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1.  Effect of Inhaled Iloprost on the Exercise Function of Fontan Patients 
International journal of cardiology  2013;168(3):2435-2440.
Background
Exercise capacity following Fontan surgery is often depressed. An inability to reduce pulmonary vascular resistance appropriately during exercise may contribute to this phenomenon. The aim of this study was to determine whether administration of iloprost, a selective pulmonary vasodilator, would improve exercise function after Fontan procedure.
Methods
Double-blind, randomized, placebo controlled, crossover trial. Patients performed two cardiopulmonary exercise tests (CPX) separated by <1 month. A single nebulizer treatment (iloprost or placebo) was administered before each CPX.
Results
18 patients aged 12–49 (median 17) yrs were recruited. Mild throat discomfort developed in 10/18 patients during iloprost administration; all but 1 were able to complete treatment. No symptoms developed during placebo treatments (p<0.001). Two additional patients did not complete CPX: one with atrial flutter; another with developmental issues that precluded adequate CPX. In the 15 remaining subjects oxygen pulse (a surrogate for forward stroke volume) at peak exercise was higher following iloprost (median increase 1.2 ml/beat; p<0.001). Peak VO2 also rose (median increase 1.3 ml/kg/min; p<0.04). Nine patients had peak VO2 <30 ml/kg/min; each of these patients had higher peak VO2 following iloprost. Only 3/6 patients with peak VO2 >30 ml/kg/min had higher peak VO2 following iloprost (p<0.04).
Conclusions
Iloprost improves the peak oxygen pulse and peak VO2 of patients with Fontan physiology and appears to be particularly beneficial among patients with impaired exercise function. Treatment is associated with minor side effects. These findings support the concept of pulmonary vasodilator therapy in Fontan patients with limited functional capacity.
doi:10.1016/j.ijcard.2013.03.014
PMCID: PMC4288936  PMID: 23545150
Fontan procedure; heart defects; congenital; exercise testing; vasodilation
2.  Serial Cardiopulmonary Exercise Testing in Patients with Previous Fontan Surgery 
Pediatric cardiology  2010;31(2):175-180.
Patients with previous Fontan surgery have reduced peak oxygen consumption (VO2), and data regarding progression of exercise intolerance is limited. The purpose of this study was to assess the evolution of exercise tolerance in patients with previous Fontan surgery. We performed a retrospective cohort study of patients with previous Fontan surgery who underwent cardiopulmonary exercise testing between November 2002 and January 2009. Patients were required to have completed at least two tests, with adequate levels of effort, ≥6 months apart. We identified 78 patients (55% male) who had undergone a total of 215 cardiopulmonary exercise tests. Age at initial study was 19.7 ± 10.2 years; time interval since previous Fontan surgery 13.3 ± 5.7 years; and time interval between the initial and the most recent exercise test was 3.0 ± 1.4 years. Morphologic left ventricle was present in 58 (74.4%) patients. At baseline, peak VO2 averaged 24.7 ± 7.0 ml/kg/min (63.7% ± 15.8% predicted). At most recent study, peak VO2 averaged 23.2 ± 7.2 ml/kg/min, (60.4% ± 13.9% predicted). Percent predicted peak VO2 declined slowly, with a mean rate of decline of 1.25 ± 0.36 percentage points/y (p < 0.001). Most of the decline occurred in patients < 18 years of age (mean rate of decline 1.78 ± 0.46 percentage points/y; p = 0.0004). Thereafter, the rate of decline was less marked (mean rate of decline 0.54 ± 0.57 percentage points/y; p = not significant). Ventricular morphology, type of Fontan procedure, and cardiac medications were not predictive of progressive exercise intolerance. In conclusion, although the exercise function of previous Fontan patients tends to decline during late adolescence, it appears to stabilize during early adulthood.
doi:10.1007/s00246-009-9580-5
PMCID: PMC4235332  PMID: 19915891
Congenital heart disease; Exercise tests; Fontan procedure
3.  Trends in Revision Circumcision at Pediatric Hospitals 
Clinical pediatrics  2013;52(8):699-706.
Background
We sought to determine the incidence of revision circumcision at free-standing children’s hospitals, and examine trends over time.
Methods
We searched the Pediatric Health Information Systems (PHIS) database to identify boys undergoing (revision circumcision (RC), primary non-newborn circumcision (PC) or lysis of penile adhesions (LPA)) from 2004–2009. Rates of RC procedures were calculated by dividing the incidence of procedures by the total male ambulatory surgical volume.
Results
We identified 34,568 patients of whom 5,632 underwent RC, 25,768 PC, and 3,168 LPA. The rate of RC increased 119%; significantly more than PC (19%; p<.001) or LPA (37%; p<.001). Urologists performed 76% of RC and 12% were performed in with other genitourinary procedures. Boys undergoing RC were predominately white (60%) and publicly insured (61%).
