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Children with congenital heart disease are at increased risk of neurodevelopmental delay throughout their lifespan. This risk is exacerbated following congenital heart surgery (CHS) in infancy. However, there are few modifiable risk factors for postoperative neurodevelopmental delay. In this study, we assessed the Residual Lesion Score (RLS), a quality assessment metric that evaluates residual lesion severity following CHS, as a predictor of neurodevelopmental delay. This was a single-center, retrospective review of patients who underwent CHS from 01/2011 to 03/2021 and post-discharge neurodevelopmental evaluation from 12 to 42 months of age using the Bayley Scales of Infant Development, 3rd Edition (BSID-III). RLS was assigned per published criteria: RLS 1, no residua; RLS 2, minor residua; and RLS 3, major residua or pre-discharge reintervention. Associations between RLS and BSID-III scores, as well as trends in neurodevelopmental outcomes over time, were evaluated. Of 517 patients with median age at neurodevelopmental testing of 20.0 (IQR 18.0-22.7) months, 304 (58.8%), 146 (28.2%), and 67 (13.0%) were RLS 1, 2, and 3, respectively. RLS 3 patients had significantly lower scaled scores in the cognitive, receptive, and expressive communication, and fine and gross motor domains, compared with RLS 1 patients. Multivariable models accounted for 21.5%-31.5% of the variation in the scaled scores, with RLS explaining 1.4-7.3% of the variation. In a subgroup analysis, RLS 3 patients demonstrated relatively fewer gains in cognitive, expressive communication, and gross motor scores over time (all p < 0.05). In conclusion, RLS 3 patients are at increased risk for neurodevelopmental delay, warranting closer follow-up and greater developmental support for cognitive, language, and motor skills soon after surgery., (© 2023. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

Background: Many patients with repaired tetralogy of Fallot require pulmonary valve replacement (PVR) due to significant pulmonary regurgitation (PR). Transcatheter PVR (TPVR) is an equally effective and less invasive alternative to surgical PVR but many native right ventricular outflow tracts (RVOTs) are too large for TPVR at time of referral. Understanding the rate of growth of the RVOT may help optimize timing of referral. This study aims to examine the longitudinal growth of the native RVOT over time in repaired tetralogy of Fallot (TOF)., Methods: A retrospective review of serial cardiac MRI cardiovascular magnetic resonance (CMR) data from 121 patients with repaired TOF and a native RVOT (median age at first CMR 14.7 years, average interval between the first and last CMR of 8.1 years) was performed to measure serial changes in RVOT diameter, cross-sectional area, perimeter-derived diameter, and length., Results: All parameters of RVOT size continued to grow with increasing age but growth was more rapid in the decade after TOF repair (for minimum systolic diameter, mean increase of 5.7 mm per 10 years up to year 12, subsequently 2.3 mm per 10 years). The RVOT was larger with a transannular patch and in patients without pulmonary stenosis (p < 0.001 for both), but this was not associated with rate of growth. More rapid RVOT enlargement was noted in patients with larger right ventricular end-diastolic volume (RVEDV), higher PR fraction, and greater rates of increases in RVEDV and PR (p < 0.001 for all) CONCLUSIONS: in patients with repaired TOF, using serial CMR data, we found that RVOT size increased progressively at all ages, but the rate was more rapid in the first decade after repair. More rapid RVOT enlargement was noted in patients with a larger RV, more PR, and greater rates of increases in RV size and PR severity. These results may be important in considering timing of referral for transcatheter pulmonary valves, in planning transcatheter and surgical valve replacement, and in designing future valves for the native RVOT., Competing Interests: Declaration of Competing Interest The authors declare the following financial interests/personal relationships which may be considered as potential competing interests. Ashwin Prakash reports a relationship with Moderna Inc that includes: equity or stocks. If there are other authors, they declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2024 The Authors. Published by Elsevier Inc. All rights reserved.)

Objective: We sought to simplify reporting of outcomes in congenital heart surgery that compares well-defined patient groups and accommodates multiple stakeholder needs while being easily understandable., Methods: We selected 19 commonly performed congenital heart surgeries ranging in complexity from repair of atrial septal defects to the Norwood procedure. Strict inclusion/exclusion criteria ensured the creation of 19 well-defined diagnosis/procedure cohorts. Preoperative, procedural, and postoperative data were collected for consecutive eligible patients from 9 centers between January 1, 2016, and December 31, 2021. Unadjusted operative mortality rates and hospital length of stay for each of the 19 diagnosis/procedure cohorts were summarized in aggregate and stratified by each center., Results: Of 8572 eligible cases included, numbers in the 19 diagnosis/procedure cohorts ranged from 73 for tetralogy of Fallot repair after previous palliation to 1224 for ventricular septal defect (VSD) repair for isolated VSD. In aggregate, the unadjusted mortality ranged from 0% for atrial septal defect repair to 28.4% for hybrid stage I. There was significant heterogeneity in case mix and mortality for different diagnosis/procedure cohorts across centers (eg, arterial switch operation/VSD, n = 7-42, mortality 0%-7.4%; Norwood procedure, n = 16-122, mortality 5.3%-25%)., Conclusions: Reporting of institutional case volumes and outcomes within well-defined diagnosis/procedure cohorts can enable centers to benchmark outcomes, understand trends in mortality, and direct quality improvement. When made public, this type of report could provide parents with information on institutional volumes and outcomes and allow them to better understand the experience of each program with operations for specific congenital heart defects., (Copyright © 2023 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.)

Objectives: Extracorporeal membrane oxygenation to support cardiopulmonary resuscitation (CPR) is increasingly used in children suffering cardiac arrest after cardiac surgery. However, its efficacy in promoting survival has not been evaluated. We compared survival of pediatric cardiac surgery patients suffering in-hospital cardiac arrest who were resuscitated with extracorporeal CPR (E-CPR) to those resuscitated with conventional CPR (C-CPR) using propensity matching., Design: Retrospective study using multicenter data from the American Heart Association Get With The Guidelines-Resuscitation registry (2008-2020)., Setting: Multicenter cardiac arrest database containing cardiac arrest and CPR data from U.S. hospitals., Patients: Cardiac surgical patients younger than 18 years old who suffered in-hospital cardiac arrest and received greater than or equal to 10 minutes of CPR., Interventions: None., Measurements and Main Results: Among 1223 patients, 741 (60.6%) received C-CPR and 482 (39.4%) received E-CPR. E-CPR utilization increased over the study period ( p < 0.001). Duration of CPR was longer in E-CPR compared with C-CPR recipients (42 vs. 26 min; p < 0.001). In a propensity score matched cohort (382 E-CPR recipients, 382 C-CPR recipients), E-CPR recipients had survival to discharge (odds ratio [OR], 2.22; 95% CI, 1.7-2.9; p < 0.001). E-CPR survival was only higher when CPR duration was greater than 18 minutes. Propensity matched analysis using patients from institutions contributing at least one E-CPR case ( n = 35 centers; 353 E-CPR recipients, 353 C-CPR recipients) similarly demonstrated improved survival in E-CPR recipients compared with those who received C-CPR alone (OR, 2.08; 95% CI, 1.6-2.8; p < 0.001)., Conclusions: E-CPR compared with C-CPR improved survival in children suffering cardiac arrest after cardiac surgery requiring CPR greater than or equal to 10 minutes., Competing Interests: Dr. Alexander has received funding from National Institutes of Health (NIH)/National Institute of Child Health and Human Development (NICHD) (R13HD104432), Food and Drug Administration (FP01029501), U.S. Department of Defense (W81XWH2210301), and unrestricted grants from the Extracorporeal Life Support Organization (ELSO); she received funding from Novartis (consultant on the end-point adjudication committee for Prospective Trial to Assess the Angiotensin Receptor Blocker Neprilysin Inhibitor LCZ696 Versus Angiotensin-Converting Enzyme Inhibitor for the Medical Treatment of Pediatric HF (PANORAMA-HF) clinical trial); and she serves as the ELSO Treasurer of the Board of Directors. Dr. Bembea has received grants from NIH/National Institute of Neurological Disorders and Stroke (R01NS106292), NIH/NICHD, ELSO, and Grifols Investigator Sponsored Research Grant. Dr. Thiagarajan has received grants from the U.S. Department of Defense Clinical Trial Award (W81XWH2210301), ELSO, and Society of Critical Care Medicine; he is supported, in part, by the Callaghan Family Chair in Cardiac Critical Care. The remaining authors have disclosed that they do not have any potential conflicts of interest., (Copyright © 2024 by the Society of Critical Care Medicine and Wolters Kluwer Health, Inc. All Rights Reserved.)

