groups with genetic conditions, excluding trisomy 21 RACHS 3-5, experienced increased length of stay and cost among survivors. Complications varied by genetic condition, with patients with trisomy 21 having increased odds of pulmonary hypertension and nosocomial infections. Patients with 22q11 only had increased odds of infection. Patients with Turner syndrome had increased odds of acute renal failure (OR 2.35). Patients with trisomy 13 or 18 had increased odds of pulmonary hypertension (OR 3.13), acute renal failure (OR 2.93), cardiac arrest (OR 2.84), and nosocomial infections (OR 3.53), and those with "other" genetic conditions had increased odds of all complications. CONCLUSIONS: Children with congenital heart disease and genetic conditions, except trisomy 21 RACHS 3-5, had increased costs and length of stay. Perioperative morbidities were more common and differed across genetic condition subgroups. Patient-specific risk factors are important for risk stratification, benchmarking, and counseling with families.

Use of Extracorporeal Membrane Oxygenation and Mortality in Pediatric Cardiac Surgery Patients With Genetic Conditions: A Multicenter Analysis.

OBJECTIVE: Congenital heart disease is commonly a manifestation of genetic conditions. Surgery and/or extracorporeal membrane oxygenation were withheld in the past from some patients with genetic conditions. We hypothesized that surgical care of children with genetic conditions has increased over the last decade, but their cardiac extracorporeal membrane oxygenation use remains lower and mortality greater. DESIGN: Retrospective cohort study. SETTING: Patients admitted to the Pediatric Health Information System database 18 years old or younger with cardiac surgery during 2003-2014. Genetic conditions identified by International Classification of Diseases, 9th Edition codes were grouped as follows: trisomy 21, trisomy 13 or 18, 22q11 deletion, and all "other" genetic conditions and compared with patients without genetic condition. PATIENTS: A total of 95,253 patients met study criteria, no genetic conditions (85%), trisomy 21 (10%), trisomy 13 or 18 (0.2%), 22q11 deletion (1%), and others (5%). INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Annual surgical cases did not vary over time. Compared to patients without genetic conditions, trisomy 21 patients, extracorporeal membrane oxygenation use was just over half (odds ratio, 0.54), but mortality with and without extracorporeal membrane oxygenation were similar. In trisomy 13 or 18 patients, extracorporeal membrane oxygenation use was similar to those without genetic condition, but all five treated with extracorporeal membrane oxygenation died. 22q11 patients compared with those without genetic condition had similar extracorporeal membrane oxygenation use, but greater odds of extracorporeal membrane oxygenation mortality (odds ratio, 3.44). Other genetic conditions had significantly greater extracorporeal membrane oxygenation use (odds ratio, 1.22), mortality with extracorporeal membrane oxygenation (odds ratio, 1.42), and even greater mortality odds without (odds ratio, 2.62). CONCLUSIONS: The proportion of children undergoing cardiac surgery who have genetic conditions did not increase during the study. Excluding trisomy 13 or 18, all groups of genetic conditions received and benefited from extracorporeal membrane oxygenation, although extracorporeal membrane oxygenation mortality was greater for those with 22q11 deletion and other genetic conditions.