Conclusions
There was a disproportionate increased rate of RC performed at PHIS hospitals compared to PC or LPA. Wide variation exists in rate increases among hospitals.
doi:10.1177/0009922813492878
PMCID: PMC4130460  PMID: 23820002
4.  Deceased Donor Liver Transplantation in Infants and Small Children: Are Partial Grafts Riskier Than Whole Organs? 
Background
Infants have the highest waitlist mortality of all liver transplant candidates. While previous studies have demonstrated that young children may be at increased risk when receiving partial grafts from adult and adolescent deceased donors (DD), with few size-matched organs available, these grafts have increasingly been used to expand the pediatric donor pool. We aimed to determine the current adjusted risk of graft failure and mortality in young pediatric recipients of DD partial livers, and to determine if these risks have changed over time.
Methods
We analyzed 2,683 first-time DD liver-alone recipients under the age of 2 years in the UNOS database (1995-2010), including 1,118 DD partial livers and 1,565 DD whole organs. Transplant factors associated with graft loss on bivariate analyses (p<0.1) were included in multivariable proportional hazards models of graft and patient survival. Interaction analysis was used to examine risks over time (time-periods:1995-2000, 2001-2005, 2006-2010).
Results
While there were significant differences in crude graft survival by graft type in 1995-2000 (p<.001), graft survival between partial and whole grafts was comparable in 2001-2005 (p=.43) and 2006-2010 (p=.36). Furthermore, while the adjusted hazards of partial graft failure and mortality were 1.40 (1.05-1.89) and 1.41 (.95-2.09) respectively in 1995-2000, the adjusted risk of graft failure and mortality was comparable between partial and whole organs in 2006-2010 (Graft failure HR .81 95%CI .56-1.18; Mortality HR 1.02 95%CI .66-1.71).
Conclusions
Deceased-donor partial liver transplantation has become less risky over time, and now has comparable outcomes to whole liver transplantation in infants and young children.
doi:10.1002/lt.23667
PMCID: PMC3837552  PMID: 23696310
Pediatrics; partial liver; risk factors; multivariate analysis; United Network of Organ Sharing
5.  Temporal Trends in Pulse Pressure and Mean Arterial Pressure During the Rise of Pediatric Obesity in US Children 
Background
Somatic growth in childhood is accompanied by substantial remodeling of the aorta. Obesity is associated with increased aortic stiffness and flow and may interfere with aortic remodeling during growth. Wide pulse pressure (PP) indicates mismatch between aortic impedance and pulsatile flow and increases risk for future systolic hypertension and cardiovascular disease (CVD). We hypothesized that the rise of pediatric obesity would be associated with a temporal trend to higher PP.
Methods and Results
We analyzed demographic, anthropometric, and blood pressure (BP) data for 8‐ to 17‐year‐old children (N=16 457) from the cross‐sectional National Health and Nutrition Examination Surveys (NHANES) for 1976 through 2008. Multivariable adjusted survey regression was used to examine temporal trends in PP and mean arterial pressure (MAP) and the relation to obesity. Across this period, unadjusted PP was higher (0.29 mm Hg/y, 95% CI 0.26 to 0.33 mm Hg/y; P<0.0001), while MAP was lower (−0.24 mm Hg/y, 95% CI −0.27 to −0.20 mm Hg/y; P<0.0001) across examinations. Adjusting for body mass index partially attenuated the temporal trend for PP by 32% (P<0.0001). Obesity amplified the relation between taller height and higher PP (from 0.23 [95% CI 0.19 to 0.28] to 0.27 [95% CI 0.21 to 0.34] mm Hg/cm height in boys and from 0.08 [95% CI 0.04 to 0.13] to 0.22 [95% CI 0.13 to 0.31] mm Hg/cm height in girls; P<0.01 for both).
Conclusions
PP has increased during the rise of pediatric obesity. Higher PP may indicate mismatch between aortic diameter, wall stiffness, and flow in obese children during a period of rapid somatic growth when the aorta is already under considerable remodeling stress.
doi:10.1161/JAHA.113.000725
PMCID: PMC4309055  PMID: 24811611
blood pressure; child; NHANES; pulse pressure
6.  Prevalence and Predictors of Gaps in Care Among Adult Congenital Heart Disease Patients (The Health, Education and Access Research Trial: HEART-ACHD) 
Objective
The goal of this project was to quantify the prevalence of gaps in cardiology care, identify predictors of gaps, and assess barriers to care among adult congenital heart disease (ACHD) patients.
Background
ACHD patients risk interruptions in care that are associated with undesired outcomes.
Methods
Patients (≥18years) with first presentation to an ACHD clinic completed a survey regarding gaps in, and barriers to, care.