We aimed to characterize the ranges, temporal trends, influencing factors, and prognostic significance of postoperative troponin levels after congenital heart surgery. This single-center retrospective study included patients from 2006 to 2021 who had ≥ 1 postoperative troponin-T measurement collected within 96 h of congenital heart surgery (CHS). Patients were grouped as Anomalous Aortic Origin of the Coronary Artery-"AAOCA repair," or congenital heart surgery with "Other Coronary Interventions" other than AAOCA repair, or "No Coronary Intervention." In each group, information on concomitant surgery requiring one or more of the following-atriotomy, ventriculotomy, right ventricular muscle bundle resection, and/or septal myectomy-was collected. Clinical correlates of troponin values were analyzed in three postoperative windows: < 8, 8-24, and 24-48 h. The highest median [range] troponin levels (ng/mL) for the samples were 0.34 [0.06, 1.32] at < 8 h for "AAOCA repair," 1.35 [0.14, 12.0] at < 8 h for those undergoing CHS with "Other Coronary Interventions," and 0.87 [0.06, 25.1] at 8-24 h for those undergoing CHS with "No Coronary Interventions." Atriotomy was associated with higher median troponin levels in the AAOCA group at < 8 h (0.40 [0.31, 0.77] vs. 0.29 [0.17, 0.54], P = 0.043) and in the Other Coronary Intervention group at 8-24 h (1.67 [1.04, 2.63] vs. 0.40 [0.19, 1.32], P = 0.002). Patients experiencing major postoperative complications (vs. those who did not) had higher troponin levels in the AAOCA group as early as 8-24 h (0.36 [0.24, 0.57] vs. 0.21 [0.14, 0.33], P = 0.03). Similar findings were noted in the Coronary Intervention (2.20 [1.34, 3.90] vs. 1.11 [0.51, 2.90], P = 0.028) and No Coronary Intervention (2.2 [1.49, 15.1] vs. 0.74 [0.40, 2.34], P = 0.027) groups but earlier at < 8 h. In the AAOCA group, 2/18 (11%) troponin outliers experienced cardiac arrest in comparison to 0/80 (0%) non-outliers (P = 0.032). In the Other Coronary Intervention group, troponin outliers had longer median times to ICU discharge (10 vs. 4 days) and hospital discharge (21 vs. 10 days) (both P < 0.001). Postoperative troponin levels depend on a multitude of factors and may have prognostic value in patients undergoing congenital heart surgery with coronary interventions., (© 2023. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

Objective: To use neighborhood-level Child Opportunity Index (COI) measures to investigate disparities in congenital heart surgery postoperative outcomes and identify potential targets for intervention., Study Design: In this single-institution retrospective cohort study, children <18 years old who underwent cardiac surgery between 2010 and 2020 were included. Patient-level demographics and neighborhood-level COI were used as predictor variables. COI-a composite US census tract-based score measuring educational, health/environmental, and social/economic opportunities-was dichotomized as lower (<40th percentile) vs higher (≥40th percentile). Cumulative incidence of hospital discharge was compared between groups using death as a competing risk, adjusting for clinical characteristics associated with outcomes. Secondary outcomes included hospital readmission and death within 30 days., Results: Among 6247 patients (55% male) with a median age of 0.8 years (IQR, 0.2-4.3), 26% had lower COI. Lower COI was associated with longer hospital lengths of stay (adjusted HR, 1.2; 95% CI, 1.1-1.2; P < .001) and an increased risk of death (adjusted OR, 2.0; 95% CI. 1.4-2.8; P < .001), but not hospital readmission (P = .6). At the neighborhood level, lacking health insurance coverage, food/housing insecurity, lower parental literacy and college attainment, and lower socioeconomic status were associated with longer hospital length of stay and increased risk of death. At the patient-level, public insurance (adjusted OR, 1.4; 95% CI, 1.0-2.0; P = .03) and caretaker Spanish language (adjusted OR 2.4; 95% CI, 1.2-4.3; P < .01) were associated with an increased risk of death., Conclusions: Lower COI is associated with longer length of stay and higher early postoperative mortality. Risk factors identified including Spanish language, food/housing insecurity, and parental literacy serve as potential intervention targets., Competing Interests: Declaration of Competing Interest J.M. was supported by an American Academy of Pediatrics Resident Research Grant. The funder/sponsor did not participate in the work. The authors have no conflicts of interest relevant to this article to disclose., (Copyright © 2023 Elsevier Inc. All rights reserved.)

Background: The utility of the intraoperative technical performance score (IO-TPS) in predicting outcomes after congenital cardiac surgery remains unknown., Methods: Data from patients undergoing surgery for congenital heart disease from January 2011 to December 2019 at a single institution were retrospectively reviewed. Intraoperative echocardiograms were used to assign IO-TPS for each index operation (class 1, no residua; class 2, minor residua; class 3, major residua). The primary outcome was a composite of in-hospital mortality, transplant, unplanned reintervention in the anatomic area of repair, and new permanent pacemaker implantation. Secondary outcomes included postdischarge (late) mortality or transplant and unplanned reintervention. Associations between IO-TPS and outcomes were assessed using logistic (primary) and Cox or competing risk (secondary) models, adjusting for preoperative patient- and procedure-related covariates., Results: The primary outcome was observed in 784 (11.5%) of 6793 patients who met entry criteria. On multivariable analysis, IO-TPS was a significant predictor of the primary outcome (class 2: odds ratio, 1.7 [95% CI, 1.4-2.0; P < .001]; class 3: odds ratio, 6.0 [95% CI, 4.0-8.9; P < .001]). Among 6661 transplant-free survivors of hospital discharge observed for up to 10.5 years, there were 185 (2.8%) deaths or transplants and 1171 (17.6%) reinterventions. Class 3 patients had a greater adjusted risk of late mortality or transplant (hazard ratio, 2.2; 95% CI, 1.2-4.2; P = .012) and late reintervention (subdistribution hazard ratio, 2.5; 95% CI, 1.8-3.3; P < .001) vs class 1 patients., Conclusions: IO-TPS is significantly associated with adverse early and late outcomes after congenital heart surgery and may serve as an important adjunct for self-assessment and quality improvement., (Copyright © 2023 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: Unplanned catheter-based or surgical reinterventions after congenital heart operations are independently associated with operative mortality and increased postoperative length of stay., Objectives: This study assessed the long-term outcomes of transplant-free survivors of hospital discharge requiring predischarge reinterventions after congenital cardiac surgery., Methods: Data from patients who required predischarge reinterventions in the anatomic area of repair after congenital cardiac surgery and survived to hospital discharge at a quaternary referral center from January 2011 to December 2019 were retrospectively reviewed. Previously published echocardiographic criteria were used to assess the severity of persistent residual lesions at discharge (Grade 1, no residua; Grade 2, minor residua; and Grade 3, major residua). Outcomes included postdischarge (late) mortality or transplant and unplanned reintervention. Associations between predischarge residual lesion severity and outcomes were assessed by using Cox or competing risk models, adjusting for baseline patient characteristics, case complexity, and preoperative risk factors., Results: Among the 408 patients who met entry criteria, there were 58 (14.2%) postdischarge deaths or transplants and 208 (51.0%) late reinterventions at a median follow-up of 3.0 years (IQR: 1.1-6.8 years). Greater predischarge residual lesion severity was associated with worse transplant-free survival and freedom from reintervention (both, P < 0.05). On multivariable analyses, Grade 3 patients had an increased risk of postdischarge mortality or transplant (HR: 4.8; 95% CI: 2.0-11; P < 0.001) and late reintervention (subdistribution HR: 2.1; 95% CI: 1.4-3.1; P < 0.001) vs Grade 1 patients., Conclusions: Among transplant-free survivors requiring predischarge reinterventions after congenital cardiac surgery, those with persistent major residua have significantly worse long-term outcomes. These high-risk patients warrant closer surveillance., Competing Interests: Funding Support and Author Disclosures The authors have reported that they have no relationships relevant to the contents of this paper to disclose., (Copyright © 2022 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)

Objectives: We have previously shown in experimental settings that a leaflet foldoplasty device reduces redundant leaflet area to re-establish mitral valve (MV) coaptation. The current study investigates the in vivo device retention and functional durability following foldoplasty., Methods: The prototype is of superelastic nitinol formed into a 3-dimensional shape. It is unfolded to engage a specified area of leaflet tissue and then folded to exclude this tissue from the coaptation surface. Design modifications were made and tested in benchtop studies to determine the optimal design for durable retention within the leaflet. To evaluate in vivo performance, posterior leaflet chordae were severed in Yorkshire pigs to produce complete posterior leaflet prolapse and severe mitral regurgitation. Design modifications were then used for MV repair. Five animals that underwent repair using the optimal design were observed for 2 weeks postoperative to evaluate the functional result and implant retention., Results: Device position and orientation were maintained at 2 weeks while preserving the functional MV repair in all 5 animals. Coaptation height was 5.5 ± 1.5 mm, which was not significantly different from a baseline of 4.9 ± 0.8 mm. The degree of leaflet excursion was 41.0 ± 16.0 compared to a baseline of 58.7 ± 27.5., Conclusions: Device foldoplasty is a new concept for MV repair based on the reduction of redundant leaflet tissue area. This study demonstrates the feasibility of safe maintenance of this repair without early dislodgement or embolization., (© The Author(s) 2021. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.)

Objectives: Acute coronary artery obstruction is a rare complication of congenital heart disease surgery but imposes a high burden of morbidity and mortality. Previous case series have described episodes in specific congenital heart lesions or surgical repairs but have not examined the complication in all-comers to congenital heart surgery. We hypothesize that shorter time from a clinically recognized postoperative sentinel event suggestive of coronary ischemia to diagnosis of coronary obstruction is associated with improved clinical outcomes., Methods: This was a single-center, retrospective review of patients diagnosed with acute coronary artery obstruction by angiography following surgical repair of congenital heart disease between January 2000 and June 2016., Results: In total, 34 patients were identified. The most common procedures associated with coronary artery obstruction were the Norwood procedure, arterial switch operation, and aortic valve repair/replacement. In total, 79% required mechanical circulatory support, 41% died, and 27% were listed for heart transplant. Patients who died or were listed for heart transplant had longer median sentinel-event-to-cardiac-catheterization time (28 [6-168] hours vs 10 [3-56] hours, P = .001), and longer median sentinel-event-to-intervention time (32 [11-350] hours vs 13 [5-59] hours, P = .003). Patients with hypoplastic left heart syndrome were at greater risk of death or transplant listing (odds ratio, 9.23, P = .03)., Conclusions: Time from clinically relevant postoperative sentinel event to diagnosis of coronary artery obstruction by angiography was associated with transplant-listing-free survival. Clinicians should maintain a high index of suspicion for coronary obstruction and consider early catheterization and coronary angiography for patients in whom post-operative coronary compromise is suspected., (Copyright © 2019 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.)

