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Background: Anomalous aortic origin of the left coronary artery (AAOLCA) confers a rare, but significant, risk of sudden cardiac death in children. Surgery is recommended for interarterial AAOLCA, and other subtypes considered benign. We aimed to determine the clinical characteristics and outcomes of 3 AAOLCA subtypes. Methods: All patients with AAOLCA <21 years old were prospectively enrolled (December 2012–November 2020), including group 1: AAOLCA from the right aortic sinus with interarterial course, group 2: AAOLCA from the right aortic sinus with intraseptal course, and group 3: AAOLCA with a juxtacommissural origin between the left and noncoronary aortic sinus. Anatomic details were assessed using computed tomography angiography. Provocative stress testing (exercise stress testing and stress perfusion imaging) was performed in patients >8 years old or younger if concerning symptoms. Surgery was recommended for group 1 and in select cases in group 2 and group 3. Results: We enrolled 56 patients (64% males) with AAOLCA (group 1, 27; group 2, 20; group 3, 9) at median age of 12 years (interquartile range, 6–15). Intramural course was common in group 1 (93%) compared with group 3 (56%) and group 2 (10%). Seven (13%) presented with aborted sudden cardiac death (group 1, 6/27; group 3, 1/9); 1 (group 3) with cardiogenic shock. Fourteen/42 (33%) had inducible ischemia on provocative testing (group 1, 32%; group 2, 38%; group 3, 29%). Surgery was recommended in 31/56 (56%) patients (group 1, 93%; group 2, 10%; and group 3, 44%). Surgery was performed in 25 patients at a median age 12 (interquartile range, 7–15) years; all have been asymptomatic and free from exercise restrictions at median follow-up of 4 (interquartile range, 1.4–6.3) years. Conclusions: Inducible ischemia was noted in all 3 AAOLCA subtypes while most aborted sudden cardiac deaths occurred in interarterial AAOLCA (group 1). Aborted sudden cardiac death and cardiogenic shock may occur in AAOLCA with left/nonjuxtacommissural origin and intramural course, thus also deemed high-risk. A systematic approach is essential to adequately risk stratify this population. [ABSTRACT FROM AUTHOR]

Objective: The aim was to analyze the impact of decreased head computed tomography (CT) imaging on detection of abnormalities and outcomes for children with isolated head trauma., Methods: The study involves a multicenter retrospective cohort of patients younger than 19 years presenting for isolated head trauma to emergency departments in the Pediatric Health Information System database from 2003 to 2015. Patients directly admitted or transferred to another facility and those with a discharge diagnosis code for child maltreatment were excluded. Outcomes were ascertained from administrative and billing data. Trends were tested using mixed effects logistic regression, accounting for clustering within hospitals and adjusted for age, sex, insurance type, race, presence of a complex chronic condition, and hospital-level case mix index., Results: Between 2003 and 2015, 306,041 children presented for isolated head trauma. The proportion of children receiving head CT imaging was increasing until 2008, peaking at just under 40%, before declining to 25% by 2015. During the recent period of decreased head CT imaging, the detection of skull fractures (odds ratio [OR]/year, 0.96; 95% confidence interval [CI], 0.95-0.97) and intracranial bleeds (OR/year, 0.96; 95% CI, 0.94-0.97), hospitalization (OR/year, 0.96; 95% CI, 0.95-0.96), neurosurgery (OR/year, 0.91; 95% CI, 0.87-0.95), and revisit (OR/year, 0.98; 95% CI, 0.96-1.00) also decreased, without significant changes in mortality (OR/year, 0.93; 95% CI, 0.84-1.04) or persistent neurologic impairment (OR/year, 1.03; 95% CI, 0.92-1.15)., Conclusions: The recent decline in CT scanning in children with isolated head trauma was associated with a reduction in detection of intracranial abnormalities, and a concomitant decrease in interventions, without measurable patient harm., Competing Interests: Disclosure: The authors declare no conflict of interest., (Copyright © 2018 Wolters Kluwer Health, Inc. All rights reserved.)

