Search

Purpose: We reviewed all 64 articles ever published by The Congenital Heart Surgeons' Society (CHSS) Data Center to estimate the academic impact of these peer-reviewed articles., Materials and Methods: The Congenital Heart Surgeons' Society has performed research based on 12 Diagnostic Inception Cohorts. The first cohort (Transposition) began enrolling patients on January 1, 1985. We queried PubMed to determine the number of publications that referenced each of the 64 journal articles generated by the datasets of the 12 Diagnostic Inception Cohorts that comprise the CHSS Database. Descriptive summaries of the data were tabulated using mean with standard deviation and median with range., Results: Sixty-four peer-reviewed papers have been published based on the CHSS Database. Fifty-nine peer-reviewed articles have been published based on the 12 Diagnostic Inception Cohorts, and five additional articles have been published based on Data Science. Excluding the recently established Diagnostic Inception Cohort for patients with Ebstein malformation of tricuspid valve, the number of papers published per cohort ranged from 1 for coarctation to 11 for transposition of the great arteries. The 11 articles generated from the CHSS Transposition Cohort were referenced by a total of 111 articles (median number of references per journal article = 9 [range = 0-22, mean = 10.1]). Overall, individual articles were cited by an average of 11 (mean), and a maximum of 41 PubMed-listed publications. Overall, these 64 peer-reviewed articles based on the CHSS Database were cited 692 times in PubMed-listed publications. The first CHSS peer-reviewed article was published in 1987, and during the 35 years from 1987 to 2022, inclusive, the annual number of CHSS publications has ranged from 0 to 7, with a mean of 1.8 publications per year (median = 1, mode = 1)., Conclusion: Congenital Heart Surgeons' Society studies are widely referenced in the pediatric cardiac surgical literature, with over 10 citations per published article. These cohorts provide unique information unavailable in other sources of data. A tool to access this analysis is available at: [https://data-center.chss.org/multimedia/files/2022/CAI.pdf].

Objectives: During congenital heart surgery, the pulmonary valve and root may be placed into the systemic position, yielding a "neoaortic" valve. With the stress of systemic pressure, the pulmonary roots can dilate, creating aneurysms and/or neoaortic insufficiency (neoAI). This report analyzes the early outcomes of patients undergoing neoaortic valve repair incorporating geometric ring annuloplasty., Methods: Twenty-one patients underwent intended repair at six centers and formed the study cohort. Thirteen had previous Ross procedures, five had arterial switch operations, and three Fontan physiology. Average age was 21.7 ± 12.8 years (mean  ±  SD), 80% were male, and 11 (55%) had symptomatic heart failure. Preoperative neoAI Grade was 3.1  ±  1.1, and annular diameter was 30.7  ±  6.5 mm., Results: Valve repair was accomplished in 20/21, using geometric annuloplasty rings and leaflet plication (n = 13) and/or nodular release (n = 7). Fourteen had neoaortic aneurysm replacement (13 with root remodeling). Two underwent bicuspid valve repair. Six had pulmonary conduit changes, one insertion of an artificial Nodulus Arantius, and one resection of a subaortic membrane. Ring size averaged 21.9  ±  2.3 mm, and aortic clamp time was 171  ±  54 minutes. No operative mortality or major morbidity occurred, and postoperative hospitalization was 4.3  ±  1.4 days. At discharge, neoAI grade was 0.2  ±  0.4 ( P  < .0001), and valve mean gradient was ≤20 mm Hg. At average 18.0  ±  9.1 months of follow-up, all patients were asymptomatic with stable valve function., Conclusions: Neoaortic aneurysms and neoAI are occasionally seen late following Ross, arterial switch, or Fontan procedures. Neoaortic valve repair using geometric ring annuloplasty, leaflet reconstruction, and root remodeling provides a patient-specific approach with favorable early outcomes.

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.)