Objective: Technical performance score (TPS) has been associated with both early and late outcomes across a wide range of congenital cardiac procedures. A previous study has shown that the presence of residual lesions before discharge, as measured by TPS, is accurately able to identify patients who required postdischarge reinterventions after complete atrioventricular septal defect (CAVSD) repair. The aim of this study is to determine which subcomponents of TPS best predict postdischarge reinterventions after CAVSD repair., Methods: This was a single-center retrospective review of patients with CAVSD after repair between January 2000 and March 2016. We assigned TPS (class 1, no residua; class 2, minor residua; class 3, major residua or reintervention before discharge for residua) based on subcomponent scores from discharge echocardiograms. Outcome of interest was postdischarge reintervention., Results: Among 344 patients, median age was 3.2 months (interquartile range [IQR], 2.4-4.2). There were 34 (10%) postdischarge reinterventions. Median follow-up was 2.6 years (IQR, 0.09-7.9). Trisomy 21 and concomitant procedure were associated with postdischarge reinterventions. After adjusting for these factors, among the subcomponents, left atrioventricular valve stenosis and regurgitation, right atrioventricular valve regurgitation, residual ventricular septal defect, and abnormal conduction at discharge were significantly associated with postdischarge reinterventions., Conclusions: We demonstrated the ability of TPS to predict postdischarge reinterventions in patients who underwent CAVSD repair. Residual left and right atrioventricular valve regurgitation and abnormal conduction at discharge were among the subcomponents strongly associated with postdischarge reinterventions. Thus, TPS may aid clinicians in identifying children at higher risk for reintervention.

Background: Following stage 1 palliation, delayed sternal closure may be used as a technique to enhance thoracic compliance but may also prolong the length of stay and increase the risk of infection., Methods: We reviewed all neonates undergoing stage 1 palliation at our institution between 2010 and 2017 to describe the effects of delayed sternal closure., Results: During the study period, 193 patients underwent stage 1 palliation, of whom 12 died before an attempt at sternal closure. Among the 25 patients who underwent primary sternal closure, 4 (16%) had sternal reopening within 24 hours. Among the 156 infants who underwent delayed sternal closure at 4 [3,6] days post-operatively, 11 (7.1%) had one or more failed attempts at sternal closure. Patients undergoing primary sternal closure had a shorter duration of mechanical ventilation and intensive care unit length of stay. Patients who failed delayed sternal closure had a longer aortic cross-clamp time (123±42 versus 99±35 minutes, p=0.029) and circulatory arrest time (39±28 versus 19±17 minutes, p=0.0009) than those who did not fail. Failure of delayed sternal closure was also closely associated with Technical Performance Score: 1.3% of patients with a score of 1 failed sternal closure compared with 18.9% of patients with a score of 3 (p=0.0028). Among the haemodynamic and ventilatory parameters studied, only superior caval vein saturation following sternal closure was different between patients who did and did not fail sternal closure (30±7 versus 42±10%, p=0.002). All patients who failed sternal closure did so within 24 hours owing to hypoxaemia, hypercarbia, or haemodynamic impairment., Conclusion: When performed according to our current clinical practice, sternal closure causes transient and mild changes in haemodynamic and ventilatory parameters. Monitoring of SvO2 following sternal closure may permit early identification of patients at risk for failure.

Background: Risk factors for adverse clinical outcomes have been identified in patients with repaired tetralogy of Fallot before pulmonary valve replacement (PVR). However, pre-PVR predictors for post-PVR sustained ventricular tachycardia and death have not been identified., Methods: Patients with repaired tetralogy of Fallot enrolled in the INDICATOR cohort (International Multicenter TOF Registry), a 4-center international cohort study, who had a comprehensive preoperative evaluation and subsequently underwent PVR were included. Preprocedural clinical, ECG, cardiovascular magnetic resonance, and postoperative outcome data were analyzed. Cox proportional hazards multivariable regression analysis was used to evaluate factors associated with time from pre-PVR cardiovascular magnetic resonance until the primary outcome: death, aborted sudden cardiac death, or sustained ventricular tachycardia., Results: Of the 452 eligible patients (median age at PVR, 25.8 years), 36 (8%) reached the primary outcome (27 deaths, 2 resuscitated death, and 7 sustained ventricular tachycardia) at a median time after PVR of 6.5 years. Cox proportional hazards regression identified pre-PVR right ventricular ejection fraction <40% (hazard ratio, 2.39; 95% CI, 1.18-4.85; P=0.02), right ventricular mass-to-volume ratio ≥0.45 g/mL (hazard ratio, 4.08; 95% CI, 1.57-10.6; P=0.004), and age at PVR ≥28 years (hazard ratio, 3.10; 95% CI, 1.42-6.78; P=0.005) as outcome predictors. In a subgroup analysis of 230 patients with Doppler data, predicted right ventricular systolic pressure ≥40 mm Hg was associated with the primary outcome (hazard ratio, 3.42; 95% CI, 1.09-10.7; P=0.04). Preoperative predictors of a composite secondary outcome, postoperative arrhythmias and heart failure, included older age at PVR, pre-PVR atrial tachyarrhythmias, and a higher left ventricular end-systolic volume index., Conclusions: In this observational investigation of patients with repaired tetralogy of Fallot, an older age at PVR and pre-PVR right ventricular hypertrophy and dysfunction were predictive of a shorter time to postoperative death and sustained ventricular tachycardia. These findings may inform the timing of PVR if confirmed by prospective clinical trials.

Background: Isolated reports from low- and middle-income countries (LMICs) for surgical results in tetralogy of Fallot (TOF) are available. The International Quality Improvement Collaborative for Congenital Heart Disease (IQIC) seeks to improve surgical results promoting reductions in infection and mortality in LMICs., Methods: All cases of TOF in the IQIC database performed between 2010 and 2014 at 32 centers in 20 LMICs were included. Excluded from the analysis were TOF with any associated lesions. A logistic regression analysis was performed to identify risk factors for in-hospital mortality after surgery for TOF., Results: A total of 2,164 patients were identified. There were 1,839 initial primary repairs, 200 with initial systemic-to-pulmonary artery shunt, and 125 underwent secondary repair after initial palliation. Overall mortality was 3.6% (78 of 2,164), initial primary repair was 3.3% (60 of 1,839), initial systemic-to-pulmonary artery shunt was 8.0% (16 of 200), and secondary repair was 1.6% (2 of 125; p = 0.003). Major infections occurred in 5.9% (128 of 2,164) of the entire cohort. Risk factors for death after the initial primary repair were oxygen saturation less than 90% and weight/body mass index for age below the fifth percentile (p < 0.001). The initial primary repair occurred after age 1 year in 54% (991 of 1,839). Older age at initial primary repair was not a risk factor for death (p = 0.21)., Conclusions: TOF patients are often operated on after age 1 year in LMICs. Unlike in developed countries, older age is not a risk factor for death. Nutritional and hypoxemic status were associated with higher mortality and infection. This information fills a critical knowledge gap for surgery in LMIC., (Copyright © 2018 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: Blood transfusion has well-documented adverse effects. As part of a blood conservation initiative at our center, we began routine use of cell saver for all congenital heart surgery performed on cardiopulmonary bypass since 2014., Aims: This study aimed to compare transfusion rates prior to, and in the first and second year after this initiative. We hypothesized that cell saver use would decrease transfusion requirements in second year after use of the cell saver compared to the pre cell saver group., Methods: Consecutive patients under 18 years undergoing congenital heart surgery on cardiopulmonary bypass were retrospectively analyzed as 3 one-year cohorts defined above. We excluded patients who required mechanical support or reoperation at index admission. Baseline characteristics, and use of blood intraoperatively and postoperatively were compared between groups., Results: The 3 groups had similar baseline characteristics. Blood use was significantly lower in year 2 after cell saver initiation as compared to the pre cell saver group both intra- and postoperatively. The median difference in volume of intraoperative blood transfusion was lower by 138 mL/m 2 (-266, -10 mL/m 2 ) in year 2 when compared to the pre cell saver group. Similarly, the proportion of subjects requiring red blood cell transfusion postoperatively on day of surgery was lower by 10% (-15%, -6%)., Conclusion: Standardized use of cell saver significantly decreased perioperative blood use in children undergoing cardiac surgery at our center. A risk-adjusted transfusion threshold for children undergoing heart surgery needs to be developed to further decrease exposure to blood products and associated costs., (© 2018 John Wiley & Sons Ltd.)

Objectives: Although pseudoaneurysm is an uncommon complication after right ventricle-to-pulmonary artery conduit placement, it has the potential to cause significant morbidity and mortality., Methods: We performed a review of patients with pseudoaneurysms diagnosed at our institution in a 20-year period (from 1995 through 2015) and compared their clinical characteristics with a group of age- and sex-matched control patients., Results: We found that younger age, smaller size, the diagnosis of tetralogy of Fallot, the use of a pulmonary homograft conduit, the presence of an unrestrictive ventricular septal defect after conduit placement, and having at least systemic right ventricular pressure were all more common in patients who had pseudoaneurysms develop., Conclusions: This study is unique in identifying both patient and surgical factors that may predispose to pseudoaneurysm development and can help inform optimal strategies to monitor and evaluate this patient population., (Copyright © 2017 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.)

Background: The International Quality Improvement Collaborative (IQIC) was formed to reduce mortality and morbidity from congenital heart disease (CHD) surgeries in low/middle-income countries., Objectives: We conducted this study to compare the postoperative outcomes of CHD surgeries at a centre in Pakistan before and after joining IQIC., Methods: The IQIC provides guidelines targeting key drivers responsible for morbidity and mortality in postoperativepatients with CHD. We focused primarily on nurse empowerment and improving the infection control strategies at our centre. Patients with CHD who underwent surgery at this site during the period 2011-2012 (pre-IQIC) were comparedwith those getting surgery in 2013-2014 (post-IQIC). Morbidity (major infections), mortality and factors associated with them were assessed., Results: There was a significant decrease in surgical site infections and bacterial sepsis in the post-IQIC versus pre-IQIC period (1% vs 30%, p=0.0001, respectively). A statistically insignificant decrease in the mortality rate was also noted in post-IQIC versus pre-IQIC period (6% vs 9%, p=0.17, respectively). Durations of ventilation and intensive care unit (ICU) and hospital stay were significantly reduced in the post-IQIC period. Age <1 year, malnutrition, low preoperative oxygen perfusion, Risk Adjustment for Congenital Heart Surgery score >3, major chromosomal anomalies, perfusion-related event, longer ventilation and ICU/hospital stay durations were associated with greater odds of morbidity and mortality., Conclusion: Enrolling in the IQIC programme was associated with an improvement in the postsurgical outcomes of the CHD surgeries at our centre., Competing Interests: Competing interests: None declared., (© Article author(s) (or their employer(s) unless otherwise stated in the text of the article) 2017. All rights reserved. No commercial use is permitted unless otherwise expressly granted.)

