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Objectives: This study aimed to determine if the presence of a pericardial effusion is associated with adverse outcomes among children admitted with juvenile idiopathic arthritis. Patients and methods: The multicenter, retrospective cohort study was conducted with 4,332 patients (1,554 males, 2,778 females; median age: 12 years; IQR, 7, 15 years) using the Pediatric Health Information System. Data from hospital admissions between January 1, 2004, and September 15, 2015, were obtained for patients with an International Disease Classification, Ninth Revision code for juvenile idiopathic arthritis. Pericardial effusion was the primary predictor variable; the outcomes of interest were length of stay, hospital costs, and readmission within 90 days. Multivariate models were created to evaluate associations between pericardial effusion and adverse outcomes. We also analyzed factors associated with increased odds of having pericardial effusion in juvenile idiopathic arthritis. Results: One hundred twenty (3%) patients had a code for pericardial effusion. Children with pericardial effusion had a longer median length of stay (7 days (IQR 3-12) vs. 3 days (IQR 2-6), p<0.001), higher median costs ($17,688 (IQR 8,657-40,623) vs. $8,456 (IQR 4,865-16,302), p<0.001), and greater rates of readmission (22% vs. 15%, p=0.045). Multivariate analysis demonstrated no significant association between pericardial effusion and outcomes of interest. Black race and male sex were associated with increased odds of having pericardial effusion. Conclusion: Pericardial effusion is rare among children admitted with juvenile idiopathic arthritis but is associated with significant morbidity; its presence may be a marker of disease severity. Black children and males admitted with juvenile idiopathic arthritis warrant special consideration and may benefit from screening echocardiography. [ABSTRACT FROM AUTHOR]

Objective Despite heterogeneity among Pacific Islanders, most studies aggregate them regardless of origin. Thus, limited information is available about perinatal outcomes among various subgroups of Pacific Islanders in the United States, including immigrants from the Republic of the Marshall Islands. We sought to evaluate perinatal outcomes among Marshallese women. Methods We conducted a cross-sectional study of women with at least one singleton live birth between 1997 and 2013 in two Arkansas counties using birth certificate data from the Arkansas Department of Health. Unadjusted and adjusted prevalence ratios (PR) and 95% confidence intervals (CI) were calculated from modified Poisson regression models. Results Of the 91,662 singleton births in both counties during the study period, 2488 were to Marshallese women. In adjusted analyses, Marshallese women had higher prevalence of "other medical risk factors" (PR = 1.47; 95% CI 1.30, 1.65) than NH White women. Marshallese women had higher rates of precipitous labor and fetal distress during labor compared to NH White women (PR = 2.65; 95% CI 2.22, 3.17 and 1.89; 95% CI 1.62, 2.21, respectively). Marshallese were also more likely to have tocolysis (PR = 1.43; 95% CI 1.16, 1.76), forceps (PR = 1.68; 95% CI 1.16, 2.43) or vacuum (PR = 1.89; 95% CI 1.60, 2.22) used in delivery and cesarean section (PR = 1.13; 95% CI 1.01, 1.27). Marshallese infants had higher rates of anemia (PR = 3.10; 95% CI 2.01, 4.77), birth injury (PR = 2.13; 95% CI 1.50, 3.03), assisted ventilation < 30 min (PR = 2.11; 95% CI 1.64, 2.71), preterm birth (PR = 1.67; 95% CI 1.50, 1.83), and small-for-gestational age (PR = 1.25; 95% CI 1.12, 1.39) than NH White infants. Conclusions Marshallese women and infants had higher rates of adverse perinatal outcomes compared to their NH White counterparts. Additional studies are needed to determine if perinatal outcomes among the Marshallese differed from other Pacific Islander subgroups. [ABSTRACT FROM AUTHOR]

We sought to determine the natural history of aortic valve disease in patients with unrepaired perimembranous ventricular septal defects (pVSDs) and to identify echocardiographic parameters predictive of increased risk of surgical repair of pVSD because of aortic valve disease. We retrospectively analyzed all echocardiograms of patients with a diagnosis of pVSD at our institution from January 1999 to January 2015. All available echocardiographic data were collected. Patients were excluded if there was another structural cardiac anomaly other than bicuspid aortic valve, small patent foramen ovale, or ductus arteriosus. The prevalences of aortic valve prolapse and regurgitation, as well as aortic valve disease progression, were determined. A total of 2,114 echocardiograms from 657 patients with unrepaired pVSD were reviewed. Median age at the time of echocardiogram was 1.9 years (interquartile range [IQR] 0.2 to 5.4). Median duration of follow-up was 1.7 years (IQR 0.2 to 7.4). pVSD-associated aortic valve disease prompted surgical intervention in 1.5% (10 of 657) of patients. Median age at the time of surgery was 4.8 years (IQR 1.7 to 8.4). A pVSD-to-aortic annulus diameter ratio of 0.66 ± 0.05 was present in 90% (9 of 10) of patients who underwent surgical closure because of pVSD-associated aortic valve disease. In conclusion, pVSD-associated aortic valve disease is uncommon, and progression of aortic regurgitation is rare. These data suggest that the majority of patients with pVSD do not require frequent follow-up and that frequent follow-up can be saved for a subset with echocardiographic markers placing them at higher risk of aortic valve diseases., (Copyright © 2017 Elsevier Inc. All rights reserved.)