Objective: To determine shared comorbidities and to identify underrecognized or unexpected morbidities in children with leukodystrophies using an unbiased phenome-wide association study (PheWAS) analysis of a nationwide pediatric clinical and financial database., Methods: Data were extracted from the Pediatric Health Information System database. Patients with leukodystrophy were identified with International Classification of Diseases, 10th revision, clinical modification, diagnostic codes for any of 4 specific leukodystrophies (X-linked adrenoleukodystrophy (E71.52x), Hurler disease (E76.01), Krabbe disease (E75.23), and metachromatic leukodystrophy (E75.25)) over a 3-year time period. Confirmed leukodystrophy cases (n = 553) were matched with 1659 controls. A PheWAS analysis was performed on all available ICD diagnostic codes for cases and controls. Comparisons were performed for all 4 leukodystrophies as a group and individually., Results: We found 174 phecodes (grouped ICD codes) associated with leukodystrophies, including 28 codes with a rate difference (RD) > 20%. Known comorbidities of leukodystrophies including developmental delay, epilepsy, and adrenal insufficiency were identified. Unexpected associations identified included hypertension (RD 30%, OR 25), hearing loss (RD 28%, OR 15), and cardiac dysrhythmias (RD 27%, OR 9). Hurler disease had a greater number of unique disease conditions., Conclusions: PheWAS analysis from a national database demonstrates shared and unique features of leukodystrophies. Developmental delay, cardiac dysrhythmias, fluid and electrolyte disturbances, and respiratory issues were common to all 4 leukodystrophy diseases. Use of a PheWAS in leukodystrophies and other pediatric neurologic diseases offers a method for targeting improved care for patients by identification of morbidities., (© 2019 American Academy of Neurology.)

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.

Centers with higher surgical and extracorporeal membrane oxygenation (ECMO) volumes have improved survival for children undergoing pediatric cardiac surgery and ECMO, respectively. We examined the relationship between both cardiac surgical and cardiac ECMO volumes, with survival. Using data from the Pediatric Health Information System, we reviewed patients who underwent ECMO during the hospitalization for cardiac surgery or heart transplantation from January 2003 to June 2014. Among 106,967 patients in 43 centers undergoing a Risk Adjustment for Congenital Heart Surgery-1 1-6 procedure (n = 104,951) or cardiac transplantation (n = 2,016), 2.9% (n = 3,069) underwent ECMO support. Centers were categorized into volume quartiles based on annual ECMO and cardiac surgical volumes. Multivariable logistic regression models controlling for clustering by center and adjusting for factors associated with mortality were constructed. Although mortality was lower in ECMO centers that performed ≥7 ECMO runs (odds ratio [OR]: 0.44, 95% confidence interval [CI]: 0.22-0.88)] and centers performing ≥158 cardiac surgical cases (OR: 0.37, 95% CI: 0.22-0.63), surgical volume was more strongly associated with ECMO mortality. Centers with higher cardiac surgical volume had fewer ECMO complications. Cardiac surgical volume, compared with ECMO volume, is more strongly associated with cardiac ECMO survival.

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.

Objectives: Only a small fraction of pediatric cardiac surgical patients are supported with extracorporeal membrane oxygenation following cardiac surgery, but extracorporeal membrane oxygenation use is more common among those undergoing higher complexity surgery. We evaluated extracorporeal membrane oxygenation metrics indexed to annual cardiac surgical volume to better understand extracorporeal membrane oxygenation use among U.S. cardiac surgical programs., Design: Retrospective analysis SETTING:: Forty-three U.S. Children's Hospitals in the Pediatric Health Information System that performed cardiac surgery and used extracorporeal membrane oxygenation., Patients: All patients (< 19 yr) undergoing cardiac surgery during January 2003 to July 2014., Interventions: None., Measurements and Main Results: Both extracorporeal membrane oxygenation use and surgical mortality were risk adjusted using Risk Adjustment for Congenital Heart Surgery 1. Extracorporeal membrane oxygenation metrics indexed to annual cardiac surgery cases were calculated for each hospital and the metric values divided into quintiles for comparison across hospitals. Among 131,786 cardiac surgical patients, 3,782 (2.9%) received extracorporeal membrane oxygenation. Median case mix adjusted rate of extracorporeal membrane oxygenation use was 2.8% (interquartile range, 1.6-3.4%). Median pediatric cardiac case mix adjusted surgical mortality was 3.5%. Extracorporeal membrane oxygenation-associated surgical mortality was 1.3% (interquartile range, 0.7-1.6%); without extracorporeal membrane oxygenation, median case mix adjusted surgical mortality would increase from 3.5% to 5.0%. Among patients who died, 36.7% (median) were supported with extracorporeal membrane oxygenation. The median reduction in case mix adjusted surgical mortality from extracorporeal membrane oxygenation surgical survival was 30.1%. The median extracorporeal membrane oxygenation free surgical survival was 95% (interquartile range, 94-96%). Centers with less than 150 annual surgical cases had significantly lower median extracorporeal membrane oxygenation use (0.78%) than centers with greater than 275 cases (≥ 2.8% extracorporeal membrane oxygenation use). Extracorporeal membrane oxygenation use and mortality varied within quintiles and across quintiles of center annual surgical case volume., Conclusions: Risk adjusted extracorporeal membrane oxygenation metrics indexed to annual surgical volume provide potential for benchmarking as well as a greater understanding of extracorporeal membrane oxygenation utilization, efficacy, and impact on cardiac surgery mortality.