Objectives: Management strategy for unbalanced complete atrioventricular septal defects (CASVSDs) includes single-ventricle (SV) palliation and primary or staged biventricular (BiV) repair. More recently, BiV conversion (BiVC) from SV palliation and staged BiV recruitment (BiVR) have also been advocated. This study assesses mid-term outcomes in patients with unbalanced CASVSDs according to management strategy., Methods: Consecutive patients with unbalanced CASVSDs who underwent surgery at a tertiary care centre from January 2000 to February 2016 with institutional review board approval. The index surgery was defined as the first palliation procedure for the SV group, a BiV repair for the BiV repair group and conversion or the first procedure for recruitment for the BiVC/BiVR group. Kaplan-Meier and Cox regression were used for time-to-event analysis of death/transplant and unplanned reinterventions that occurred after the index surgery., Results: There were 212 patients: 82 (38.7%) had SV palliation, 67 (31.6%) had BiV repair and 63 (29.7%) had BiVC/BiVR, respectively; 50 patients had undergone a successful BiVC. There were 93 (43.9%) boys; 51 (24%) patients had Down syndrome; and 101 (48%) patients had heterotaxy. In the entire cohort, there were 40 (18.9%) deaths, 110 (51.9%) reinterventions, 82 (38.7%) surgical reinterventions and 70 (33%) catheter reinterventions, with some patients having more than 1 reintervention. Median length of follow-up was 35 (range 1-192) months. The BiVC/BiVR group had a transplant-free survival benefit similar to that of the primary BiV repair group compared with the SV group. The BiV repair group needed fewer catheter-based reinterventions compared with the SV and BiVC/BiVR groups., Conclusions: BiVC or BiVR from an SV pathway can be achieved with low rates of mortality and morbidity in patients with unbalanced CASVSDs., (© The Author 2017. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.)

Background: Postoperative infections contribute substantially to morbidity and mortality after congenital heart disease surgery and are often preventable. We sought to identify risk factors for postoperative infection and the impact on outcomes after congenital heart surgery, using data from the International Quality Improvement Collaborative for Congenital Heart Surgery in Developing World Countries., Methods and Results: Pediatric cardiac surgical cases performed between 2010 and 2012 at 27 participating sites in 16 developing countries were included. Key variables were audited during site visits. Demographics, preoperative, procedural, surgical complexity, and outcome data were analyzed. Univariate and multivariable logistic regression were used to identify risk factors for infection, including bacterial sepsis and surgical site infection, and other clinical outcomes. Standardized infection ratios were computed to track progress over time. Of 14 545 cases, 793 (5.5%) had bacterial sepsis and 306 (2.1%) had surgical site infection. In-hospital mortality was significantly higher among cases with infection than among those without infection (16.7% versus 5.3%; P <0.001), as were postoperative ventilation duration (80 versus 14 hours; P <0.001) and intensive care unit stay (216 versus 68 hours; P <0.001). Younger age at surgery, higher surgical complexity, lower oxygen saturation, and major medical illness were independent risk factors for infection. The overall standardized infection ratio was 0.65 (95% confidence interval, 0.58-0.73) in 2011 and 0.59 (95% confidence interval, 0.54-0.64) in 2012, compared with that in 2010., Conclusions: Postoperative infections contribute to mortality and morbidity after congenital heart surgery. Younger, more complex patients are at particular risk. Quality improvement targeted at infection risk may reduce morbidity and mortality in the developing world., (© 2017 American Heart Association, Inc.)

Background: Little has been published regarding surgery for transposition of the great arteries (TGA) in the developing world., Objectives: This study sought to identify patient characteristics, surgical interventions, institutional characteristics, risk factors for mortality, and outcomes among patients undergoing surgery for TGA in this setting., Methods: Developing world congenital heart surgical programs submitted de-identified data to a novel international collaborative database as part of a quality improvement project. We conducted a retrospective cohort study that included all cases of TGA with intact ventricular septum and TGA with ventricular septal defect performed from 2010 to 2013. Demographic, surgical, and institutional characteristics and their associations with in-hospital mortality were identified., Results: There were 778 TGA operations performed at 26 centers, 480 (62%) for TGA with intact ventricular septum and 298 (38%) for TGA with ventricular septal defect. Most (80%) were single-stage arterial switch operations, but 20% were atrial baffling procedures (atrial switch operation) or 2-stage repairs (pulmonary artery band followed by arterial switch operation). Age at operation was >30 days in one-half of the cases and did not vary significantly with operation type. Survival was 85% and did not significantly vary with age at operation or operation type. Preceding septostomy was infrequently reported (16%) and was not associated with surgical mortality. Mortality was associated with lower World Health Organization weight/body mass index-for-age percentile and lower institutional volume of TGA repair., Conclusions: Surgical repair of TGA performed in the developing world is associated with an early survival of 85%. Type of surgical repair and age at operation varied considerably, but no associations with mortality were identified. In contrast, poor nutrition and small surgical volume were most strongly associated with mortality. Multicenter collaborative quality improvement efforts may benefit patients with TGA in the developing world., (Copyright © 2017 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)

Background: Previous studies have demonstrated the effect of adult nursing skill mix, staffing ratios, and level of education on patient deaths, complication rates, and failure to rescue (FTR). To date, only one known study had examined the effect of nursing experience and education on postoperative pediatric cardiac operations., Methods: Nursing survey data were linked to The Society of Thoracic Surgeons (STS) Congenital Heart Surgery Database for patients undergoing cardiac operations (2010 to 2011). Logistic regression models were used to estimate associations of nursing education and years of clinical experience with in-hospital mortality rates, complication rates, and FTR. Generalized estimating equations and robust standard error estimates were used to account for within-center correlation of outcomes., Results: Among 15,463 patients (29 hospitals), the in-hospital mortality rate was 2.8%, postoperative complications occurred in 42.4%, and the FTR rate was 6.4%. After covariate adjustment, pediatric critical care units with a higher proportion of nurses with a Bachelor of Science degree or higher had lower odds of complication (odds ratio for 10% increase, 0.85; 95% confidence interval, 0.76 to 0.96; p = 0.009). Units with a higher proportion of nurses with more than 2 years of experience had lower mortality rates (odds ratio for 10% increase, 0.92; 95% confidence interval, 0.85 to 0.99; p = 0.025)., Conclusions: This is the first study to demonstrate that higher levels of nursing education and experience are significantly associated with fewer complications after pediatric cardiac operations and aligns with our previous findings on their association with reduced deaths. These results provide data for pediatric hospital leaders and reinforce the importance of organization-wide mentoring strategies for new nurses and retention strategies for experienced nurses., (Copyright © 2016 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: The National Quality Forum previously approved a quality indicator for mortality after congenital heart surgery developed by the Agency for Healthcare Research and Quality (AHRQ). Several parameters of the validated Risk Adjustment for Congenital Heart Surgery (RACHS-1) method were included, but others differed. As part of the National Quality Forum endorsement maintenance process, developers were asked to harmonize the 2 methodologies., Methods and Results: Parameters that were identical between the 2 methods were retained. AHRQ's Healthcare Cost and Utilization Project State Inpatient Databases (SID) 2008 were used to select optimal parameters where differences existed, with a goal to maximize model performance and face validity. Inclusion criteria were not changed and included all discharges for patients <18 years with International Classification of Diseases, Ninth Revision, Clinical Modification procedure codes for congenital heart surgery or nonspecific heart surgery combined with congenital heart disease diagnosis codes. The final model includes procedure risk group, age (0-28 days, 29-90 days, 91-364 days, 1-17 years), low birth weight (500-2499 g), other congenital anomalies (Clinical Classifications Software 217, except for 758.xx), multiple procedures, and transfer-in status. Among 17 945 eligible cases in the SID 2008, the c statistic for model performance was 0.82. In the SID 2013 validation data set, the c statistic was 0.82. Risk-adjusted mortality rates by center ranged from 0.9% to 4.1% (5th-95th percentile)., Conclusions: Congenital heart surgery programs can now obtain national benchmarking reports by applying AHRQ Quality Indicator software to hospital administrative data, based on the harmonized RACHS-1 method, with high discrimination and face validity., (© 2016 The Authors. Published on behalf of the American Heart Association, Inc., by Wiley Blackwell.)