Results
Among 12 ACHD centers, 922 subjects (54% female) were recruited. A >3 year gap in cardiology care was identified in 42%, with 8% having gaps longer than a decade. Mean age at first gap was 19.9 years. The majority of respondents had more than high school education, and knew their heart condition. Most common reasons for gaps included feeling well, unaware follow-up required, and complete absence from medical care. Disease complexity was predictive of gap in care with 59% of mild, 42% of moderate and 26% of severe disease subjects reporting gaps (p<0.0001). Clinic location significantly predicted gaps (p<0.0001) while gender, race, and education level did not. Common reasons for returning to care were new symptoms, referral from provider, and desire to prevent problems.
Conclusions
ACHD patients have gaps in cardiology care; the first lapse commonly occurred around 19 years, a time when transition to adult services is contemplated. Gaps were more common among subjects with mild and moderate diagnoses and at particular locations. These results provide a framework for developing strategies to decrease gaps and address barriers to care in the ACHD population.
doi:10.1016/j.jacc.2013.02.048
PMCID: PMC3664125  PMID: 23542112
Congenital; access to care; barriers
7.  Association of Urolithiasis With Systemic Conditions Among Pediatric Patients at Children’s Hospitals 
The Journal of urology  2012;188(4 0):1618-1622.
Introduction
Urolithiasis is associated with systemic medical conditions in adults, but associations have not been well studied in children. We investigated the association of urolithiasis with diabetes mellitus (DM), hypertension (HTN), and obesity among children with and without urolithiasis.
Methods
We performed a matched case-control study using the Pediatric Health Information System (PHIS) database. ICD-9 codes identified urolithiasis cases from 2004–2009. Four randomly selected controls were matched by age, hospital, patient care setting, and year of treatment. Diagnoses from all hospital encounters were ascertained for comorbid conditions. Univariate and multivariable conditional logistic regression were used to assess the associations of urolithiasis with DM, HTN, and obesity.
Results
We identified 9,843 urolithiasis cases and 39,047 controls. On univariate analysis, stone patients had significantly higher odds of obesity (OR 1.44, 95% CI 1.27–1.64) and HTN (OR 2.12, 95% CI 1.88–2.40) compared to controls. The odds of Type I DM was lower among cases compared to controls (OR 0.38, 95% CI 0.30–0.48). After adjusting for gender, race, insurance type, and number of visits using logistic regression, children with urolithiasis still had higher odds of obesity (AOR 1.30, 95% CI 1.12–1.51) and HTN (AOR 1.61, 95% CI 1.40–1.86) and lower odds of Type I DM (AOR 0.32, 95% CI 0.25–0.41) as compared to controls.
Conclusions
Among pediatric patients at freestanding children’s hospitals, urolithiasis is associated with higher odds of obesity and hypertension and a lower odds of Type I DM. These findings may be helpful in further elucidating the etiology of pediatric urolithiasis.
doi:10.1016/j.juro.2012.02.019
PMCID: PMC4005878  PMID: 22906655
Urolithiasis; Kidney stone; Diabetes; Hypertension; Obesity
8.  Vancomycin Use for Pediatric Clostridium difficile Infection Is Increasing and Associated with Specific Patient Characteristics 
In adults with Clostridium difficile infection (CDI), enteral vancomycin is considered the preferred initial regimen for severe disease; however, patterns of antimicrobial use for children with CDI are unknown. We sought to describe trends in and predictors of vancomycin use for the treatment of children with CDI admitted to tertiary-care children's hospitals in the United States. We used a database of freestanding children's hospitals to identify patients 1 to 18 years old with CDI between January 2006 and June 2011. The first hospitalization with a diagnosis of CDI for each patient was identified, and CDI-directed therapy was assessed. Generalized estimating equations were used to identify predictors of vancomycin receipt, controlling for clustering within hospitals. Vancomycin use has increased significantly (P = 0.005), with substantial variability between hospitals (0 to 16%). In multivariate analyses, vancomycin use was more common in children age 7 to 13 years old (versus children 1 to 2 years old: adjusted odds ratio [AOR] = 1.57; 95% confidence interval [CI] = 1.13 to 2.18), 14 to 18 years old (AOR = 1.40; 95% CI = 1.11 to 1.76), in an ICU (AOR = 1.37; 95% CI = 1.05 to 1.80), or with chronic gastrointestinal conditions (AOR = 2.01; 95% CI = 1.44 to 2.81). Vancomycin use was less common in black (AOR = 0.53; 95% CI = 0.39 to 0.73) and Hispanic (AOR = 0.63; 95% CI = 0.47 to 0.84) patients and in children with malignancies (AOR = 0.57; 95% CI = 0.36 to 0.89). Despite a lack of empirical evidence to suggest superiority, vancomycin use for pediatric CDI is increasing. Furthermore, there is substantial variability in vancomycin use between hospitals. Further studies are needed to explore potential racial and ethnic differences in CDI management and to investigate clinicians' rationale for using vancomycin for initial therapy in selected populations.
doi:10.1128/AAC.00661-13
PMCID: PMC3754290  PMID: 23796942
9.  FLUID BALANCE IN CRITICALLY ILL CHILDREN WITH ACUTE LUNG INJURY 
Critical care medicine  2012;40(10):2883-2889.