Objective: Arch obstruction after the Norwood procedure is common and contributes to mortality. We determined the prevalence, associated factors, and practice variability of arch reintervention and assessed whether arch reintervention is associated with mortality., Methods: From 2005 to 2017, 593 neonates in the Congenital Heart Surgeons' Society Critical Left Heart Obstruction cohort underwent a Norwood procedure. Median follow-up was 3.7 years. Multivariable parametric models, including a modulated renewal analysis, were performed., Results: Of the 593 neonates, 146 (25%) underwent 218 reinterventions for arch obstruction after the Norwood procedure: catheter-based (n = 168) or surgical (n = 50) at a median age of 4.3 months (quartile 1-quartile 3, 2.6-5.7). Interdigitation of the distal aortic anastomosis was protective against arch reintervention. Development of ≥ moderate tricuspid valve regurgitation and right ventricular dysfunction at any point was associated with arch reintervention. Nonsignificant variables for arch reintervention included shunt type and preoperative aortic measurements. Surgical arch reintervention was protective against arch reintervention, but transcatheter reintervention was associated with increased reintervention. Arch reintervention was not associated with increased mortality. There was wide institutional variation in incidence of arch reintervention (range, 0-40 reinterventions per 100 years patient follow-up) and in preintervention gradient (range, 0-64 mm Hg)., Conclusions: Interdigitation of the distal aortic anastomosis during the Norwood procedure decreased the risk of arch reintervention. Surgical arch reintervention is more definitive than transcatheter. Arch reintervention after the Norwood procedure is not associated with increased mortality. Serial surveillance for arch obstruction, integrated with changes in right ventricular function and tricuspid valve regurgitation, is recommended after the Norwood procedure to improve outcomes., (Copyright © 2018 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.)

Objective: Patient-based congenital heart surgery (CHS) training is opportunity-based and difficult. Three-dimensional (3D) print models of the heart were used for hands-on surgical training (HOST) at the 2015 AATS and subsequently in 2 local institutions. We aim to introduce the process of 3D printing for surgical simulation and to present the attendee's responses., Methods: Using CT or MR angiograms, the models of congenital heart disease were created and printed with flexible rubberlike material. Altogether, 81 established surgeons or trainees performed simulated surgical procedures with the expert surgeons' guidance and supervision. At the completion of the session, 50 of 81 attendees participated in the questionnaire assessment of the program., Results: All responders found the course helpful in improving their surgical skills. All would consider including HOST sessions in the training programs. All found that the models showed the necessary pathologic findings. Most found that the consistency and elasticity of the model material were different from those of the human myocardium. However, the responders thought that the quality of the models was acceptable (88%) or manageable (12%) for surgical practice. The major weaknesses listed were related to the print material and poor representation of the cardiac valves., Conclusions: HOST using 3D print heart models is achievable and allows surgical practice on pathological hearts without patients' risk. HOST is a highly applicable surgical simulation format for CHS. Incorporation of HOST in training programs could change the traditional opportunity-based education to the requirement-based standardized education., (Copyright © 2017 The American Association for Thoracic Surgery. All rights reserved.)

Evidence is accumulating that blood flow patterns in the cardiovascular system and in cardiovascular devices do, in some instances, depend on blood viscoelasticity. Thus, to better understand the challenges to providing circulatory support and surgical therapies for pediatric and adult patients, viscous and elastic components of complex blood viscoelasticity of 31 pediatric patients were compared to those of 29 adult patients with a Vilastic-3 rheometer. A random effects model with categorical age covariates found statistically significant differences between pediatric and adult patients for log viscosity (p = 0.005). Log strain (p < 0.0001) and hematocrit (p < 0.0001) effects were also significant, as were the hematocrit-by-log-strain (p = 0.0006) and age-by-log strain (p = 0.001) interactions. The hematocrit-by-age interaction was not significant. For log elasticity, age differences were insignificant (p = 0.39). The model for log elasticity had significant log strain (p < 0.0001), log strain squared (p < 0.0001) and hematocrit (p < 0.0001) effects, as well as hematocrit-by-log-strain and hematocrit-by-log-strain-squared interactions (p = 0.014). A model for log viscosity with continuous age was also fit to the data, which can be used to refine cardiovascular device design and operation to the age of the patient. We conclude that there are distinct differences between pediatric and adult blood viscosity, as well as substantial variation within the pediatric population, that may impact the performance of devices and procedures.

