Search

Background and Objective: Prenatal diagnosis allows improved perioperative outcomes for fetuses with certain forms of congenital heart disease (CHD). Variability in prenatal diagnosis has been demonstrated in other countries, leading to efforts to improve fetal imaging protocols and access to care, but has not been examined across the United States. The objective was to evaluate national variation in prenatal detection across geographic region and defect type in neonates and infants with CHD undergoing heart surgery., Methods: Cardiovascular operations performed in patients ≤6 months of age in the United States and included in the Society of Thoracic Surgeons Congenital Heart Surgery Database (2006-2012) were eligible for inclusion. Centers with >15% missing prenatal diagnosis data were excluded from the study. Prenatal diagnosis rates were compared across geographic location of residence and defect type using the χ(2) test., Results: Overall, the study included 31,374 patients from 91 Society of Thoracic Surgeons Congenital Heart Surgery Database participating centers across the United States. Prenatal detection occurred in 34% and increased every year, from 26% (2006) to 42% (2012). There was significant geographic variation in rates of prenatal diagnosis across states (range 11.8%-53.4%, P < .0001). Significant variability by defect type was also observed, with higher rates for lesions identifiable on 4-chamber view than for those requiring outflow tract visualization (57% vs 32%, P < .0001)., Conclusions: Rates of prenatal CHD detection in the United States remain low for patients undergoing surgical intervention, with significant variability between states and across defect type. Additional studies are needed to identify reasons for this variation and the potential impact on patient outcomes., (Copyright © 2015 by the American Academy of Pediatrics.)

Background: A better understanding of costs associated with common and resource-intense conditions such as congenital heart disease has become increasingly important as children's hospitals face growing pressure to both improve quality and reduce costs. We linked clinical information from a large registry with resource utilization data from an administrative data set to describe costs for common congenital cardiac operations and assess variation across hospitals., Methods: Using linked data from The Society of Thoracic Surgeons and Pediatric Health Information Systems Databases (2006-2010), estimated costs/case for 9 operations of varying complexity were calculated. Between-hospital variation in cost and associated factors were assessed by using Bayesian methods, adjusting for important patient characteristics., Results: Of 12,718 operations (27 hospitals) included, median cost/case increased with operation complexity (atrial septal defect repair, [$25,499] to Norwood operation, [$165,168]). Significant between-hospital variation (up to ninefold) in adjusted cost was observed across operations. Differences in length of stay (LOS) and complication rates explained an average of 28% of between-hospital cost variation. For the Norwood operation, high versus low cost hospitals had an average LOS of 50.8 vs. 31.8 days and a major complication rate of 50% vs. 25.3%. High volume hospitals had lower costs for the most complex operations., Conclusions: This study establishes benchmarks for hospital costs for common congenital heart operations and demonstrates wide variability across hospitals related in part to differences in LOS and complication rates. These data may be useful in designing initiatives aimed at both improving quality of care and reducing cost.

Background: Recent studies have called into question the benefit of perioperative corticosteroids in children undergoing heart surgery, but have been limited by the lack of placebo control, limited power, and grouping of various steroid regimens together in analysis. We evaluated outcomes across methylprednisolone regimens versus no steroids in a large cohort of neonates., Methods: Clinical data from the Society of Thoracic Surgeons Database were linked to medication data from the Pediatric Health Information Systems Database for neonates (≤30 days) undergoing heart surgery (2004-2008) at 25 participating centers. Multivariable analysis adjusting for patient and center characteristics, surgical risk category, and within-center clustering was used to evaluate the association of methylprednisolone regimen with outcome., Results: A total of 3180 neonates were included: 22% received methylprednisolone on both the day before and day of surgery, 12% on the day before surgery only, and 28% on the day of surgery only; 38% did not receive any perioperative steroids. In multivariable analysis, there was no significant mortality or length-of-stay benefit associated with any methylprednisolone regimen versus no steroids, and no difference in postoperative infection. In subgroup analysis by surgical-risk group, there was a significant association of methylprednisolone with infection consistent across all regimens (overall odds ratio 2.6, 95% confidence interval 1.3-5.2) in the lower-surgical-risk group., Conclusions: This multicenter observational analysis did not find any benefit associated with methylprednisolone in neonates undergoing heart surgery and suggested increased infection in certain subgroups. These data reinforce the need for a large randomized trial in this population.