Objective
In the Fluid and Catheter Treatment Trial (FACTT)(NCT00281268), adults with acute lung injury (ALI) randomized to a conservative versus liberal fluid management protocol had increased days alive and free of mechanical ventilator support (ventilator-free days). Recruiting sufficient children with ALI into a pediatric trial is challenging. A Bayesian statistical approach relies on the adult trial for the a priori effect estimate, requiring fewer patients. Preparing for a Bayesian pediatric trial mirroring FACTT, we aimed to: a.)Identify an inverse association between fluid balance and VFDs; and b.)Determine if fluid balance over time is more similar to adults in the FACTT liberal or conservative arms.
Design
Multi-centered retrospective cohort study.
Setting
Five pediatric intensive care units.
Patients
Mechanically ventilated children (age ≥1 month to <18 years) with ALI admitted 2007–2010.
Interventions
None.
Measurements and Main Results
Fluid intake, output and net fluid balance were collected days 1–7 in 168 children with ALI (median age 3 years, median PaO2/FiO2 138) and weight-adjusted (ml/kg). Using multivariable linear regression to adjust for age, gender, race, admission day illness severity, PaO2/FiO2 and vasopressor use, increasing cumulative fluid balance (ml/kg) at day 3 was associated with fewer VFDs (p=0.02). Adjusted for weight, daily fluid balance on days 1–3 and cumulative fluid balance on days 1–7 were higher in these children compared to adults in the FACTT conservative arm (p<0.001, each day) and was similar to adults in the liberal arm.
Conclusions
Increasing fluid balance at day three in children with ALI at these centers is independently associated with fewer VFDs. Our findings and the similarity of fluid balance patterns in our cohort to adults in the FACTT liberal arm demonstrate the need to determine whether a conservative fluid management strategy improves clinical outcomes in children with ALI and support a Bayesian trial mirroring the FACTT trial.
doi:10.1097/CCM.0b013e31825bc54d
PMCID: PMC3455114  PMID: 22824936
acute lung injury; acute respiratory distress syndrome; fluid balance; children; critical illness; fluid; mechanical ventilation
10.  Predictors of Clinical Outcomes and Hospital Resource Use of Children After Tracheotomy 
Pediatrics  2009;124(2):563-572.
OBJECTIVES
The objectives are to describe health outcomes and hospital resource use of children after tracheotomy and identify patient characteristics that correlate with outcomes and hospital resource use.
PATIENTS AND METHODS
A retrospective analysis of 917 children aged 0 to 18 years undergoing tracheotomy from 36 children’s hospitals in 2002 with follow-up through 2007. Children were identified from ICD-9-CM tracheotomy procedure codes. Comorbid conditions (neurologic impairment [NI], chronic lung disease, upper airway anomaly, prematurity, and trauma) were identified with ICD-9-CM diagnostic codes. Patient characteristics were compared with in-hospital mortality, decannulation, and hospital resource use by using generalized estimating equations.
RESULTS
Forty-eight percent of children were ≤6 months old at tracheotomy placement. Chronic lung disease (56%), NI (48%), and upper airway anomaly (47%) were the most common underlying comorbid conditions. During hospitalization for tracheotomy placement, children with an upper airway anomaly experienced less mortality (3.3% vs 11.7%; P < .001) than children without an upper airway anomaly. Five years after tracheotomy, children with NI experienced greater mortality (8.8% vs 3.5%; P≤.01), less decannulation (5.0% vs 11.0%; P≤.01), and more total number of days in the hospital (mean [SE]: 39.5 [4.0] vs 25.6 [2.6] days; P≤.01) than children without NI. These findings remained significant (P < .01) in multivariate analysis after controlling for other significant cofactors.
CONCLUSIONS
Children with upper airway anomaly experienced less mortality, and children with NI experienced higher mortality rates and greater hospital resource use after tracheotomy. Additional research is needed to explore additional factors that may influence health outcomes in children with tracheotomy.
doi:10.1542/peds.2008-3491
PMCID: PMC3614342  PMID: 19596736
tracheotomy; children; mortality; hospitalization; health services; outcomes
11.  Contemporary Use of Nephron-Sparing Surgery for Children with Malignant Renal Tumors at Freestanding Children’s Hospitals 
Urology  2011;78(2):422-426.
