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Echocardiographic measurements carry the promise of improving interrater (IR) agreement over subjective assessment. The aim of this study was to assess the effect of implementing a measurement-based protocol on IR agreement and accuracy in reporting of right ventricular (RV) systolic pressure in children. The effect of this reporting protocol on IR agreement in reporting RV dilation, hypertrophy, and systolic function was also evaluated.Five echocardiography readers reported their assessment of RV systolic pressure, dilation, hypertrophy, and systolic function on 40 deidentified echocardiograms using their individual accustomed methods and then using an agreed-upon protocol based solely on RV measurements. IR agreement was assessed using κ statistics. Accuracy of RV systolic pressure ratings was assessed using the McNemar test in comparison with hemodynamic data obtained by cardiac catheterization. The reliability of the RV measurements was assessed using intraclass correlation coefficient (ICC) and coefficient of variation.IR agreement and accuracy of RV systolic pressure assessment improved after using the measurement-based protocol (agreement from 0.39 [95% CI, 0.27-0.5] to 0.62 [95% CI, 0.48-0.76] and accuracy from 18 of 40 to 29 of 40 [P = .03]). IR agreement of RV dilation improved (from 0.36 [95% CI, 0.25-0.48] to 0.63 [95% CI, 0.48-0.79]), while IR agreement of RV hypertrophy (from 0.29 [95% CI, 0.17-0.42] to 0.35 [95% CI, 0.15-0.55]) and RV systolic function (from 0.57 [95% CI, 0.45-0.69] to 0.53 [95% CI, 0.41-0.66]) did not improve. The reliability of the measurements was good (ICC0.8), except for RV free wall thickness (ICC = 0.62, coefficient of variation = 24%) and RV fractional area change (ICC = 0.47, coefficient of variation = 22%), suggesting a possible reason for the lack of improvement in IR agreement of RV hypertrophy and RV systolic function. Heteroscedasticity was observed in the reliability of RV measurements, with the ICC being significantly lower at larger magnitudes for all RV measurements.Standardization of reporting protocols using RV measurements in place of subjective assessment improved IR agreement and accuracy of RV systolic pressure assessment. Reliable measurements (RV systolic pressure and dilation) resulted in improvement in IR agreement while unreliable measurements (RV hypertrophy and systolic function) did not. Special attention to measurements' reliability and heteroscedasticity of reliability is required when designing clinical protocols to decrease IR disagreement as a source of error.

Patients with early-onset lysosomal storage diseases are ideal candidates for prenatal therapy because organ damage starts in utero. We report the safety and efficacy results of in utero enzyme-replacement therapy (ERT) in a fetus with CRIM (cross-reactive immunologic material)-negative infantile-onset Pompe's disease. The family history was positive for infantile-onset Pompe's disease with cardiomyopathy in two previously affected deceased siblings. After receiving in utero ERT and standard postnatal therapy, the current patient had normal cardiac and age-appropriate motor function postnatally, was meeting developmental milestones, had normal biomarker levels, and was feeding and growing well at 13 months of age.

Our multi-institutional university consortium implemented a gastroschisis pathway in 2015 to standardize and improve care by promoting avoidance of routine intubation and paralysis during silo placement, expeditious abdominal wall closure, discontinuation of antibiotics/narcotics within 48 h of closure, and early initiation/advancement of feeds.Adherence to the gastroschisis pathway was prospectively monitored. Outcomes for the contemporary cohort (2015-2018) were compared with a historical cohort (2007-2012).Good adherence to the pathway was observed for 70 cases of inborn uncomplicated gastroschisis. The contemporary cohort had significantly lower median mechanical ventilator days (2 versus 5; p 0.01) and antibiotic days (5.5 versus 9; p 0.01) as well as earlier days to initiation of feeds (12 versus 15; p 0.01). However, no differences were observed in length of stay (28 versus 29 days; p = 0.70). A skin closure technique was performed in 66% of the patients, of which 46% were performed at bedside without intubation, the assistance of an operating-room team, or general anesthesia.In this study, adherence to a clinical pathway for gastroschisis across different facilities was feasible and led to reduction in exposure to mechanical ventilation and antibiotics. The adoption of a bedside skin closure technique appears to facilitate compliance with the pathway.Level II/III TYPE OF STUDY: Prospective comparative study with historical cohort.