The Society of Thoracic Surgeons Congenital Heart Surgery Database is the largest congenital and pediatric cardiac surgical clinical data registry in the world. It is the platform for all activities of The Society of Thoracic Surgeons related to the analysis of outcomes and the improvement of quality in this subspecialty. This report summarizes current aggregate national outcomes in congenital and pediatric cardiac surgery and reviews related activities in the areas of quality measurement, performance improvement, and transparency. The reported data about aggregate national outcomes are exemplified by an analysis of 10 benchmark operations performed from January 2012 to December 2015. This analysis documents the overall aggregate operative mortality (interquartile range among all participating programs) for the following procedural groups: off-bypass coarctation repair, 1.3% (0.0% to 1.8%); ventricular septal defect repair, 0.6% (0.0% to 0.9%); tetralogy of Fallot repair, 1.1% (0.0% to 1.4%); complete atrioventricular canal repair, 3.0% (0.0% to 4.7%); arterial switch operation, 2.7% (0.0% to 4.1%); arterial switch operation and ventricular septal defect repair, 5.3% (0.0% to 6.7%); Glenn/hemi-Fontan, 2.5% (0.0% to 4.5%); Fontan operation, 1.2% (0.0% to 1.2%); truncus arteriosus repair, 9.4% (0.0% to 16.7%); and Norwood procedure, 15.7% (8.9% to 25.0%)., (Copyright © 2017 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: Previous analyses of The Society of Thoracic Surgeons (STS) Adult Cardiac Surgery Database have demonstrated a reduction over time of risk-adjusted operative mortality after coronary artery bypass grafting. The STS Congenital Heart Surgery Database (STS CHSD) was queried to assess multiinstitutional trends over time in discharge mortality and postoperative length of stay (PLOS)., Methods: Since 2009, operations in the STS CHSD have been classified according to STAT (The Society of Thoracic Surgeons-European Association for Cardio-Thoracic Surgery) Congenital Heart Surgery Mortality Categories. The five STAT Mortality Categories were chosen to be optimal with respect to minimizing variation within categories and maximizing variation between categories. For this study, all index cardiac operations from 1998 to 2014, inclusive, were grouped by STAT Mortality Category (exclusions: patent ductus arteriosus ligation in patients weighing less than or equal to 2.5 kg and operations that could not be assigned to a STAT Mortality Category). End points were discharge mortality and PLOS in survivors for the entire period and for 4-year epochs. The Cochran-Armitage trend test was used to test the null hypothesis that the mortality was the same across epochs, by STAT Mortality Category., Results: The analysis encompassed 202,895 index operations at 118 centers. The number of centers participating in STS CHSD increased in each epoch. Overall discharge mortality was 3.4% (6,959 of 202,895) for 1998 to 2014 and 3.1% (2,308 of 75,337) for 2011 to 2014. Statistically significant improvement in discharge mortality was seen in STAT Mortality Categories 2, 3, 4, and 5 (p values for STAT Mortality Categories 1 through 5 are 0.060, <0.001, 0.015, <0.001, and <0.001, respectively). PLOS in survivors was relatively unchanged over the same time intervals. Sensitivity analyses reveal that the finding of declining risk-stratified rates of discharge mortality over time is not simply attributable to the addition of more centers to the cohort over time., Conclusions: This 16-year analysis of STS CHSD reveals declining discharge mortality over time, especially for more complex operations., (Copyright © 2016 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