Purpose
It is widely accepted that, when feasible, nephron-sparing surgery (NSS) is preferable to radical nephrectomy (RN) for treatment of renal tumors in adults. However, RN is more frequently used in children. We sought to compare in-hospital outcomes after NSS and RN for malignant pediatric renal tumors.
Patients & Methods
The Pediatric Health Information System (PHIS) combines data from over 40 North American pediatric hospitals. We queried PHIS to identify children with malignant renal tumors who underwent surgery from 2003 to 2009. We examined whether outcomes (complication rates, cost and length of stay) differed by procedure type. Multivariate regression models were used to adjust for confounding and generalized estimating equations were used to adjust for hospital clustering.
Results
We identified 1,235 children with renal tumors who underwent RN (91%) or NSS (9%). Patients undergoing RN and NSS had similar median co-morbidity scores (p=0.98), hospital length of stays (each 6.0 days, p=0.54), in-hospital charges, ($25,700 v. $37,000, p=0.11), and surgical complication rates (16.4 v. 20.5%, p=0.24). These outcomes remained similar after adjusting for other patient and hospital factors.
Conclusions
The majority of children with malignant renal tumors treated at children’s hospitals undergo RN. RN and NSS use were not significantly different in terms of their length of hospital stay, in-hospital charges, and complication rates. While oncologic outcomes are lacking, these data suggest that NSS may be performed in selected children with malignant renal tumors without significantly increasing their hospital charges, length of stay, or surgical complication rates.
doi:10.1016/j.urology.2010.12.048
PMCID: PMC3152612  PMID: 21689846
Kidney Neoplasms; Wilms Tumor; Renal Cell Carcinoma; Pediatrics
12.  A Novel Approach to Gathering and Acting on Relevant Clinical Information: SCAMPs 
Congenital Heart Disease  2010;5(4):343-353.
The current tools to adequately inform the process of improving health-care delivery consist primarily of retrospective studies, prospective trials, and clinical practice guidelines. We propose a novel and systematic approach that bridges the gap of our current tools to affect change, provides an infrastructure to improve health-care delivery, and identifies unnecessary resource utilization. The objective of this special article is to introduce the rationale and methods for this endeavor entitled “Standardized Clinical Assessment and Management Plans” (SCAMPs). SCAMPs take a relatively heterogeneous patient population and through a process of iterative analysis and modification of standardized assessment and management algorithms, SCAMPs allow the intrinsic biologic variability in a patient population to emerge and be understood. SCAMPs can be used to complement our currently available tools in order to result in incremental and sustained improvement in health-care delivery.
doi:10.1111/j.1747-0803.2010.00438.x
PMCID: PMC3376528  PMID: 20653701
Congenital Heart Disease; Practice Guidelines; Resource Utilization; Health Policy and Outcomes
13.  Resource Utilization After Introduction of a Standardized Clinical Assessment and Management Plan 
Congenital Heart Disease  2010;5(4):374-381.
Introduction
A Standardized Clinical Assessment and Management Plan (SCAMP) is a novel quality improvement initiative that standardizes the assessment and management of all patients who carry a predefined diagnosis. Based on periodic review of systemically collected data the SCAMP is designed to be modified to improve its own algorithm. One of the objectives of a SCAMP is to identify and reduce resource utilization and patient care costs.
Methods
We retrospectively reviewed resource utilization in the first 93 arterial switch operation (ASO) SCAMP patients and 186 age-matched control ASO patients. We compared diagnostic and laboratory testing obtained at the initial SCAMP clinic visit and control patient visits. To evaluate the effect of the SCAMP over time, the number of clinic visits per patient year and echocardiograms per patient year in historical control ASO patients were compared to the projected rates for ASO SCAMP participants.
Results
Cardiac magnetic resonance imaging (MRI), stress echocardiogram, and lipid profile utilization were higher in the initial SCAMP clinic visit group than in age-matched control patients. Total echocardiogram and lung scan usage were similar. Chest X-ray and exercise stress testing were obtained less in SCAMP patients. ASO SCAMP patients are projected to have 0.5 clinic visits and 0.5 echocardiograms per year. Historical control patients had more clinic visits (1.2 vs. 0.5 visits/patient year, P < .01) and a higher echocardiogram rate (0.92 vs. 0.5 echocardiograms/patient year, P <.01)
Conclusion
Implementation of a SCAMP may initially lead to increased resource utilization, but over time resource utilization is projected to decrease.
doi:10.1111/j.1747-0803.2010.00434.x
PMCID: PMC3376534  PMID: 20653704
Congenital Heart Disease; Tests; Practice Guidelines; Resource Utilization; Health Policy and Outcomes
14.  Postnatal Left Ventricular Diastolic Function After Fetal Aortic Valvuloplasty 
The American Journal of Cardiology  2011;108(4):556-560.