Background: In response to societal pressure to reduce expenditures and increase quality, we sought to develop a methodology to predict hospital charges related to congenital heart surgery., Methods: Patients undergoing congenital heart surgery at Boston Children's Hospital in fiscal years 2007 to 2009 comprised the derivation cohort. Clinical data, including Current Procedural Terminology coding of the primary surgical intervention, were collected prospectively and linked to total hospital charges for an episode of care. Surgical charge categories were developed to group surgical procedure types using empiric data and expert consensus. A multivariable model was built using surgical charge categories and additional patient and procedural characteristics to predict the outcome, total hospital charges. A contemporary cohort for fiscal years 2010 to 2012 was used to validate surgical charge categories and the multivariable model., Results: In the derivation cohort, 2,105 cases met inclusion criteria. One hundred three surgical procedure types were categorized into seven surgical charge categories, yielding a grouper variable with an R(2) explanatory value of 47.3%. Explanatory value increased with consideration of patient age, admission status, and preoperative ventilator dependence (R(2) = 59.4%), as well as weight category, noncardiac abnormality, and genetic syndrome other than trisomy 21 (R(2) = 61.5%). Additional variability in charge was explained when extracorporeal membrane oxygenation utilization and greater than one operating room visit during the episode of care were added (R(2) = 74.3%). The contemporary cohort yielded an R(2) explanatory value of 67.7%., Conclusions: The combination of clinical data with resource utilization information resulted in a statistically valid predictive model for total hospital charges in congenital heart surgery., (Copyright © 2015 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Objectives: In a previous study of infants less than 6 month old, we found that delayed revision of residual lesions resulted in worse patient outcomes compared with intraoperative revision. We explored a larger cohort to determine if this finding persisted., Methods: A prospective cohort followed from index surgery to discharge from January 2011 to September 2013 were divided into 4 groups: (1) intraoperative revisions (IO) of residual lesions, (2) delayed postoperative revision (PO) of residual lesions during the same hospital stay, (3) both intraoperative and delayed (BOTH) revision of residual lesions, (4) no intraoperative or postoperative revision (NO). Linear and logistic regression analyses were used to compare outcomes of postoperative hospital length of stay, postoperative adverse events (AE), hospital costs, and mortality, after adjusting for age, prematurity, presence of extracardiac anomalies, and RACHS-1 (Risk Adjustment for Congenital Heart Surgery-1) risk category known to affect outcomes., Results: Of the 2427 patients discharged after a congenital cardiac operation, 1886 were eligible for this study after exclusion of adults, procedures performed off cardiopulmonary bypass, and transplants and assist devices. On multivariable modeling adjusting for other significant patient factors, the NO group fared better than the other 3 groups. The IO group had significantly lower postoperative length of stay, AE rate, and hospital costs compared with the PO and BOTH groups, but showed no significant differences in mortality., Conclusions: Intraoperative correction of residual lesions results in shorter length of stay, and lower postoperative AE and costs compared with delayed postoperative revision of residual lesions., (Copyright © 2014 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.)

Objective: We compared the histologic findings in explanted CorMatrix (9 patients) and autologous pericardium (9 patients) used for valvuloplasty of the aortic (7 patients) and/or mitral (11 patients) valve in patients with congenital heart defects., Methods: We used standard tissue stains and immunohistochemistry to identify the inflammatory cell type., Results: CorMatrix was associated with an intense inflammatory response in the surrounding native tissue, extending into CorMatrix in 8 of 9 cases, continuing to the longest follow-up point (9 months). The typical response included macrophages and giant cells in contact with the material, surrounded by lymphocytes, macrophages, plasma cells, and eosinophils. The thickness of the residual CorMatrix material was 280 to 300 μm, similar to the nominal thickness at implantation and unrelated to the implantation duration. Only at the longest follow-up interval was any significant resorption of CorMatrix material evident. A neointima had formed on the surface of CorMatrix, increasing in thickness with the period in situ. Mild cellular infiltration of CorMatrix was noted in all cases; however, in no case, did it appear that CorMatrix was being remodeled into tissue resembling a 3-layered native valve. In contrast, a near absence of any inflammatory reaction was seen and no eosinophilia associated with autologous pericardium was present, irrespective of the duration in situ. Furthermore, we observed more tissue infiltration, remodeling, vascularization, and neointima formation with autologous pericardium., Conclusions: Although CorMatrix used for valve repair induced an intense inflammatory response, little or no remodeling to form tissue resembling a 3-layered native valve was seen at ≤9 months after implantation., (Copyright © 2014 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.)

Background: There is little information about congenital heart surgery outcomes in developing countries. The International Quality Improvement Collaborative for Congenital Heart Surgery in Developing World Countries uses a registry and quality improvement strategies with nongovernmental organization reinforcement to reduce mortality. Registry data were used to evaluate impact., Methods: Twenty-eight sites in 17 developing world countries submitted congenital heart surgery data to a registry, received annual benchmarking reports, and created quality improvement teams. Webinars targeted 3 key drivers: safe perioperative practice, infection reduction, and team-based practice. Registry data were audited annually; only verified data were included in analyses. Risk-adjusted standardized mortality ratios (SMRs) and standardized infection ratios among participating sites were calculated., Results: Twenty-seven sites had verified data in at least 1 year, and 1 site withdrew. Among 15,049 cases of pediatric congenital heart surgery, unadjusted mortality was 6.3% and any major infection was 7.0%. SMRs for the overall International Quality Improvement Collaborative for Congenital Heart Surgery in Developing World Countries were 0.71 (95% confidence interval [CI] 0.62-0.81) in 2011 and 0.76 (95% CI 0.69-0.83) in 2012, compared with 2010 baseline. SMRs among 7 sites participating in all 3 years were 0.85 (95% CI 0.71-1.00) in 2011 and 0.80 (95% CI 0.66-0.96) in 2012; among 14 sites participating in 2011 and 2012, the SMR was 0.80 (95% CI 0.70-0.91) in 2012. Standardized infection ratios were similarly reduced., Conclusions: Congenital heart surgery risk-adjusted mortality and infections were reduced in developing world programs participating in the collaborative quality improvement project and registry. Similar strategies might allow rapid reduction in global health care disparities., (Copyright © 2014 by the American Academy of Pediatrics.)

Objective: This study explored pediatric critical care nursing and organizational factors that impact in-hospital mortality for cardiac surgery patients across children's hospitals in the United States., Background: Congenital heart disease is the most common birth defect and the no. 1 cause of death for infants with a congenital defect. Little is known about the impact of pediatric critical care nursing and organizational factors on pediatric mortality., Methods: Nursing leaders from 38 children's hospitals that contribute data to the Pediatric Health Information System data set completed an organizational assessment for years 2009 and 2010. These data were linked with patient-level data. The Risk Adjustment for Congenital Heart Surgery method was used to adjust for baseline patient differences in patients younger than 18 years., Results: The odds of death increased as the institutional percentage of pediatric critical care unit nurses with 2 years' clinical experience or less increased. The odds of mortality were highest when the percentage of RNs with 2 years' clinical experience or less was 20% or greater. The odds of death decreased as the institutional percentage of critical care nurses with 11 years' clinical experience or more increased and for hospitals participating in national quality metric benchmarking. Clinical experience was independently associated with in-hospital mortality., Conclusions: These data are the 1st to link clinical nursing experience with pediatric patient outcomes. A cut point of 20% RNs or greater with 2 years' clinical experience or less was determined to significantly affect inpatient mortality. Participation in national quality metric benchmarking programs was significantly associated with improved mortality.

Objectives: Technical Performance Score (TPS) has been shown to have a strong association with early and late outcomes after congenital cardiac surgery, with greater morbidity and reintervention in children with major residual lesions (TPS class 3). We sought to explore the effect of TPS on the neurodevelopmental outcomes., Methods: All infants undergoing cardiac surgery, excluding those with trisomy 21, were offered neurodevelopmental testing at 1 year of age using the Bayley Scales of Infant Development, 3rd edition. TPSs from the discharge echocardiograms were graded as class 1 (optimal), class 2 (minor residual), or class 3 (major residual). Multivariate regression analysis was performed using patient characteristics and preoperative variables., Results: Neurodevelopmental testing was performed in 140 patients at a median age of 16 months. Of these, 28 (20%) had single ventricle palliation; 39 (28%) were in Risk Adjustment for Congenital Heart Surgery category 4 to 6. Significant differences between the groups were found in the cognitive (P = .01) and motor (P = .05) domains, with subjects in TPS class 3 having significantly lower cognitive and motor composite scores. The scores did not vary significantly according to single ventricle versus biventricular repair or Risk Adjustment for Congenital Heart Surgery categorization. In multivariate modeling, class 3 TPS remained significantly associated with a lower Bayley cognitive score (P = .02), with a trend toward a lower Bayley motor score (P = .08)., Conclusions: We found that TPS is an independent predictor of neurodevelopmental outcomes after infant heart surgery. Future research should explore whether a structured program of intraoperative recognition and intervention on residual lesions can improve the TPS and neurodevelopmental outcomes., (Copyright © 2014 The American Association for Thoracic Surgery. Published by Mosby, Inc. All rights reserved.)

Objective: This study explored pediatric critical care nursing and organizational factors that impact in-hospital mortality for cardiac surgery patients across children's hospitals in the United States., Background: Congenital heart disease is the most common birth defect and the no. 1 cause of death for infants with a congenital defect. Little is known about the impact of pediatric critical care nursing and organizational factors on pediatric mortality., Methods: Nursing leaders from 38 children's hospitals that contribute data to the Pediatric Health Information System data set completed an organizational assessment for years 2009 and 2010. These data were linked with patient-level data. The Risk Adjustment for Congenital Heart Surgery method was used to adjust for baseline patient differences in patients younger than 18 years., Results: The odds of death increased as the institutional percentage of pediatric critical care unit nurses with 2 years' clinical experience or less increased. The odds of mortality were highest when the percentage of RNs with 2 years' clinical experience or less was 20% or greater. The odds of death decreased as the institutional percentage of critical care nurses with 11 years' clinical experience or more increased and for hospitals participating in national quality metric benchmarking. Clinical experience was independently associated with in-hospital mortality., Conclusions: These data are the 1st to link clinical nursing experience with pediatric patient outcomes. A cut point of 20% RNs or greater with 2 years' clinical experience or less was determined to significantly affect inpatient mortality. Participation in national quality metric benchmarking programs was significantly associated with improved mortality.