Objective: Our objective was to develop and validate a prognostic score for predicting mortality at the time of extracorporeal membrane oxygenation initiation for children with respiratory failure. Preextracorporeal membrane oxygenation mortality prediction is important for determining center-specific risk-adjusted outcomes and counseling families., Design: Multivariable logistic regression of a large international cohort of pediatric extracorporeal membrane oxygenation patients., Setting: Multi-institutional data., Patients: Prognostic score development: A total of 4,352 children more than 7 days to less than 18 years old, with an initial extracorporeal membrane oxygenation run for respiratory failure reported to the Extracorporeal Life Support Organization's data registry during 2001-2013 were used for derivation (70%) and validation (30%). Bidirectional stepwise logistic regression was used to identify factors associated with mortality. Retained variables were assigned a score based on the odds of mortality with higher scores indicating greater mortality. External validation was accomplished using 2,007 patients from the Pediatric Health Information System dataset., Interventions: None., Measurements and Main Results: The Pediatric Pulmonary Rescue with Extracorporeal Membrane Oxygenation Prediction score included mode of extracorporeal membrane oxygenation; preextracorporeal membrane oxygenation mechanical ventilation more than 14 days; preextracorporeal membrane oxygenation severity of hypoxia; primary pulmonary diagnostic categories including, asthma, aspiration, respiratory syncytial virus, sepsis-induced respiratory failure, pertussis, and "other"; and preextracorporeal membrane oxygenation comorbid conditions of cardiac arrest, cancer, renal and liver dysfunction. The area under the receiver operating characteristic curve for internal and external validation datasets were 0.69 (95% CI, 0.67-0.71) and 0.66 (95% CI, 0.63-0.69)., Conclusions: Pediatric Pulmonary Rescue with Extracorporeal Membrane Oxygenation Prediction is a validated tool for predicting in-hospital mortality among children with respiratory failure receiving extracorporeal membrane oxygenation support.

Objectives: Recent analyses show higher mortality at low-volume centers providing extracorporeal membrane oxygenation. We sought to identify factors associated with center volume and mortality to explain survival differences and identify areas for improvement., Design: Retrospective cohort study., Setting: Patients admitted to children's hospitals in the Pediatric Health Information System database and supported with extracorporeal membrane oxygenation for respiratory failure from 2003 to 2014., Patients: A total of 5,303 patients aged 0-18 years old met inclusion criteria: 3,349 neonates and 1,954 children., Interventions: None., Measurements and Main Results: Low center volume was defined as less than 20, medium 20-49, and large greater than or equal to 50 cases per year. Center volume was also assessed as a continuous integer. Among neonates, clinical factors including intraventricular hemorrhage (relative risk, 1.4; 95% CI, 1.24-1.56) and acute renal failure (relative risk, 1.38; 95% CI, 1.20-1.60) were more common at low-volume compared to larger centers and were associated with in-hospital death. After adjustment for differences in demographic factors and primary pulmonary conditions, mild prematurity, acute renal failure, intraventricular hemorrhage, and receipt of dialysis remained independently associated with mortality, as did center volume measured as a continuous number. Among children, the risk of acute renal failure was almost 20% greater (relative risk, 1.18; 95% CI, 1.02-1.38) in small compared to large centers, but dialysis and bronchoscopy were used significantly less but were associated with mortality. After adjustment for differences in demographic factors and primary pulmonary conditions, acute renal failure, acute liver necrosis, acute pancreatitis, and receipt of bronchoscopy remained independently associated with mortality. Center volume measurement was not associated with mortality given these factors., Conclusions: Among neonates, investigation for intraventricular hemorrhage prior to extracorporeal membrane oxygenation and preservation of renal function are important factors for improvement. Earlier initiation of extracorporeal membrane oxygenation and careful attention to preservation of organ function are important to improve survival for children.