OBJECTIVES: To perform a multicenter study to assess growth failure in hospitalized infants with gastroschisis. STUDY DESIGN: This study included neonates with gastroschisis within sites in the University of California Fetal Consortium (UCFC). The study’s primary outcome was growth failure at hospital discharge, defined as a weight or length z-score decrease > 0.8 from birth. Regression analysis was performed to assess changes in z-scores over time. RESULTS: Among 125 infants with gastroschisis, the median gestational age was 37 weeks (IQR 35–37). Length of stay was 32 days (23–60); 55% developed weight or length growth failure at discharge (28% had weight growth failure, 42% had length growth failure, and 15% had both weight and length growth failure). Weight and length z-scores at 14 d, 30 d, and discharge were less than birth (p

Objective: Seven public and private California cardiac centers have joined to form the first statewide consortium created to improve the quality and value of congenital cardiac care. The overall goals of the consortium are: 1) to understand variation in modifiable perioperative processes of care among participating hospitals, and 2) to determine whether standardization of those process measures will reduce adverse events and cost. In this pilot study, preoperative management of neonates undergoing arterial switch operation (ASO) within one center was analyzed for variation and these process measures were then associated with increased inpatient costs and length of stay. Methods: Through retrospective analysis of neonates undergoing ASO for Transposition of the Great Arteries with Intact Ventricular Septum (TGA/IVS) (n=34) at one center between 2004-2014, variation within the following preoperative process measures was described: feeding protocols, ventilation management, use of balloon atrial septostomy (BAS) and prostaglandin, and timing of surgery. Independent review by echocardiologists (SS, SP, AMG) confirmed preoperative and discharge diagnoses. Risk factors for increased total hospital length of stay (TLOS), postoperative length of stay (PLOS) and inpatient hospital costs were analyzed by univariable linear regression. Results: The population (N=34) was predominantly male (80%, 27/34) with low overall prenatal diagnosis rate (27%, 9/34). Birth weight was 3295±521 grams and gestational age (GA) was 39.1±1.4 weeks. Variation was found in duration of preoperative hospital stay (7.1±3.7 days), proportion of preoperative stay spent in the Cardiac ICU (0.58±0.35), use of BAS (53%, 18/34), preoperative enteral feeding (50%, 17/34) and preoperative extubation (24%, 8/34). Increased birth weight was negatively associated with costs (p=0.01), TLOS (p=0.14) and PLOS (p=0.02). Delayed timing of surgery was positively associated with TLOS (p=0.0004) and total costs (p=0.01) but not PLOS (p=0.93). Preoperative Cardiac ICU management trended towards negative association with total costs (p=0.20) but not TLOS (p=0.35) or PLOS (p=0.99) when compared with preoperative Neonatal ICU management. Lower GA, preoperative BAS, preoperative enteral feeding and preoperative extubation were not significantly associated with increased costs, TLOS, and PLOS. Conclusions: Variations in preoperative management of neonates undergoing ASO for TGA/IVS are significantly associated with total healthcare costs and hospital LOS. Concatenated data from the remaining consortium members will be critical to validate these findings and to power morbidity and mortality analysis. Integrated findings will allow the consortium to develop and implement consensus based recommendations regarding standardization of those process measures that will reduce major complications and cost.

Infants with critical congenital heart disease (CCHD) are more likely to have fetal growth restriction and subsequently be small for gestational age (SGA) at birth. While SGA infants with CCHD are known to have poor postnatal outcomes, birth weight (BW) has only been evaluated as a dichotomous

A 13-month-old boy presented with acute onset of complete atrioventricular block and wide complex tachycardia but normal hemodynamics. Endomyocardial biopsy disclosed active myocarditis with eosinophils, suggesting a hypersensitivity reaction. With no treatment, the rhythm disturbance resolved within days of onset. Our patient's presentation and self-limited illness is unique. To our knowledge, this is only the second reported case of eosinophilic myocarditis in a young child or infant.