Fetal aortic balloon valvuloplasty (FAV) has shown promise in altering in utero progression of aortic stenosis to hypoplastic left heart syndrome. In patients who achieve a biventricular circulation after FAV, left ventricular (LV) compliance may be impaired. Echocardio-graphic indexes of diastolic function were compared between patients with biventricular circulation after FAV, congenital aortic stenosis (AS), and age-matched controls. In the neonatal period, patients with FAV had similar LV, aortic, and mitral valve dimensions but more evidence of endocardial fibroelastosis than patients with AS. Patients with FAV underwent more postnatal cardiac interventions than patients with AS (p = 0.007). Mitral annular early diastolic tissue velocity (E′) was lower in patients with FAV and those with AS and controls in the neonatal period and over follow-up (p <0.001). Septal E′ was similar among all 3 groups in the neonatal period. In follow-up patients, with FAV had lower septal E′ than patients with AS or controls (p <0.001). Early mitral inflow velocity/E′ was higher in patients with FAV as neonates and at follow-up (p <0.001). Mitral inflow pulse-wave Doppler-derived indexes of diastolic function were similar between groups. In conclusion, echocardiographic evidence of LV diastolic dysfunction is common in patients with biventricular circulation after FAV and persists in short-term follow-up. LV diastolic dysfunction in this unique population may have important implications on long-term risk of left atrial and subsequent pulmonary hypertension.
doi:10.1016/j.amjcard.2011.03.085
PMCID: PMC3374950  PMID: 21624551
15.  Resource Utilization Reduction for Evaluation of Chest Pain in Pediatrics Using a Novel Standardized Clinical Assessment and Management Plan (SCAMP) 
Background
Chest pain is a common reason for referral to pediatric cardiologists. Although pediatric chest pain is rarely attributable to serious cardiac pathology, extensive and costly evaluation is often performed. We have implemented a standardized approach to pediatric chest pain in our pediatric cardiology clinics as part of a broader quality improvement initiative termed Standardized Clinical Assessment and Management Plans (SCAMPs). In this study, we evaluate the impact of a SCAMP for chest pain on practice variation and resource utilization.
Methods and Results
We compared demographic variables, clinical characteristics, and cardiac testing in a historical cohort (n=406) of patients presenting to our outpatient division for initial evaluation of chest pain in the most recent pre-SCAMP calendar year (2009) to patients enrolled in the chest pain SCAMP (n=364). Demographic variables including age at presentation, sex, and clinical characteristics were similar between groups. Adherence to the SCAMP algorithm for echocardiography was 84%. Practice variation decreased significantly after implementation of the SCAMP (P<0.001). The number of exercise stress tests obtained was significantly lower in the SCAMP-enrolled patients compared with the historic cohort (∼3% of patients versus 29%, respectively; P<0.001). Similarly, there was a 66% decrease in utilization of Holter monitors and 75% decrease in the use of long-term event monitors after implementation of the chest pain SCAMP (P=0.003 and P<0.001, respectively). The number of echocardiograms obtained was similar between groups.
Conclusions
Implementation of a SCAMP for evaluation of pediatric chest pain has lead to a decrease in practice variation and resource utilization. (J Am Heart Assoc. 2012;1:jah3-e000349 doi: 10.1161/JAHA.111.000349.)
doi:10.1161/JAHA.111.000349
PMCID: PMC3487367  PMID: 23130120
chest pain; pediatrics; quality improvement; resource utilization
16.  Trends in Resource Utilization by Children with Neurological Impairment in the United States Inpatient Health Care System: A Repeat Cross-Sectional Study 
PLoS Medicine  2012;9(1):e1001158.
Jay Berry and colleagues report findings from an analysis of hospitalization data in the US, examining the proportion of inpatient resources attributable to care for children with neurological impairment.
Background
Care advances in the United States (US) have led to improved survival of children with neurological impairment (NI). Children with NI may account for an increasing proportion of hospital resources. However, this assumption has not been tested at a national level.
Methods and Findings
We conducted a study of 25,747,016 US hospitalizations of children recorded in the Kids' Inpatient Database (years 1997, 2000, 2003, and 2006). Children with NI were identified with International Classification of Diseases, 9th Revision, Clinical Modification diagnoses resulting in functional and/or intellectual impairment. We assessed trends in inpatient resource utilization for children with NI with a Mantel-Haenszel chi-square test using all 4 y of data combined. Across the 4 y combined, children with NI accounted for 5.2% (1,338,590) of all hospitalizations. Epilepsy (52.2% [n = 538,978]) and cerebral palsy (15.9% [n = 164,665]) were the most prevalent NI diagnoses. The proportion of hospitalizations attributable to children with NI did not change significantly (p = 0.32) over time. In 2006, children with NI accounted for 5.3% (n = 345,621) of all hospitalizations, 13.9% (n = 3.4 million) of bed days, and 21.6% (US$17.7 billion) of all hospital charges within all hospitals. Over time, the proportion of hospitalizations attributable to children with NI decreased within non-children's hospitals (3.0% [n = 146,324] in 1997 to 2.5% [n = 113,097] in 2006, p<.001) and increased within children's hospitals (11.7% [n = 179,324] in 1997 to 13.5% [n = 209,708] in 2006, p<0.001). In 2006, children with NI accounted for 24.7% (2.1 million) of bed days and 29.0% (US$12.0 billion) of hospital charges within children's hospitals.