Objective: Previous analyses have suggested center volume is associated with outcome in children undergoing heart surgery. However, data are limited regarding potential mediating factors, including the relationship of center volume with postoperative complications and mortality in those who suffer a complication. We examined this association in a large multicenter cohort., Methods: Children 0 to 18 years undergoing heart surgery at centers participating in the Society of Thoracic Surgeons Congenital Heart Surgery Database (2006-2009) were included. In multivariable analysis, we evaluated outcomes associated with annual center volume, adjusting for patient factors and surgical risk category., Results: A total of 35 776 patients (68 centers) were included. Overall, 40.6% of patients had ≥1 complication, and the in-hospital mortality rate was 3.9%. The mortality rate in those patients with a complication was 9.0%. In multivariable analysis, lower center volume was significantly associated with higher in-hospital mortality. There was no association of center volume with the rate of postoperative complications, but lower center volume was significantly associated with higher mortality in those with a complication (P = .03 when volume examined as a continuous variable; odds ratio in centers with <150 vs >350 cases per year = 1.59 [95% confidence interval: 1.16-2.18]). This association was most prominent in the higher surgical risk categories., Conclusions: These data suggest that the higher mortality observed at lower volume centers in children undergoing heart surgery may be related to a higher rate of mortality in those with postoperative complications, rather than a higher rate of complications alone.

Objective: Recently, there has been a shift toward care of children undergoing heart surgery in dedicated pediatric cardiac intensive care units (CICU). The impact of this trend on patient outcomes is unclear. We evaluated postoperative outcomes associated with a CICU versus other ICU models., Patients and Methods: Society of Thoracic Surgeons Congenital Heart Surgery Database participants (2007-2009) who completed an ICU survey were included. In multivariable analysis, we evaluated outcomes associated with a CICU versus other ICUs, adjusting for center volume, patient factors, and Society of Thoracic Surgeons-European Association for Cardiothoracic Surgery surgical risk category., Results: A total of 20 922 patients (47 centers; 25 with a CICU) were included. Overall unadjusted mortality was 3.8%, median length of stay was 6 days (interquartile range: 4-13), and 21% had 1 or more complications. In multivariable analysis, there was no difference in mortality comparing CICUs versus other ICUs (odds ratio: 0.88 [95% confidence interval: 0.65-1.19]). In stratified analysis, CICUs were associated with lower mortality only among those in Society of Thoracic Surgeons-European Association for Cardiothoracic Surgery category 3 (odds ratio: 0.47 [95% confidence interval: 0.25-0.86]), primarily related to atrioventricular canal repair and arterial switch operation. There was no difference in length of stay or complications overall or in stratified analysis., Conclusions: We were not able to detect a difference in postoperative morbidity or mortality associated with the presence of a dedicated CICU for children undergoing heart surgery. There may be a survival benefit in certain subgroups .

Objective: We describe patient characteristics and postoperative morbidity and mortality rates for patients with Down syndrome undergoing congenital heart disease surgery., Methods: This retrospective cohort study used the Society of Thoracic Surgeons Congenital Heart Surgery Database to compare patient characteristics and postoperative outcomes for patients (0-18 years) with or without Down syndrome who underwent surgery in 2000-2008., Results: A total of 45,579 patients (4350 patients with Down syndrome and 41,229 without Down syndrome) were included (median age: 7 months [interquartile range [IQR]: 47 days to 4 years]; 56% male). Patients with Down syndrome were younger at surgery, with the exception of those undergoing tetralogy of Fallot repair or atrioventricular septal defect repair. Mortality rates for patients with or without Down syndrome did not differ significantly. Lengths of stay were prolonged for patients with Down syndrome undergoing atrial septal defect closure (median: 4 days [IQR: 3-5 days] vs 3 days [IQR: 2-4 days]; P < .0001), ventricular septal defect closure (median: 5 days [IQR: 4-8 days] vs 4 days [IQR: 3-6 days]; P < .0001), or tetralogy of Fallot repair (7 days [IQR: 5-10 days] vs 6 days [IQR: 5-9 days]; P < .001) and were associated with postoperative respiratory and infectious complications. Patients with Down syndrome undergoing ventricular septal defect closure had a higher rate of heart block requiring pacemaker placement (2.9% vs 0.8%; P < .0001)., Conclusion: In this large, contemporary cohort, Down syndrome did not confer a significant mortality risk for the most common operations; however, postoperative morbidity remained common.