Objectives: The purposes of this study, in children with traumatic brain injury (TBI), to describe cervical spine imaging practice, to assess for recent changes in imaging practice, and to determine whether cervical spine computed tomography (CT) is being used in children at low risk for cervical spine injury., Methods: The setting was children's hospitals participating in the Pediatric Health Information System database, from January 2001 to June 2011. Participants were children (younger than 18 y) with TBI who were evaluated in the emergency department, admitted to the hospital, and received a head CT scan on the day of admission. The primary outcome measures were cervical spine imaging studies. This study was exempted from institutional review board assessment., Results: A total of 30,112 children met study criteria. Overall, 52% (15,687/30,112) received cervical spine imaging. The use of cervical spine radiographs alone decreased between 2001 (47%) and 2011 (23%), with an annual decrease of 2.2% (95% confidence interval [CI], 1.1%-3.3%), and was largely replaced by an increased use of CT, with or without radiographs (8.6% in 2001 and 19.5% in 2011, with an annual increase of 0.9%; 95% CI, 0.1%-1.8%). A total of 2545 children received cervical spine CT despite being discharged alive from the hospital in less than 72 hours, and 1655 of those had a low-risk mechanism of injury., Conclusions: The adoption of CT clearance of the cervical spine in adults seems to have influenced the care of children with TBI, despite concerns about radiation exposure.

Objective: Extracorporeal membrane oxygenation, an accepted rescue therapy for refractory cardiopulmonary failure, requires a complex multidisciplinary approach and advanced technology. Little is known about the relationship between a center's case volume and patient mortality. The purpose of this study was to analyze the relationship between hospital extracorporeal membrane oxygenation annual volume and in-hospital mortality and assess if a minimum hospital volume could be recommended., Design: Retrospective cohort study., Setting: A retrospective cohort admitted to children's hospitals in the Pediatric Health Information System database from 2004 to 2011 supported with extracorporeal membrane oxygenation was identified. Indications were assigned based on patient age (neonatal vs pediatric), diagnosis, and procedure codes. Average hospital annual volume was defined as 0-19, 20-49, or greater than or equal to 50 cases per year. Maximum likelihood estimates were used to assess minimum annual case volume., Patients: A total of 7,322 pediatric patients aged 0-18 were supported with extracorporeal membrane oxygenation and had an indication assigned., Interventions: None., Measurements and Main Results: Average hospital extracorporeal membrane oxygenation volume ranged from 1 to 58 cases per year. Overall mortality was 43% but differed significantly by indication. After adjustment for case-mix, complexity of cardiac surgery, and year of treatment, patients treated at medium-volume centers (odds ratio, 0.86; 95% CI, 0.75-0.98) and high-volume centers (odds ratio, 0.75; 95% CI, 0.63-0.89) had significantly lower odds of death compared with those treated at low-volume centers. The minimum annual case load most significantly associated with lower mortality was 22 (95% CI, 22-28)., Conclusions: Pediatric centers with low extracorporeal membrane oxygenation average annual case volume had significantly higher mortality and a minimum volume of 22 cases per year was associated with improved mortality. We suggest that this threshold should be evaluated by additional study.

The expression of MHC class II antigens and ICAM-1 and the composition of lymphocyte infiltrates have been studied in frozen sections of transbronchial biopsies from lung transplant recipients. First, biopsies obtained from patients who showed acute rejection, OB, and normal features were compared. Second, we compared first-year biopsies from patients developing OB and patients with a good clinical outcome. HLA-DR was widely expressed on epithelia and vascular endothelium. Increased vascular HLA-DP expression was found in OB biopsies. In OB patients there was a significantly increased frequency of bronchial HLA-DP and vascular HLA-DQ expression. Expression of ICAM-1 by bronchial and bronchiolar basal cells, a phenomenon not reported previously in humans, was seen in a small number of biopsies. CD8 predominant lymphocytic infiltrates were present in all groups and were increased in OB biopsies and OB patients. Increased numbers of CD4-positive cells were found in rejection and OB when compared with normal biopsies. These findings support an immunological basis for the development of OB.