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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.

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.

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: To (1) identify the major expenses for same-day adenotonsillectomy (T&A) and the costs for postoperative complication encounters in a children's hospital and (2) compare differences for variations in costs by surgeon., Study Design: Observational cohort study., Setting: Tertiary children's hospital., Subjects and Methods: A standardized activity-based hospital accounting system was used to determine total hospital costs per encounter (not including professional fees for surgeons or anesthetists) for T&A cases at a tertiary children's hospital from 2007 to 2012. Hospital costs were subdivided into categories, including operating room (OR), OR supplies, postanesthesia care unit (PACU), same-day services (SDS), anesthesia, pharmacy, and other. Costs for postoperative complication encounters were included to identify a mean total cost per case per surgeon., Results: The study cohort included 4824 T&As performed by 14 different surgeons. The mean cost per T&A was $1506 (95% confidence interval, $1492-$1519, with a range of $1156-$1828 for the lowest and highest cost per case per surgeon; P < .01). Including the cost for postoperative complications, the mean cost increased to $1599 ($1570-$1629). The largest cost categories included OR (31.9%), SDS (28.1%), and OR supplies (15.6%)., Conclusion: A large portion of T&A expenses are due to OR and supply costs. Significant differences in costs between surgeons for outpatient T&A were identified. Studies to understand the reasons for this variation and the impact on outcomes are needed. If this variation does not affect patient outcomes, then reducing this variation may improve health care value by limiting waste.