Background: We have shown previously that technical performance score (TPS) is strongly associated with early mortality and major postoperative adverse events in a diverse group of patients. We now report evaluation of the validity of TPS in predicting late outcomes in the same group of patients., Methods: Patients who underwent surgery between June 1, 2005 and June 30, 2006 were included. The TPS were assigned based on discharge echocardiograms and certain clinical criteria as previously described. Follow-up data for up to 4 years were retrospectively collected. Cox proportional hazards models were used for analysis., Results: A total of 679 patients were included in the analysis. One hundred twenty-three (18%) were neonates, 213 (31%) infants, 291 (435) children, and 52 (8%) adults. Four hundred ninety-one (72%) were in low-risk adjustment in congenital heart surgery (RACHS; 1 to 3), 109 (16%) in high risk (4 to 6), and 27 (4%) were less than 18 years and could not be assigned a RACHS score. Three hundred thirty-one (48%) had an optimal TPS, 283 (42%) adequate, 61 (9%) inadequate, and 4 (1%) could not be scored. There were 34 (5%) late deaths and 149 (22%) late unplanned reinterventions. By univariate analysis, age, RACHS-1 categories, and TPS were all significantly associated with late reintervention (p < 0.001 for all), while TPS and RACHS-1 were significant factors for mortality (p < 0.001). On multivariable modeling, inadequate TPS was strongly associated with both late mortality (p = 0.001; HR [hazard ratio] 3.8, CI [confidence interval] 1.7 to 8.4) and late reintervention (p = 0.002, HR 2.1, CI 1.3 to 3.3) after controlling for RACHS-1 and age., Conclusions: The TPS has a strong association with late outcomes across a wide range of age and disease complexity and may serve as a tool to identify patients who are at a higher risk for late reintervention or mortality., (Copyright © 2013 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Objective: Risk factors for mortality after neonatal cardiac surgery have been seldom studied. We sought to identify contemporary risk factors for mortality and the impact of surgical technical performance on surgical outcomes after neonatal cardiac surgery., Methods: We conducted a matched case-control study comparing 56 neonates who died after cardiac surgery (2002-2008) with 56 survivors matched by surgical procedure and year of surgery. Surgical efficacy for repair or palliation was graded using a reliable simple surgical technical score. Patient and surgical characteristics were compared for the survivors and nonsurvivors using paired analyses., Results: There was no significant difference between patients who died and their matched controls in terms of age, Aristotle score, Risk Adjustment in Congenital Heart Surgery-1 category, and single versus biventricular repair. When compared with survivors, patients who died were more likely to be premature (41% vs 5%, P < .001), to weigh less than 2.5 kg (25% vs 9%, P = .05), and to have inadequate surgical repair or palliation (55% vs 9%, P < .001). Cardiopulmonary bypass time was longer for the patients who died (median, 159 vs 133 minutes, P = .002). Highest postoperative lactate (median, 9.0 vs 6.0, P < .001), use of extracorporeal membrane oxygenation (71% vs 13%, P < .001), and reoperation during the same admission (75% vs 2%, P < .001) were also associated with death. In multivariable analysis, inadequate surgical repair or palliation (odds ratio, 11, P = .02) and need for postoperative extracorporeal membrane oxygenation (odds ratio, 5.1, P = .009) were the only risk factors associated with hospital death., Conclusions: Our study highlights the need for optimal technical performance to minimize neonatal deaths. This has important implications when sustaining or developing a pediatric cardiac program., (Copyright © 2012 The American Association for Thoracic Surgery. Published by Mosby, Inc. All rights reserved.)

Objective: Mortality among children with congenital and acquired heart disease has decreased significantly over the past decades. We sought to determine whether the underlying problems leading to death in these patients had changed over the past decade., Methods: We reviewed medical records for 100 deaths of cardiac patients in 2004-2005 and 100 deaths in 1995-1996. Demographic, clinical, and procedural data as well as circumstances of death were collected. A consensus committee reviewed each case and sought to identify the condition leading to death. These conditions were classified as predominantly surgical or medical., Results: General patient characteristics (age, gender, cardiac history, comorbidities, proportion of surgical patients) did not change significantly between the two time periods. However, in 1995-1996, 64% of deceased surgical patients had died within 30 days of surgery. This rate was nearly halved to only 38% by 2004-2005 (P= .003). Furthermore, the conditions leading to death changed significantly: 51% of patient deaths in 1995-1996 resulted from a surgical problem, 29% from a medical condition. This ratio was reversed in 2004-2005: Only 31% of patient deaths were due to a surgical problem, while 50% of deaths resulted from a medical condition (P= .005). The most common medical conditions resulting in death were pulmonary vein stenosis, pulmonary arterial hypertension, and primary myocardial failure., Conclusions: The proportion of deaths within 30 days of cardiac surgery decreased significantly over the past decade. While surgical causes accounted for the majority of these deaths in 1995-1996, most patient deaths in 2004-2005 resulted from cardiac medical causes., (© 2012 Wiley Periodicals, Inc.)

Background: Long-term outcomes of repair of tetralogy of Fallot associated with complete atrioventricular septal defect are seldom reported. We report our survival and reintervention outcomes over a 29-year time period., Methods: Between March 1979 and April 2008, 61 patients with the combined cardiac defect of atrioventricular septal defect and tetralogy of Fallot were surgically managed. Trisomy 21 was present in 49 (80%) patients. Primary repair was performed in 36 patients at a median age of 9 months (range, 1 month to 16 years), whereas 25 patients had initial palliation by systemic-pulmonary shunt at a median age of 21 months (range, 0 days to 36 years). Thirty-one (51%) patients had a transannular patch. Fifty-three patients required right ventriculotomy for relief of the right ventricular outflow tract obstruction. Four patients had a right ventricle-pulmonary artery conduit with a homograft. Relationships between patient characteristics and outcome variables were examined using Kaplan-Meier survival curves; comparisons were performed using the log-rank test., Results: Median follow-up was 4.7 years. A total of 12 patients died during the course of follow-up: 4 (7%)deaths within 30 days of surgery and 8 late deaths (range, 4 months to 9.9 years after repair). Since 2000, there have been no early deaths and 1 late death, 5 months after the operation. The estimated survival at 5 years after definitive repair was 82% (95% confidence interval, 69%, 90%). Time to death was not associated with any patient or surgical variables examined. Overall, 30% of the survivors required a reoperation. The type of reoperations was on the mitral valve (4 repairs, 4 replacements) and 7 pulmonary valve replacements. We did not find an effect of era on mortality (P = .23 for comparison of 1979-1989, 1990-1999, and 2000-2008). The percentage of patients with primary repair did not change during the different quartiles. The estimated freedom from reoperation at 5 years was 80% (65%, 90%). Time to reoperation was shorter for patients with a conduit (P = .01)., Conclusions: Excellent long-term survival was achieved after repair of tetralogy of Fallot associated with complete atrioventricular septal defect. Palliation and primary repair resulted in comparable outcomes; as such, primary repair is favored. The choice of right ventricular outflow tract reconstruction affects the need for reoperation., (Copyright © 2012 The American Association for Thoracic Surgery. Published by Mosby, Inc. All rights reserved.)

Background: Despite the central role that pediatric hospitals play in the surgical treatment of congenital heart disease, little is known about outcomes of adult congenital cardiac surgical care in pediatric hospitals. Risk factors for inpatient death, including adult congenital heart (ACH) surgery volume, are poorly described., Methods and Results: We obtained inpatient data from 42 free-standing pediatric hospitals using the Pediatric Health Information System data base 2000 to 2008 and selected ACH surgery admissions (ages 18 to 49 years). We examined admission characteristics and hospital surgery volume. Of 97 563 total (pediatric and adult) congenital heart surgery admissions, 3061 (3.1%) were ACH surgery admissions. Median adult age was 22 years and 39% were between ages 25 to 49 years. Most frequent surgical procedures were pulmonary valve replacement, secundum atrial septal defect repair, and aortic valve replacement. Adult mortality rate was 2.2% at discharge. Multivariable analyses identified the following risk factors for death: age 25 to 34 years (adjusted odds ratio [AOR], 2.1; P=0.009), age 35 to 49 years (AOR, 3.2; P=0.001), male sex (AOR, 1.8; P=0.04), government-sponsored insurance (AOR, 1.8; P=0.03), and higher surgical risk categories 4+ (AOR, 21.5; P=0.001). After adjusting for case mix, pediatric hospitals with high ACH surgery volume had reduced odds for death (AOR, 0.4; P=0.003). There was no relationship between total congenital heart surgery volume and ACH inpatient mortality., Conclusions: Older adults, male sex, government-sponsored insurance, and greater surgical case complexity have the highest likelihood of in-hospital death when adult congenital surgery is performed in free-standing pediatric hospitals. After risk-adjustment, pediatric hospitals with high ACH surgery volume have the lowest inpatient mortality.

Objective: The objective of the study was to examine the impact of staffing ratios on risk-adjusted outcomes for pediatric cardiac surgery programs in California and relative to other states combined., Background: California performs 20% of the nation's pediatric cardiac surgery and is the only state with a nurse ratio law. Understanding the imposition of mandated ratios on pediatric outcomes is necessary to inform the debate about nurse staffing., Data Sources: Patient variables were extracted from the Healthcare Cost and Utilization Project Kids' Inpatient Database. The American Hospital Association database was used for institutional variables., Methods: Descriptive analyses were used to identify and describe patient, nursing, and hospital characteristics. Changes in nursing ratios and full-time equivalents (FTEs) between 2003 and 2006 were examined. Associations between nursing characteristics and each outcome variable were examined using general estimating equation models. The RACHS-1 (Risk Adjustment for Congenital Heart Surgery) risk adjustment method was used for mortality., Results: Hospitals in California significantly increased RN FTEs (P = .025) and RN ratios (P = .036) after enactment of AB 394 in 2006. Neither RN FTEs nor RN ratios were associated with mortality, complications, or resource utilization after risk adjustment. After the law, California's standardized mortality ratio (SMR) decreased more (33%) than in all other states combined (29%). Standardized complication ratio (SCR) increased by 5% but decreased by 5% for all other states combined, and the increase in charge differential ($53,443) was more than twice the increase ($23,119) for other states combined., Conclusion: Hospitals in California made upward adjustments in nursing FTEs and ratios after enactment of AB 394. There was a substantial increase in California's charge differential, a decrease in SMR, and an increase in SCR after enactment of the legislation.

