Search

The World Journal for Pediatric and Congenital Heart Surgery ( WJPCHS ) was established in 2009, as a means of advancing the educational and scholarship goals of the World Society for Pediatric and Congenital Heart Surgery. WJPCHS has grown steadily since the first issue was published in April 2010. In 2017, the Congenital Heart Surgeons' Society and the European Congenital Heart Surgeons Association both designated WJPCHS as the official journal of their respective organizations. The CHSS and ECHSA represent the face and the voice of congenital heart surgery in North America (United States and Canada) and in Europe, respectively. Each organization has advanced the science of surgical management of congenital heart disease through multicenter outcomes analyses, which have strongly and positively influenced the care of patients around the world.

Objectives: Airway anomalies are common in children with cardiac disease but with an unquantified impact on outcomes. We sought to define the association between airway anomalies and tracheal surgery with cardiac surgery outcomes using the Society of Thoracic Surgery Congenital Heart Surgery Database., Methods: Index cardiac operations in children aged less than 18 years (January 2010 to September 2018) were identified from the Society of Thoracic Surgery Congenital Heart Surgery Database. Patients were divided on the basis of reported diagnosis of an airway anomaly and subdivided on the basis of tracheal lesion and tracheal surgery. Multivariable analysis evaluated associations between airway disease and outcomes controlling for covariates from the Society of Thoracic Surgery Congenital Heart Surgery Database Mortality Risk Model., Results: Of 198,674 index cardiovascular operations, 6861 (3.4%) were performed in patients with airway anomalies, including 428 patients (0.2%) who also underwent tracheal operations during the same hospitalization. Patients with airway anomalies underwent more complex cardiac operations (45% vs 36% Society of Thoracic Surgeons/European Association for Cardiothoracic Surgery Congenital Heart Surgery Mortality category ≥3 procedures) and had a higher prevalence of preoperative risk factors (73% vs 39%; both P < .001). In multivariable analysis, patients with airway anomalies had increased odds of major morbidity and tracheostomy (P < .001). Operative mortality was also increased in patients with airway anomalies, except those with malacia. Tracheal surgery within the same hospitalization increased the odds of operative mortality (adjusted odds ratio, 3.9; P < .0001), major morbidity (adjusted odds ratio, 3.7; P < .0001), and tracheostomy (adjusted odds ratio, 16.7; P < .0001)., Conclusions: Patients undergoing cardiac surgery and tracheal surgery are at significantly higher risk of morbidity and mortality than patients receiving cardiac surgery alone. Most of those with unoperated airway anomalies have higher morbidity and mortality, which makes it an important preoperative consideration., (Copyright © 2020 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.)