Objective: Multiple reports have shown echocardiograms for certain indications are neither cost-effective nor of high diagnostic yield. Given the ease with which tests can be obtained at a tertiary academic children's hospital, our aims were to: (1) determine the diagnostic yield of inpatient studies by in-hospital location; (2) evaluate inpatient echocardiograms to determine indications and level of appropriateness; and (3) evaluate the frequency of cardiology involvement prior to those echocardiograms., Design: All initial inpatient echocardiograms interpreted at our institution from February 2009 to December 2014 were reviewed retrospectively. Patient location was grouped as pediatric intensive care (PICU), emergency department (ED), and general floor., Results: There were 727 first-time inpatient echocardiograms that met inclusion criteria. Pathology was identified in 25% of the study echocardiograms, with 11% of all studies demonstrating pathology that could alter patient management (moderate or severe pathology). The studies performed in the PICU and ED had more severe pathology compared with those from the general floor (P < .001, .003; respectively). Few echocardiograms were performed for rarely appropriate indications on the general floor (7%) and PICU (2.2%). Over 75% of general floor echocardiograms performed for a pathologic murmur yielded normal or incidental findings. Cardiology consultation was documented in only 7.5% of general floor studies., Conclusion: The diagnostic yield of inpatient, first-time pediatric echocardiograms is relatively low. The majority of studies that identified pathology were performed on patients located in higher acuity units. General floor echocardiograms for murmurs had a low diagnostic yield, raising the question of cardiology consultation versus direct echocardiogram ordering for subjective physical exam signs., (© 2016 Wiley Periodicals, Inc.)

Background: Congenital heart defects (CHDs) are common birth defects and are associated with high hospital costs. The objectives of this study were to assess hospitalization costs, across the lifespan, of patients with CHDs in Arkansas., Methods: Data from the 2006 to 2011 Healthcare Cost and Utilization Project Arkansas State Inpatient Databases were used. We included hospitalizations of patients whose admission occurred between January 1, 2006, and December 31, 2011, and included a principal or secondary CHD ICD-9-CM diagnosis code (745.0-747.49, except 747.0 and 745.5 for preterm infants). Hospitalizations were excluded if they involved out-of-state residents, normal newborn births, or if missing data included age at admission, state of residence, or hospital charges. Children were defined as those < 18 years-old at time of admission., Results: Between 2006 and 2011, there were 2,242,484 inpatient hospitalizations in Arkansas. There were 9071 (0.4%) hospitalizations with a CHD, including 5,158 hospitalizations of children (2.2% of hospitalizations among children) and 3,913 hospitalizations of adults (0.2% of hospitalizations of adults). Hospital costs for these CHD hospitalizations totaled $355,543,696. The average annual cost of CHD hospitalizations in Arkansas was $59,257,283 during this time period. Infants accounted for 72% of all CHD-related hospital costs; total costs of CHD hospitalizations for children were almost five times those of hospitalization costs for adults with CHD., Conclusion: Hospitalizations with CHDs account for a disproportionate share of hospital costs in Arkansas. Hospitalizations of children with CHD accounted for a higher proportion of total hospitalizations than did hospitalizations of adults with CHD., (© 2015 Wiley Periodicals, Inc.)