It is universally agreed that efforts to improve quality benefit from the analysis of outcomes. Yet, it is challenging to compare results across institutions because factors other than performance also impact outcomes. Two factors that complicate the analysis of outcomes after congenital cardiac surgery are case-mix and random statistical variation. Case-mix refers to differences in the mix of patients and their risk-factors at different institutions that may cause some centres to have more frequent complications and lower survival regardless of their true performance. Random statistical variation refers to fluctuations in outcomes that occur at random and follow the laws of probability. A variety of statistical methods exist to address these issues and make provider comparisons more fair. We explain a few common approaches including stratification, regression analysis, and confidence intervals. Concepts are illustrated using artificial data from two hypothetical hospitals, as well as real data from a multi-institution registry.

Meaningful evaluation of quality of care must account for variations in the population of patients receiving treatment, or "case-mix". In adult cardiac surgery, empirical clinical data, initially from tens of thousands, and more recently hundreds of thousands of operations, have been used to develop risk-models, to increase the accuracy with which the outcome of a given procedure on a given patient can be predicted, and to compare outcomes on non-identical patient groups between centres, surgeons and eras. In the adult cardiac database of The Society of Thoracic Surgeons, algorithms for risk-adjustment are based on over 1.5 million patients undergoing isolated coronary artery bypass grafting and over 100,000 patients undergoing isolated replacement of the aortic valve or mitral valve. In the pediatric and congenital cardiac database of The Society of Thoracic Surgeons, 61,014 operations are spread out over greater than 100 types of primary procedures. The problem of evaluating quality of care in the management of pediatric patients with cardiac diseases is very different, and in some ways a great deal more challenging, because of the smaller number of patients and the higher number of types of operations. In the field of pediatric cardiac surgery, the importance of the quantitation of the complexity of operations centers on the fact that outcomes analysis using raw measurements of mortality, without adjustment for complexity, is inadequate. Case-mix can vary greatly from program to program. Without stratification of complexity, the analysis of outcomes for congenital cardiac surgery will be flawed. Two major multi-institutional efforts have attempted to measure the complexity of pediatric cardiac operations: the Risk Adjustment in Congenital Heart Surgery-1 method and the Aristotle Complexity Score. Both systems were derived in large part from subjective probability, or expert opinion. Both systems are currently in wide use throughout the world and have been shown to correlate reasonably well with outcome. Efforts are underway to develop the next generation of these systems. The next generation will be based more on objective data, but will continue to utilize subjective probability where objective data is lacking. A goal, going forward, is to re-evaluate and further refine these tools so that, they can be, to a greater extent, derived from empirical data. During this process, ideally, the mortality elements of both the Aristotle Complexity Score and the Risk Adjustment in Congenital Heart Surgery-1 methodology will eventually unify and become one and the same. This review article examines these two systems of stratification of complexity and reviews the rationale for the development of each system, the current use of each system, the plans for future enhancement of each system, and the potential for unification of these two tools.

Background: This study sought to determine whether associations exist between perioperative glucose exposure, prolonged hospitalization, and morbid events after complex congenital heart surgery., Methods and Results: Metrics of glucose control, including average, peak, minimum, and SD of glucose levels, and duration of hyperglycemia were determined intraoperatively and for 72 hours after surgery for 378 consecutive high-risk cardiac surgical patients. Multivariable regression analyses were used to determine relationships between these metrics of glucose control, hospital length of stay, and a composite morbidity-mortality outcome after controlling for multiple variables known to influence early outcomes after congenital heart surgery. Intraoperatively, a minimum glucose 126 mg/dL) during the 72 postoperative hours was associated with longer duration of hospitalization (P<0.001). In the 72 hours after surgery, average glucose <110 mg/dL (OR, 7.30; 95% CI, 1.95 to 27.25) or >143 mg/dL (OR, 5.21; 95% CI, 1.37 to 19.89), minimum glucose or=250 mg/dL (OR, 2.55; 95% CI, 1.20 to 5.43) were all associated with greater adjusted odds of reaching the composite morbidity-mortality end point., Conclusions: In children undergoing complex congenital heart surgery, the optimal postoperative glucose range may be 110 to 126 mg/dL. Randomized trials of strict glycemic control achieved with insulin infusions in this patient population are warranted.

Objective: No method of measuring technical performance exists for the stage I Norwood procedure. Hospital mortality is usually used as a surrogate for technical performance, but evidence is lacking to support this concept. A technical score was designed by expert consensus., Methods: The technical score included the following steps: (1) Stage I was divided into subprocedures according to anatomic areas where an intervention is performed. (2) For each subprocedure, three score categories (optimal, adequate, and inadequate) were defined on the basis of echocardiography, catheterization, and/or clinical data. (3) Subprocedures were analyzed for the whole group and by surgeon. (4) Overall repair was also scored: optimal if all attempted subprocedures were optimal, inadequate if any was inadequate, and adequate for everything in between. (5) All patients undergoing the stage I procedure from January 2004 to December 2006 were retrospectively studied., Results: One hundred ten patients were included (operated on by six surgeons), and 4 were excluded for lack of reliable postoperative data. Most subprocedures were scored as optimal. Subprocedures with the largest inadequate scores were distal arch reconstruction in 7 (6%) patients and aortopulmonary shunt in 3 (5%). No statistical differences were found among surgeons either by subprocedure or by overall outcome, although individual sample sizes were small. The overall score correlated with length of stay, extracorporeal membrane oxygenator support, and hospital mortality., Conclusions: Technical performance can be measured after the stage I procedure, and performance score correlates with early outcome. This score may also be useful as a self-assessment tool.

Background: The incidence and implications of anti-heparin-platelet factor 4 (PF4) antibody seroconversion in the pediatric cardiac surgical population remain largely unexplored. We sought to prospectively characterize the incidence of seroconversion in two populations: neonates undergoing primary cardiac surgery and children undergoing reoperative cardiac surgery with a history of unfractionated heparin (UFH) exposure., Methods: One hundred and thirty-five consecutive patients were studied: Neonatal = 60 neonates, first time cardiac surgery. Reoperative (ReOp) = 75 children, reoperative cardiac surgery. Preoperative and postoperative day (POD) 5 and 10 blood samples were used to determine the presence of PF4 immunoglobulin (Ig)G, IgA, and IgM antibodies with enzyme-linked immunosorbent assay., Results: No anti-heparin/PF4 antibodies were detected preoperatively in either group. On POD 5, antibodies were present in 1 of 60 (1.7%) Neonatal; and in 12 of 75 (16%) ReOp; P = 0.006. On POD 10, antibodies were present in 1 of 60 (1.7%) Neonatal; and in 39 of 75 (52%) ReOp; P < 0.001. Seroconversion in ReOp patients on POD 10 was significantly associated (P = 0.03) with previous UFH exposures. Heparin-induced thrombocytopenia (HIT) was not diagnosed in any Neonatal patients. One ReOp patient (1.3%) seroconverted and developed HIT without thrombosis or skin lesions., Conclusions: HIT is a rare occurrence in pediatric cardiac surgical patients. The incidence of anti-heparin-PF4 antibody seroconversion in children undergoing reoperation is approximately 50% at 10 days postoperatively, a finding similar to that reported in adult cardiac surgical patients. Both age and previous UFH exposure correlate with this rate of seroconversion. In contrast, the rate of seroconversion in neonates undergoing first time surgery is substantially lower.

Objective: Protein-losing enteropathy (PLE) is a known complication of surgical procedures for congenital heart disease. The pathogenesis and pathophysiology of PLE remain poorly understood. However, lymphatic insufficiency appears central to the disease process. We sought to investigate the role of lymphatic obstruction and central venous catheter-related central venous thrombosis in patients with congenital heart disease and PLE., Design: A case-control study design was constructed consisting of patients with congenital heart disease and PLE and 2:1 matched controls having undergone the same definitive surgical procedure. Obstruction to lymphatic return was considered present if the thoracic duct was ligated, or if there was complete central venous obstruction at the usual site of thoracic duct drainage., Results: Obstruction to lymphatic return was identified in 4 of 16 cases (25%) and 1 of 32 controls (4%), P = .06. There was no association between PLE and central venous catheter use or duration, and no discriminating characteristics between cases and controls with respect to anatomy, pre-Fontan hemodynamic variables, operative or perioperative factors, or hemodynamic variables at the time of PLE diagnosis. Mortality for patients with PLE was 25% compared with 9% in controls (P = not significant). Long-term resolution of PLE was obtained in six patients (38%)., Conclusion: There is a high prevalence of apparent lymphatic obstruction in patients with congenital heart disease and PLE, suggesting that physical lymphatic obstruction may play an important, and previously unrecognized role in the development of PLE in patients with complex congenital heart disease.

There have been comprehensive screening methods developed to identify unwanted inpatient events. A comprehensive assessment of complication diagnoses during congenital heart surgery admissions has not been performed. We examined the frequency of complications identified by a complication screening method and their relationship to patient characteristics among congenital heart surgery admissions. Data were obtained from the Healthcare Cost and Utilization Project Kids' Inpatient Database 2000. Among congenital heart surgery admissions, age < or =20 years, we identified International Classification of Disease, 9th Revision, Clinical Modification (ICD-9-CM) codes indicating complication. Complication diagnoses were categorized as related to drug/biologic, procedures, devices, implants and grafts, and radiation. We used the Risk Adjustment for Congenital Heart Surgery risk categories (1-6) to examine the association between case complexity and complications. Multivariate analyses estimated the odds for a complication diagnosis by patient characteristics, including age, prematurity, chromosomal anomalies, noncardiac structural anomalies, and surgical risk category. Among 12,717 cases, 4014 (32%) had at least 1 complication diagnosis code. Procedure-related complication diagnoses represented 75% of complication diagnoses; device, implant, or graft represented 21%; drug/biologic represented 4% and radiation represented 0%. Multivariate analyses demonstrated that higher surgical case complexity and older age had a greater risk for a complication diagnosis: Risk Category 2, odds ratio (OR) 1.8; 3 OR 2.9; 4 OR 2.9; 5 OR 5.0; 6 OR 4.1, relative to category 1, all p < 0.01; age > or =12 years, OR 1.3, p < 0.001; <1 year OR 1.1, p = 0.31. Premature cases had decreased odds OR 0.4, p < 0.001. This complication screening method indicates that unwanted patient events occur frequently during congenital heart surgery admissions. Children undergoing complex congenital heart surgery are at greatest risk for these unwanted events. Further study of these events is needed to determine their preventability and severity.