Background: The Society of Thoracic Surgeons Congenital Heart Surgery Database Mortality Risk Model adjusts not only for procedure and age group pairings but also for additional patient factors, including the binary presence or absence of a chromosomal abnormality (CA), syndrome (S), and/or a noncardiac congenital anatomic abnormality (NCAA). This analysis refines case-mix adjustment by adding more granular adjustment for individual conditions (CA, S, and NCAA), consistent with a hypothesis that associated risk of mortality differs between individual conditions., Methods: CA/S corresponding to the same condition were merged to a single condition code. Odds ratios were estimated for all CA/S. For CA/S associated with at least 10 deaths in neonates and infants and at least 10 deaths in children and adults, odds ratios were estimated for the effect of the CA/S separately in neonates/infants and in children/adults. In addition to these condition/age interactions, condition/age/procedure interactions were explored (eg, effect of Down syndrome was estimated based on age and procedure subgroups, including atrioventricular canal repair and single-ventricle palliation). Bayesian modeling was used to create 5 maximally homogeneous groups of CA/S from 81 candidate CA/S variables. A standard logistic regression model then incorporated indicator variables for the 5 categories of CAs/Ss, 7 unique NCAAs, and all other covariates in the previously published Society of Thoracic Surgeons Congenital Heart Surgery Database Mortality Model., Results: Analysis included 107,062 operations in 100 centers (2010 to 2015). Operative Mortality was 3,629 (3.4%). In the development sample, the C statistics of the original nonaugmented model and the augmented model were 0.872 and 0.875, respectively., Conclusions: The Society of Thoracic Surgeons Congenital Heart Surgery Database Mortality Risk Model has been augmented by addition of covariates representing individual CAs, Ss, and NCAAs., (Copyright © 2019 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: For neonates with critical left ventricular outflow tract obstruction (LVOTO), hybrid procedures are an alternative to the Norwood stage 1 procedure. Despite perceived advantages, however, outcomes are not well defined. Therefore, we compared outcomes after stage 1 hybrid and Norwood procedures., Methods: In a critical LVOTO inception cohort (2005-2014; 20 institutions), a total of 564 neonates underwent stage 1 palliation with the Norwood operation with a modified Blalock-Taussig shunt (NW-BT; n = 232; 41%), Norwood operation with a right ventricle-to-pulmonary artery conduit (NW-RVPA; n = 222; 39%), or a hybrid procedure (n = 110; 20%). Post-stage 1 outcomes were analyzed via competing-risks and parametric hazard analyses and compared among all 564 patients and between patients who underwent propensity-matched hybrid and those who underwent NW-BT/NW-RVPA., Results: By 6 years after the stage 1 operation, 50% ± 3%, 7% ± 2%, and 4% ± 1% of patients transitioned to Fontan, transplantation, and biventricular repair, respectively, whereas 7% ± 2% were alive without transition and 32% ± 2% died. Risk factors for death without transition included procedure type, smaller ascending aorta, aortic valve atresia, and lower birth weight. Risk-adjusted 4-year survival was better after NW-RVPA than after NW-BT or hybrid (76% vs 60% vs 61%; P < .001). Furthermore, for neonates with lower birth weight (<∼2 kg), an interaction between birth weight and hybrid resulted in a trend toward better survival after hybrid compared with NW-BT or NW-RVPA. For propensity-matched neonates between hybrid and NW-BT (88 pairs), 4-year survival was similar (62% vs 57%; P = .58). For propensity-matched neonates between hybrid and NW-RVPA (81 pairs), 4-year survival was better after NW-RVPA (59% vs 75%; P = .008)., Conclusions: For neonates with critical LVOTO undergoing single-ventricle palliation, NW-RVPA was associated with the best overall survival. Hybrid strategies are not a lower-risk alternative to Norwood operations overall; however, the impact of lower birth weight on survival may be mitigated after hybrid procedures compared with Norwood operations., (Copyright © 2016 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.)

Objective: Hybrid palliation is an alternative to Norwood stage 1 for the initial management of hypoplastic left heart syndrome. Contemporary multicenter hybrid use and institutional/patient factors associated with hybrid use relative to the Norwood have not been evaluated. We describe hybrid use in relation to institutional volume, patient factors, and short-term outcomes., Methods: Infants aged 60 days or less listed in The Society of Thoracic Surgeons Congenital Heart Surgery Database (2010-2012) undergoing initial palliation of hypoplastic left heart syndrome were included. Annual institutional hybrid use rates were calculated: [hybrid procedures/(Norwood + hybrid + transplant procedures)]. In-hospital outcomes for primary hybrid and primary Norwood were compared and stratified by high (defined as ≥50%) versus low (defined as ≤10%) institutional hybrid use., Results: Of 1728 patients (100 centers), most (n = 1496, 87%) underwent an index Norwood; 232 patients (13%) underwent an index hybrid procedure. Preoperative patient risk factors were more prevalent in patients undergoing the hybrid procedure. Only 13 of 100 institutions were high hybrid users, and these tended to have lower annual hypoplastic left heart syndrome index case volume. Unadjusted in-hospital mortality was higher for the hybrid compared with the Norwood procedure (30% vs 16%; P < .001). In-hospital mortality for the hybrid procedure was not associated with hybrid use (26% among institutions with low use vs 28% among institutions with high use). However, centers with high hybrid use had higher mortality after the Norwood (43%) compared with centers with low hybrid use (16%)., Conclusions: Few centers currently select the hybrid procedure for most infants with hypoplastic left heart syndrome. Although unadjusted in-hospital hybrid mortality is higher than Norwood mortality, potential risk factors are more prevalent among hybrid cases. Institutions with higher hybrid use have lower hypoplastic left heart syndrome case volume and higher Norwood mortality., (Copyright © 2015 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.)