Conclusions
Children with NI account for a substantial proportion of inpatient resources utilized in the US. Their impact is growing within children's hospitals. We must ensure that the current health care system is staffed, educated, and equipped to serve this growing segment of vulnerable children.
Please see later in the article for the Editors' Summary
Editors' Summary
Background
Disorders of the central and peripheral nervous system, often referred to as neurological impairments, are common in infants and children and can cause functional or intellectual disability. There are many causes of neurological impairments, including birth trauma, congenital abnormalities, structural defects, infections, tumors, blood flow disruption, genetic and metabolic conditions, and toxins. Symptoms can be progressive or static and vary widely depending on the condition. For example, developmental delay, changes in activity—often due to muscle wasting—and seizures may be common symptoms of neurological conditions in children. In many countries, extremely premature babies, and children with conditions such as spina bifida and muscular dystrophy, now receive better care than they used to, and may survive longer. However, although such children may have long-term care needs, they may receive crisis-driven, uncoordinated care, even in high-income countries.
Why Was This Study Done?
It is not well understood what proportion of hospital resource use is attributable to care for children with neurological impairments, although it's thought that this group may account for an increasing proportion of hospital resources. In this study, the researchers attempted to answer this question, specifically for the US, by evaluating national trends in hospital admissions for children with neurological impairments.
What Did the Researchers Do and Find?
The researchers used a multi-state database of US hospital admissions for children aged 0–18 years, known as the KID—the Healthcare Cost and Utilization Project's Kids' Inpatient Database—to identify the number of hospital admissions, total number of days spent in the hospital, and total health care costs for children with neurological impairments from 1997 to 2006. The researchers identified appropriate admissions by using diagnostic codes from the International Classification of Diseases, 9th Revision, Clinical Modification (ICD-9-CM), which were reviewed and approved by two pediatric neurologists.
The researchers found that from 1997 to 2006, there were 25,747,016 hospital admissions for children aged 0–18 years, and of these, 1,338,590 (5.2%) were associated with children who had a definite diagnosis of neurological impairment. The most prevalent diagnoses among all hospitalized children with neurological impairments were epilepsy (52.2%) and cerebral palsy (15.9%). Furthermore, across the study period, the proportion of children aged 13–18 years admitted to hospitals with neurological impairments increased from 7.3% to 9.9%. The researchers also found that children with neurological impairments accounted for an increasing proportion of days spent in a hospital (12.9% in 1997 to 13.9% in 2006). In addition, there was a substantial increase in admissions for infants with neurological impairments compared to infants without neurological impairments. The researchers also found that throughout the study period, there was a general pattern for children with neurological impairments to be admitted to pediatric hospitals, rather than general hospitals. Within children's hospitals, children with neurological impairments accounted for a substantial proportion of resources over the study period, including nearly one-third of all hospital charges.
What Do These Findings Mean?
These findings show that in the US, children with neurological impairments account for a substantial proportion of inpatient resources utilized, particularly within children's hospitals, necessitating the need for adequate clinical care and a coordination of efforts to ensure that the needs of children with neurological impairments are met. System-based efforts such as partnerships between hospitals and families of children with neurological impairments and the rigorous evaluation of care treatment strategies have the potential to promote quality care for children with neurological impairments. However, such efforts will work only if the current health care system is adequately staffed with appropriately educated professionals.
Additional Information
Please access these websites via the online version of this summary at http://dx.doi.org/pmed.1001158.
More information is available about the KID database used in this study
NHS Choices has further information about epilepsy, one of the most common types of neurological impairment examined in this study
Further information is available from PubMed Health about cerebral palsy, another neurological condition acquired during development that was studied in this dataset
doi:10.1371/journal.pmed.1001158
PMCID: PMC3260313  PMID: 22272190
17.  Patient characteristics associated with in-hospital mortality in children following tracheotomy 
Archives of disease in childhood  2010;95(9):703-710.
Objectives
To identify children at risk for in-hospital mortality following tracheotomy.
Design
Retrospective cohort study.
Setting
25 746 876 US hospitalisations for children within the Kids’ Inpatient Database 1997, 2000, 2003 and 2006.