Background: Individuals with congenital heart defects (CHDs) have high hospital resource use. We sought to identify factors associated with hospital costs and multiple hospitalizations among individuals with CHDs., Methods: Data from the 2006 to 2011 Healthcare Cost and Utilization Project Arkansas State Inpatient Databases were linked across encrypted patient identifiers to develop a cohort of Arkansas residents aged 1 to 64 years who were hospitalized at least once with a CHD during this time period. Infants were excluded because patient identifiers were missing for 18 to 52% each year. CHDs were identified using principal and secondary International Classification of Diseases, Ninth Revision, Clinical Modification diagnoses codes. All hospitalizations of individuals ever admitted with a CHD were included. Mean and median patient-level costs were estimated; the association of hospital costs and patient readmissions were examined with linear and logistic regression., Results: There were 1,185,868 inpatient hospitalizations of Arkansas residents aged 1 to 64 years between 2006 and 2011; these were accrued by 603,925 patients. Of those, 2542 patients (0.42%) had at least one hospitalization with a CHD diagnosis. Total costs for these 2542 patients were $126,999,837 and they accumulated 7898 hospitalizations. Factors associated with increased costs included patient age, CHD type, cardiac procedures, and comorbidities. Factors associated with hospital readmission within 1 year included age, CHD type, expected payer, and comorbidities., Conclusion: Individuals with CHDs in Arkansas experience variation in hospital use and costs by patient characteristics. Future research should investigate factors associated with readmissions, cardiac procedures, and comorbidities, as these are strongly associated with hospital costs. Birth Defects Research (Part A) 103:589-596, 2015. © 2015 Wiley Periodicals, Inc., (© 2015 Wiley Periodicals, Inc.)

Marfan syndrome (MFS) is an autosomal dominant connective tissue disorder affecting 1 in 3,000 people. Cardiovascular involvement is a prominent feature of MFS, with aortic dissection and/or rupture being the leading cause of death. Advances in the medical and surgical care of patients with MFS have improved survival. Hospital resource utilization and outcomes have not been evaluated in a large population of patients with MFS. We sought to analyze pediatric hospital resource utilization and outcomes in patients with MFS. Nationally distributed data from 43 pediatric hospitals in the 2004-2011 Pediatric Health Information System database were used to identify patients admitted to the hospital with International Classification of Diseases-9th Revision codes for a diagnosis of MFS. Aortic aneurysm (AA) with or without dissection, length of stay (LOS), and hospital charges were determined. During the study period, there were 1,978 admissions in 1,228 patients with MFS. AA was present in 217 (11%) admissions in 188 (15%) patients (63% male). Mean age of patients with AA was 13.8 ± 5.9 years. Aortic dissection or rupture was present in 15 (7% with AA) admissions in 15 (8% with AA) patients (mean age 15.7 ± 5.2 years). Other cardiac diagnoses occurred more commonly in the AA cohort (p < 0.0001), regardless of the reason for admission. Cardiothoracic surgical procedures were performed in 116 AA admissions (53%). Mean LOS, hospital charges per admission, and charges per day were significantly higher in AA cohort compared to those without AA. In-hospital mortality for AA was 2%. The presence of AA in patients with MFS increases hospital resource utilization. Cardiothoracic surgeries are commonly performed in this cohort. Other cardiovascular diagnoses are more prevalent in patients with AA suggesting a more severe phenotype.

Cardiovascular abnormalities in Williams syndrome (WS) are largely attributable to elastin haploinsufficiency resulting from a large deletion of the elastin-containing region on chromosome 7q11.23. The risk of sudden death in patients with WS is 25- to 100-fold greater than that in the general population. The corrected QT (QTc) interval is prolonged in 14% of patients with WS. Patients with nonsyndromic supravalvar aortic stenosis (NSVAS) have elastin mutations resulting in elastin haploinsufficiency and a vascular phenotype nearly identical to that of WS. No previous studies have evaluated the QTc duration in NSVAS. A retrospective review of all electrocardiograms (ECGs) performed on consecutive patients with NSVAS at Arkansas Children's Hospital from January 1, 1985 to January 1, 2012 was completed. ECGs with nonsinus rhythm or unmeasurable intervals were excluded. The ECGs were read by 1 reader who was unaware of previous readings. A QTc interval of ≥460 ms was defined as prolonged. The NSVAS cohort was compared to previously published WS and control groups using the mixed model for continuous electrocardiographic variables and the generalized estimating equation for binary indicators for prolonged QTc. The generalized estimating equation used bootstrapping with 1,000 replicates. A total of 300 ECGs (median 6, range 1 to 27) from the 35 identified patients with NSVAS met the inclusion criteria. A total of 482 ECGs from patients with WS and 1,522 ECGs from controls were included. The mean age of the patients with NSVAS at ECG was 7.3 ± 6.9 years; 64% were male. The mean QTc duration was 409 ± 20 ms in the NSVAS group, 418 ± 17 ms in the control group (p <0.001), and 436 ± 27 ms in the WS group (p <0.001 compared to the control group). The prevalence of QTc prolongation was 0.3% in the NSVAS group, 2.0% in the control group (p <0.001), and 14.8% in the WS group (p <0.001 compared to controls). No patients with NSVAS died. In conclusion, cardiac repolarization is normal in patients with NSVAS. Elastin haploinsufficiency does not appear to be the etiology of QTc prolongation in patients with WS. The possible contribution of other genes on 7q11.23 to QTc prolongation in WS should be investigated., (Copyright © 2013 Elsevier Inc. All rights reserved.)