Objectives: Outcomes evaluation is enhanced by assignment of operative procedures to appropriate categories based upon relative average risk. Formal risk modelling is challenging when a large number of operation types exist, including relatively rare procedures. Complexity stratification provides an alternative methodology. We report the initial application in the Congenital Heart Surgery Databases of the Society of Thoracic Surgeons (STS) and the European Association for Cardio-thoracic Surgery (EACTS) of an empirically derived system of complexity adjustment to evaluate surgical case mix and results., Methods: Complexity stratification is a method of analysis in which the data are divided into relatively homogeneous groups (called strata). A complexity stratification tool named the STS-EACTS Congenital Heart Surgery Mortality Categories (STAT Mortality Categories) was previously developed based on the analysis of 77,294 operations entered in the Congenital Heart Surgery Databases of EACTS (33,360 operations) and STS (43,934 patients). Procedure-specific mortality rate estimates were calculated using a Bayesian model that adjusted for small denominators. Operations were sorted by increasing risk and grouped into five categories (the STAT Mortality Categories) that were designed to minimize within-category variation and maximize between-category variation. We report here the initial application of this methodology in the EACTS Congenital Heart Surgery Database (47,187 operations performed over 4 years: 2006-09) and the STS Congenital Heart Surgery Database (64,307 operations performed over 4 years: 2006-09)., Results: In the STS Congenital Heart Surgery Database, operations classified as STAT Mortality Categories 1-5 were (1): 17332, (2): 20114, (3): 9494, (4): 14525 and (5): 2842. Discharge mortality was (1): 0.54%, (2): 1.6%, (3): 2.4%, (4): 7.5% and (5): 17.8%. In the EACTS Congenital Heart Surgery Database, operations classified as STAT Mortality Categories 1-5 were (1): 19874, (2): 12196, (3): 5614, (4): 8287 and (5): 1216. Discharge mortality was (1): 0.99%, (2): 2.9%, (3): 5.0%, (4): 10.3% and (5): 25.0%., Conclusions: The STAT Mortality Categories facilitate analysis of outcomes across the wide spectrum of distinct congenital heart surgery operations including infrequently performed procedures.

Objective: Multiple subsequent procedures directed at the arch and/or the left ventricular outflow tract are frequently required after interrupted aortic arch repair. We the investigated patterns and factors associated with these subsequent procedures and mortality., Methods: We reviewed the data from 447 patients with interrupted aortic arch at 33 institutions enrolled from 1987 to 1997. We classified the subsequent procedures by type (catheter-based or surgical) and focus (arch, left ventricular outflow tract, and "other" cardiovascular lesions). We used competing risks and modulated renewal analysis to explore subsequent procedures., Results: There were 158 subsequent arch and 100 left ventricular outflow tract procedures. Freedom from death at 21 years was 60% overall. The risk of additional subsequent arch procedures decreased after the first subsequent arch procedure in the acute phase, but did not significantly change in the chronic phase. The risk of additional subsequent left ventricular outflow tract procedures increased after the first subsequent left ventricular outflow tract procedure in the chronic phase. The risk factors for subsequent arch procedures and mortality, but not for subsequent outflow track procedures, were related in a complex way to previous procedures and their timing., Conclusions: Interrupted aortic arch is a chronic disease in which patients often undergo multiple subsequent procedures with persistent risk for additional intervention and mortality. The risk factors are related to the nature and timing of previous procedures and to the morphology and details of the index procedure. Interrupted aortic arch should be considered a chronic disorder., (Copyright © 2010 The American Association for Thoracic Surgery. Published by Mosby, Inc. All rights reserved.)