Participants
18 806 hospitalisations of children ages 0–18 years undergoing tracheotomy, identified from ICD-9-CM tracheotomy procedure codes.
Main outcome measure
Mortality during the initial hospitalisation when tracheotomy was performed in relation to patient demographic and clinical characteristics (neuromuscular impairment (NI), chronic lung disease, upper airway anomaly, prematurity, congenital heart disease, upper airway infection and trauma) identified with ICD-9-CM codes.
Results
Between 1997 and 2006, mortality following tracheotomy ranged from 7.7% to 8.5%. In each year, higher mortality was observed in children undergoing tracheotomy who were aged <1 year compared with children aged 1–4 years (mortality range: 10.2–13.1% vs 1.1–4.2%); in children with congenital heart disease, compared with children without congenital heart disease (13.1–18.7% vs 6.2–7.1%) and in children with prematurity, compared with children who were not premature (13.0–19.4% vs 6.8–7.3%). Lower mortality was observed in children with an upper airway anomaly compared with children without an upper airway anomaly (1.5–5.1% vs 9.1–10.3%). In 2006, the highest mortality (40.0%) was observed in premature children with NI and congenital heart disease, who did not have an upper airway anomaly.
Conclusions
Congenital heart disease, prematurity, the absence of an upper airway anomaly and age <1 year were characteristics associated with higher mortality in children following tracheotomy. These findings may assist provider communication with children and families regarding early prognosis following tracheotomy.
doi:10.1136/adc.2009.180836
PMCID: PMC3118570  PMID: 20522454
18.  Variations in Timing of Surgery Among Boys Who Underwent Orchidopexy for Cryptorchidism 
Pediatrics  2010;126(3):e576-e582.
OBJECTIVE
Current clinical guidelines recommend that orchidopexy be performed by the age of 1 in patients with congenital undescended testis. We sought to examine trends in surgical timing and to determine what factors are associated with age at surgery.
METHODS
The Pediatric Health Information System (PHIS) is a national database of >40 freestanding children’s hospitals. We searched the PHIS to identify boys with cryptorchidism who underwent orchidopexy between 1999 and 2008. Patient age at orchidopexy was evaluated, and we used multivariate models to determine factors associated with timing of surgery.
RESULTS
We identified 28 204 children who underwent orchidopexy at PHIS hospitals. Of these, 14 916 (53%) were white, and 17 070 (61%) had public insurance. Only 5031 patients (18%) underwent orchidopexy by the age of 1 year; only 12 165 (43%) underwent orchidopexy by the age of 2 years. These figures remained stable over time (P = .32). After adjusting for patient clustering, race (P < .001) and insurance status (P < .001) remained associated with patient age at orchidopexy; however, the treating hospital (P < .001) was the most important factor in predicting the timing of the procedure.
CONCLUSIONS
Only 43% had surgery by 2 years of age, which suggests that either significant numbers of boys with congenital cryptorchidism do not undergo surgery in a timely fashion or late-onset testicular ectopy is more common than generally recognized. Factors associated with the timing of orchidopexy include patient race, insurance status, and the hospital in which surgery is performed.
doi:10.1542/peds.2010-0747
PMCID: PMC3102510  PMID: 20732947
cryptorchidism; pediatrics; testis; orchidopexy; clinical practice variation
20.  Reproducibility of MRI Measurements of Right Ventricular Size and Function in Patients with Normal and Dilated Ventricles 
Purpose
To determine the inter- and intra-observer reproducibility of cardiac magnetic resonance (CMR)-derived measurements of RV mass, volume, and function in patients with normal and dilated ventricles.
Materials and Methods
CMR studies of 60 patients in three groups were studied: a normal RV group (n = 20) and two groups with RV dilation—atrial septal defect (n = 20) and repaired tetralogy of Fallot (n = 20). Two independent observers analyzed each study on two separate occasions. Inter- and intra-observer reproducibility of biventricular mass, volume, ejection fraction (EF), and stroke volume measurements were calculated.
Results
High intra-class correlation coefficients (ICC) were found for inter-observer (ICC 0.94 - 0.99) and intra-observer (ICC 0.96 - 0.99) comparisons of RV and left ventricular (LV) mass, volume, and stroke volume measurements. RV and LV EF measurements were less reproducible (ICC 0.79 - 0.87). RV mass measurements were significantly less correlated than the respective LV measurements. Small but statistically significant differences in correlation were noted in RV measurements across groups.
Conclusions
Except for RV mass, inter- and intra-observer reproducibility of RV size and function measurements is high and generally comparable to that in the LV in patients with both normal and dilated RV.
doi:10.1002/jmri.21407
PMCID: PMC2533688  PMID: 18581357
magnetic resonance imaging; congenital heart disease; reproducibility; right ventricle

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