Background: In 1997, an effort was made to disseminate US pediatric cardiac surgical practices to create a new comprehensive program in Guatemala. The objective of this study was to describe the improvement of the program by exploring the reduction in risk-adjusted in-hospital mortality., Methods and Results: A retrospective cohort study of surgical procedures performed in Guatemala from February 1997 to July 2004 was conducted. Data were divided into 3 time periods (1997 to 1999, 2000 to 2002, and 2003 to 2004) and compared with a US benchmark (2000 Kids' Inpatient Database of 27 states and 313 institutions). The risk adjustment for congenital heart surgery (RACHS-1) method was used to adjust for case mix. Mortality rates, standardized mortality ratios, and 95% confidence intervals were calculated. A total of 1215 surgical procedures were included. Median age was 3.1 years (range, 1 day to 17.9 years). The overall mortality was 10.7% (n=130). The RACHS-1 method showed better discrimination than in prior reports (area under receiver operating characteristic curve=0.854). A decreasing trend in mortality rate was observed in every RACHS-1 risk category over the 3 time periods. When compared against the US benchmark, the reduction in risk-adjusted mortality was noted by a decrease of standardized mortality ratio from 10.0 (95% confidence interval, 7.2 to 13.7) in 1997-1999, to 7.8 (95% confidence interval, 5.9 to 10.0) in 2000-2002, and to 5.7 (95% confidence interval, 3.8 to 8.3) in 2003-2004., Conclusions: In a short time period, mortality after congenital heart surgery has been reduced substantially in Guatemala. Measurement of risk-adjusted mortality is a useful method to assess pediatric cardiac program improvement in the developing world.

Background: The Institutes of Medicine estimates that patient safety events cost the United States between $17 billion and $29 billion annually. Costs associated with patient safety events or complications among congenital heart surgery admissions are understudied., Objective: To determine the independent contribution of complications on increased resource utilization during congenital heart surgery admissions., Design/methods: Data were obtained from the Healthcare Cost and Utilization Project Kids' Inpatient Database year 2000. Cases of congenital heart surgery <18 years of age were identified. High resource use admissions were defined as admissions above the 90th percentile for total hospital charges. Complications were identified by a method published by the Agency for Healthcare Research Quality using ICD-9-CM codes. Multivariate analyses using generalized estimating equations adjusted for previously identified factors associated with high resource use to estimate the independent effect of a complication on high resource use. These factors include: Risk Adjustment for Congenital Heart Surgery risk categories, age, prematurity, major noncardiac structural anomalies, Medicaid, weekend admission, and state. The added explanatory power attributed to a complication diagnosis variable was measured by an increase in the area under the receiver operator characteristic curve (ROC)., Results: Among 10,602 congenital heart surgery admissions identified, the median total charges were $51,125. The threshold for high resource use was total charges >or=$192,272. High resource admissions accounted for >40% of the total charges for all admissions. Complications were identified in 3360 (32%) of congenital heart surgery admissions. Univariate analysis demonstrated that admissions with at least 1 complication diagnosis were more likely to be a high resource use admission (18% vs. 6%, OR 3.0, P < 0.001). Multivariate analyses revealed that admissions with a complication diagnosis were much more likely to exceed the threshold for high resource utilization (OR 3.2, P < 0.001). The addition of the complication variable to the multivariate model increased the area under the ROC curve from 0.837 to 0.863., Conclusions: Congenital heart surgery admissions with a complication diagnosis are 3 times more likely to exceed $192 272 in total charges despite adjusting for known risk factors for high resource use. Complication reduction may result in both an economic and clinical benefit.

Background: We have previously reported a high rate (32%) of complications among congenital heart surgery admissions. The association among reported complications and other risk factors for mortality during congenital heart surgery admissions has not been assessed., Methods: We identified congenital heart surgery admissions, ages less than 18 years, within the Kids' Inpatient Database 2000, and applied a complication screening method using "International Classification of Disease, 9th Revision, Clinical Modification." Complication diagnoses were classified into four categories, complications due to the following: (1) drugs; (2) procedures; (3) devices, implants, and grafts; and (4) radiation. We examined the independent effect of a complication diagnosis code and complication subcategory on mortality using generalized estimating equations controlling for case-mix using the Risk Adjustment for Congenital Heart Surgery (RACHS-1) method, as well as other previously reported predictors for mortality., Results: Among the 10,032 congenital heart surgical admissions, 32% had at least one complication diagnosis. Seventy-eight percent of complication diagnoses were procedure related, 18% device, implant, or graft related, and 4% drug related. There were no radiation complications identified. After adjusting for case-mix, gender, race, insurance, and hospital surgical volume, admissions with a complication diagnosis had a substantially greater odds of death compared with admissions without a complication (odds ratio [OR] 2.4, p < 0.001). Among complication subcategories, procedure (OR 2.3, p < 0.001), and device, implant, or graft (OR 2.7, p < 0.001) related complications contributed to higher death risk., Conclusions: Complication diagnoses occur frequently during congenital heart surgery admissions and are associated with an increased risk of death even after controlling for other known mortality risk factors. Identification of complication diagnoses is an important first step. Subsequent efforts must further examine these events to determine their degree of preventability and develop strategies to reduce them. Such an effort may reduce death in this vulnerable population. This study represents preliminary work in an effort to improve outcomes in this complex patient population.

Background: There has been little research about the causes of death after congenital heart surgery., Methods: To determine whether mode of death differs after congenital heart surgery, we evaluated the cause of death for 100 consecutive postoperative deaths at our institution. Mode of death was determined based on retrospective chart review including available autopsy reports. Low output states were categorized into ventricular failure; inadequate postoperative physiology (technically adequate surgery and ventricular function, but persistent low cardiac output); pulmonary hypertension; and atrioventricular valve regurgitation., Results: There was considerable anatomic diversity among patients who died; 46 patients had single-ventricle physiology. The vast majority of patients (n = 79) were in the intensive care unit before surgery. Surgical repairs were revised at initial operation in 22 cases; 7 patients died in the operating room. Seventy-three patients had technically adequate surgical procedures, 23 had residual anatomic defects, and 4 were indeterminate. Thirty patients underwent additional surgical and 9 catheter-based procedures, although some were classified as rescue procedures performed to address minor anatomic or physiologic abnormalities as a last hope to rescue the patient from impending demise. Of 100 deaths, most (n = 52) were due to low cardiac output: 24 inadequate postoperative physiology, 19 ventricular failure, 8 pulmonary hypertension, and 1 valvar regurgitation. Other significant causes of death included sudden cardiac arrest (n = 11), sepsis (n = 11), and procedural complications (n = 8)., Conclusions: More than half of the deaths were due to low cardiac output, but not exclusively ventricular failure.

Objective: The proposed physiologic advantage of the modified Norwood procedure using a right ventricle-pulmonary artery conduit to supply pulmonary blood flow, compared with a modified Blalock-Taussig shunt, is reduced runoff from the systemic-to-pulmonary circulation during diastole, resulting in a higher diastolic blood pressure and improved systemic perfusion. We hypothesized that the modified Norwood procedure is associated with improved cerebral perfusion and oxygenation., Methods: Transcranial Doppler sonography and near-infrared spectroscopy were performed in neonates undergoing the Norwood procedure with either a modified Blalock-Taussig shunt (n = 14) or right ventricle-pulmonary artery conduit (n = 13)., Results: Diastolic blood pressure was significantly higher in the right ventricle-pulmonary artery group at 6 hours after bypass (46 +/- 7 vs 40 +/- 4 mm Hg; P = .03), on postoperative day 1 (45 +/- 6 vs 37 +/- 5 mm Hg; P = .002), and on postoperative day 2 (46 +/- 7 vs 37 +/- 4 mm Hg; P = .001). Cerebral diastolic blood flow velocity did not differ significantly between groups at any time point or over time, but cerebral systolic blood flow velocity was higher over time in the Blalock-Taussig group (P = .01). No significant differences in regional cerebral oxygen saturation were found between groups at baseline or after bypass. Blood flow velocities and cerebral oxygen saturation did not differ significantly according to use of regional low-flow perfusion., Conclusions: The higher diastolic blood pressure after the modified Norwood procedure is not associated with higher cerebral blood flow velocities or regional cerebral oxygen saturation. This may imply an equal vulnerability to the cerebral injury associated with hemodynamic instability in the early postoperative period.

Background: Although adequacy of repair after congenital heart surgery is a crucial determinant of clinical outcome, there is no current method of assessment. We sought to develop a process to measure the adequacy of repair for a diverse group of congenital heart procedures., Methods: Selected surgical procedures, consisting of repair of ventricular septal defect (VSD), tetralogy of Fallot (TOF), complete common atrioventricular canal (CAVC), and arterial switch operation, were divided into component subprocedures, each of which was assessed separately. Three outcome categories of "optimal," "adequate," and "inadequate" were defined by consensus according to postprocedure echocardiographic assessment. Outcome categories for conduction disturbance were also created. All patients undergoing one of the four procedures in 2004 were identified, and each subprocedure was assessed. Other clinical data were obtained from medical records. Repairs were scored as "optimal" if all attempted subprocedures and conduction were optimal, and "inadequate" if any was inadequate., Results: A total of 138 procedures were included. VSD repair was done in 46 patients (33%), TOF repair in 33 (24%), arterial switch operation in 36 (26%), and CAVC repair in 23 (17%). Optimal technical score was found in 28 (20%), adequate in 106 (77%), and inadequate in 4 (3%) (2 VSD, 1 TOF, 1 CAVC). Median length of stay was 8 days, and no patients died., Conclusions: Despite procedural diversity and complexity, technical adequacy of repair can be assessed for congenital heart surgery.