Objective: This pharmacologic protection trial was conducted to test the hypothesis that allopurinol, a scavenger and inhibitor of oxygen free radical production, could reduce death, seizures, coma, and cardiac events in infants who underwent heart surgery using deep hypothermic circulatory arrest (DHCA)., Design: This was a single center, randomized, placebo-controlled, blinded trial of allopurinol in infant heart surgery using DHCA. Enrolled infants were stratified as having hypoplastic left heart syndrome (HLHS) and all other forms of congenital heart disease (non-HLHS). Drug was administered before, during, and after surgery. Adverse events and the clinical efficacy endpoints death, seizures, coma, and cardiac events were monitored until infants were discharged from the intensive care unit or 6 weeks, whichever came first., Results: Between July 1992 and September 1997, 350 infants were enrolled and 348 subsequently randomized. A total of 318 infants (131 HLHS and 187 non-HLHS) underwent heart surgery using DHCA. There was a nonsignificant treatment effect for the primary efficacy endpoint analysis (death, seizures, and coma), which was consistent over the 2 strata. The addition of cardiac events to the primary endpoint resulted in a lack of consistency of treatment effect over strata, with the allopurinol treatment group experiencing fewer events (38% vs 60%) in the entire HLHS stratum, compared with the non-HLHS stratum (30% vs 27%). In HLHS surgical survivors, 40 of 47 (85%) allopurinol-treated infants did not experience any endpoint event, compared with 27 of 49 (55%) controls. There were fewer seizures-only and cardiac-only events in the allopurinol versus placebo groups. Allopurinol did not reduce efficacy endpoint events in non-HLHS infants. Treated and control infants did not differ in adverse events., Conclusions: Allopurinol provided significant neurocardiac protection in higher-risk HLHS infants who underwent cardiac surgery using DHCA. No benefits were demonstrated in lower risk, non-HLHS infants, and no significant adverse events were associated with allopurinol treatment.congenital heart defects, hypoplastic left heart syndrome, induced hypothermia, ischemia-reperfusion injury, neuroprotective agents, allopurinol, xanthine oxidase, free radicals, seizures, coma.

Objective: The purpose of this investigation was to determine the pharmacokinetic disposition of intravenous allopurinol and its metabolite oxypurinol in neonates with the hypoplastic left heart syndrome (HLHS) and to evaluate the subsequent degree of xanthine oxidase inhibition using serum uric acid as a marker., Methods: Pharmacokinetic data were evaluated in 12 stable preoperative neonates with HLHS after a single intravenous allopurinol administration of 5 mg/kg or 10 mg/kg. Pharmacokinetic parameters were determined for elimination half-life, clearance, volume of distribution, and mean residence time. Xanthine oxidase inhibition, measured by serum uric acid reduction, was also measured., Results: Pharmacokinetic parameters revealed no statistically significant differences between a 5-mg/kg and 10-mg/kg dose of intravenous allopurinol on elimination half-life, clearance, volume of distribution, and mean residence time. Mean serum uric acid levels were significantly reduced from baseline by 39.99 and 42.94%, respectively, in the 5- and 10-mg/kg treatment groups., Discussion: The enzyme xanthine oxidase plays a key biochemical role in the generation of toxic oxygen-derived free radicals during ischemia-reperfusion conditions. Allopurinol and its active metabolite oxypurinol inhibit xanthine oxidase, and significantly reduce the conversion of hypoxanthine to xanthine and xanthine to uric acid. Cell injury may be caused by toxic oxygen free radicals produced by ischemia-reperfusion injury such as could occur during the repair of HLHS under hypothermic total circulatory arrest. We hypothesize that allopurinol may provide protection from cellular injury in this clinical context.