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Introduction: Children with single ventricle (SV) congenital heart disease have high morbidity, mortality and resource utilization when admitted to the hospital but few data exist on emergency department (ED) visits. Understanding patterns in ED encounters among children with SV may target interventions to improve outcomes. We described a national sample of SV ED visits and evaluated patient characteristics, encounters, and trends in admissions, charge, and mortality. Methods: This retrospective cohort study evaluates ED encounter level data from the 2006-2018 Nationwide Emergency Department Sample. Patients < 18y with SV were identified using ICD-9/10 codes. Results: From 2006-2018, 52,512 SV ED visits were identified. Most SV ED encounters were male (61%) and < 4y (65%). Patients with > 1 complex chronic condition (CCC) comprised nearly 1/3 encounters (32%). Half of encounters (49%) resulted in admission or transfer, and the median inflation-adjusted charge per ED encounter was $1,939 (IQR $1,098-3,217). Nearly 1 in 50 SV ED visits resulted in death, in the ED or during the associated admission. Independent predictors of admission included age > 2 CCC’s [OR 6.89 (5.56, 8.53)]. Independent predictors of mortality included age > 2 CCC’s [OR 2.19 (1.43, 3.36)]. Annual SV visits increased from 3,082 to 5,483 ( p for trend=0.003) and the inflation-adjusted charge per ED encounter increased by 50%, from $1,760 to $2,612 ( p for trend=0.002). There was no identified change in mortality ( p for trend=0.08). Conclusions: Half of ED visits of children with SV result in admission/transfer and 2% result in death. While the incidence and charges of SV ED visits increased over the years studied, there was no identified change in mortality. The high admission/transfer rate for children with SV presenting to the ED reflect a high-risk population; further attention must be given to improving their outcomes.

Introduction: Congenital complete heart block (CCHB) is rare, 1:15,000 - 1:20,000 live births, with left ventricular (LV) dysfunction developing in 7-14% of subjects. We investigate longitudinal trajectories of echo parameters in CCHB and the effect of pacemaker implantation on those parameters. Methods: This is a retrospective cohort study of identifiable CCHB subjects at the Children’s Hospital of Philadelphia from 1970-2018, excluding those with structural, genetic, or acquired cardiac conditions. Subjects with echo data available before and after pacemaker implantation are included. LV end diastolic diameter (LVEDD) z-score and LV ejection fraction (EF) are modeled with least-squares regression models, with slopes compared before and after pacemaker placement. Results: Of 114 subjects with CCHB, 52 (40% male, 73% white) have echo data available before and after pacemaker placement. Median age of diagnosis is 0.6 years, age at pacemaker placement 3.4 years, and duration of pacing 10.8 years. Figure 1 displays modeled trajectories of LVEDD z-score and EF over 20 years. Mean LVEDD z-score at pacemaker placement is less than 2 and decreases significantly over time, especially 10 years post placement. Participants have mean LV ejection fraction of 54% at pacemaker placement, rising significantly prior to placement and in the first 10 years post-placement, after which significant decreases are observed. Older age at pacemaker placement is associated with a higher EF at placement but an earlier and steeper decline in EF beginning at 8 years post-placement. LVEDD and EF trajectories do not vary significantly by sex, race, maternal antibody status, age at diagnosis, or initial pacemaker mode. Conclusions: In a cohort of 52 subjects with CCHB, LVEDD z-score steadily decreases over time whereas EF rises for 10 years post-pacemaker placement and subsequently decreases. Pacemaker placement has no apparent immediate effect on trajectories of these echo parameters.

Background: The arterial switch operation (ASO) is the gold standard operative correction of neonates with transposition of the great arteries and intact ventricular septum, with excellent operative survival. The associations between patient and surgeon characteristics and outcomes are well understood, but the associations between variation in preoperative care and outcomes are less well studied. Methods: A multicenter retrospective cohort study of infants undergoing neonatal ASO between January 2010 and September 2015 at hospitals contributing data to the Pediatric Health Information Systems database was performed. The association between preoperative care (timing of ASO, preoperative use of balloon atrial septostomy, prostaglandin infusion, mechanical ventilation, and vasoactive agents) and operative outcomes (mortality, length of stay, and cost) was studied with multivariable mixed-effects models. Results: Over the study period, 2159 neonates at 40 hospitals were evaluated. Perioperative mortality was 2.8%. Between hospitals, the use of adjuvant therapies and timing of ASO varied broadly. At the subject level, older age at ASO was associated with higher mortality risk (age >6 days: odds ratio, 1.90; 95% CI, 1.11–3.26; P =0.02), cost, and length of stay. Receipt of a balloon atrial septostomy was associated with lower mortality risk (odds ratio, 0.32; 95% CI, 0.17–0.59; P P =0.03), longer length of stay ( P P Conclusions: There was significant variation in preoperative care between hospitals. Some potentially modifiable aspects of perioperative care (timing of ASO and septostomy) were significantly associated with mortality, length of stay, and cost. Further research on the perioperative care of neonates is necessary to determine whether modifying practice on the basis of the observed associations translates into improved outcomes.

Background: In infants with ductal-dependent pulmonary blood flow, initial palliation with patent ductus arteriosus (PDA) stent or modified Blalock-Taussig (BT) shunt have comparable mortality but discrepant length of stay, procedural complication rates and reintervention burdens, which may influence cost. The relative economic impact of these palliation strategies is unknown. Methods and Results: Retrospective study of infants with ductal-dependent pulmonary blood flow palliated with PDA stent (n=104) or BT shunt (n=251) from 2008 to 2015 at 4 centers of the Congenital Catheterization Research Collaborative. Inflation-adjusted inpatient hospital costs were calculated for first year of life using Pediatric Health Information System data. Costs derived from outpatient catheterizations not in Pediatric Health Information System were imputed. Costs were compared using propensity score-adjusted multivariable models, to account for baseline differences between groups. After propensity score adjustment, first year of life costs were significantly lower in PDA stent ($215 825 [190 644–244 333]) than BT shunt ($249 855 [230 693–270 609]) patients ( P =0.05). After addition of imputed costs, first year of life costs were not significantly different between PDA stent ($226 403 [200 274–255 941]) and BT shunt ($252 072 [232 955–272 759]) groups ( P =0.15). Patient characteristics associated with higher costs included: younger gestational age, genetic syndrome, noncardiac diagnoses, procedural complications, extracorporeal membrane oxygenation, duration of ventilation, intensive care unit and hospital length of stay and reintervention ( P ≤0.02 for all). Conclusions: In this first multicenter comparative cost study of PDA stent or BT shunt as palliation for infants with ductal-dependent pulmonary blood flow, adjusted for baseline differences, PDA stent was associated with lower to equivalent costs over the first year of life. Combined with previous evidence suggesting clinical noninferiority, these findings suggest that PDA stent provides competitive health care value.