The Society of Thoracic Surgeons Congenital Heart Surgery Database is the largest congenital and pediatric cardiac surgical clinical data registry in the world. It is the platform for all activities of The Society of Thoracic Surgeons related to the analysis of outcomes and the improvement of quality in this subspecialty. This article summarizes current aggregate national outcomes in congenital and pediatric cardiac surgery and reviews related activities in the areas of quality measurement, performance improvement, and transparency. The reported data about aggregate national outcomes are exemplified by an analysis of 10 benchmark operations performed from January 2011 to December 2014 and documenting overall discharge mortality (interquartile range among programs with more than 9 cases): off-bypass coarctation, 1.0% (0.0% to 0.9%); ventricular septal defect repair, 0.7% (0.0% to 1.1%); tetralogy of Fallot repair, 1.0% (0.0% to 1.7%); complete atrioventricular canal repair, 3.2% (0.0% to 6.5%); arterial switch operation, 2.7% (0.0% to 5.6%); arterial switch operation plus ventricular septal defect, 5.3% (0.0% to 6.7%); Glenn/hemiFontan, 2.1% (0.0% to 3.8%); Fontan operation, 1.4% (0.0% to 2.4%); truncus arteriosus repair, 9.6% (0.0 % to 11.8%); and Norwood procedure, 15.6% (10.0% to 21.4%)., (Copyright © 2016 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: The empirically derived 2014 Society of Thoracic Surgeons Congenital Heart Surgery Database Mortality Risk Model incorporates adjustment for procedure type and patient-specific factors. The purpose of this report is to describe this model and its application in the assessment of variation in outcomes across centers., Methods: All index cardiac operations in The Society of Thoracic Surgeons Congenital Heart Surgery Database (January 1, 2010, to December 31, 2013) were eligible for inclusion. Isolated patent ductus arteriosus closures in patients weighing less than or equal to 2.5 kg were excluded, as were centers with more than 10% missing data and patients with missing data for key variables. The model includes the following covariates: primary procedure, age, any prior cardiovascular operation, any noncardiac abnormality, any chromosomal abnormality or syndrome, important preoperative factors (mechanical circulatory support, shock persisting at time of operation, mechanical ventilation, renal failure requiring dialysis or renal dysfunction (or both), and neurological deficit), any other preoperative factor, prematurity (neonates and infants), and weight (neonates and infants). Variation across centers was assessed. Centers for which the 95% confidence interval for the observed-to-expected mortality ratio does not include unity are identified as lower-performing or higher-performing programs with respect to operative mortality., Results: Included were 52,224 operations from 86 centers. Overall discharge mortality was 3.7% (1,931 of 52,224). Discharge mortality by age category was neonates, 10.1% (1,129 of 11,144); infants, 3.0% (564 of 18,554), children, 0.9% (167 of 18,407), and adults, 1.7% (71 of 4,119). For all patients, 12 of 86 centers (14%) were lower-performing programs, 67 (78%) were not outliers, and 7 (8%) were higher-performing programs., Conclusions: The 2014 Society of Thoracic Surgeons Congenital Heart Surgery Database Mortality Risk Model facilitates description of outcomes (mortality) adjusted for procedural and for patient-level factors. Identification of low-performing and high-performing programs may be useful in facilitating quality improvement efforts., (Copyright © 2015 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Objectives: The objectives of this manuscript are two-fold: (a) to describe the clinical characteristics and management of four pediatric patients with bacterial endocarditis (BE) after Melody pulmonary valve implantation (MPVI); and (b) to review the literature regarding Melody pulmonary valve endocarditis., Background: There are several reports of BE following MPVI. The clinical course, BE management and outcome remain poorly defined., Methods: This is a multi-center report of four pediatric patients with repaired tetralogy of Fallot (TOF) and BE after MPVI. Clinical presentation, echocardiogram findings, infecting organism, BE management, and follow-up assessment are described. We review available literature on Melody pulmonary valve endocarditis and discuss the prognosis and challenges in the management of these patients., Results: Of our four BE patients, two had documented vegetations and three showed worsening pulmonary stenosis. All patients remain asymptomatic after medical treatment (4) and surgical prosthesis replacement (3) at follow-up of 17 to 40 months. Analysis of published data shows that over half of patients undergo bioprosthesis explantation and that there is a 13% overall mortality. The most common BE pathogens are the Staphylococcus and Streptococcus species., Conclusions: Our case series of four pediatric patients with repaired TOF confirms a risk for BE after MPVI. A high index of suspicion for BE should be observed after MPVI. All patients should be advised to follow lifelong BE prophylaxis after MPVI. In case of BE, surgery should be considered for valve dysfunction or no clinical improvement in spite of medical treatment., (© 2014 Wiley Periodicals, Inc.)

Objectives: Analyses of mechanical circulatory support (MCS) in pediatric heart surgery have primarily focused on single-center outcomes or narrow applications. We describe the patterns of use, patient characteristics, and MCS-associated outcomes across a large multicenter cohort., Methods: Patients (aged <18 years) in the Society of Thoracic Surgeons (STS) Congenital Heart Surgery Database (2000-2010) were included. The characteristics and outcomes of those receiving postoperative MCS were described, and bayesian hierarchical models were used to examine variations in the adjusted MCS rates across institutions., Results: Of 96,596 operations (80 centers), MCS was used in 2.4%. The MCS patients were younger (13 vs 195 days, P < .0001) and more often had STS-defined preoperative risk factors (57.2% vs 32.7%, P < .0001). The operations with the greatest MCS rates included the Norwood procedure (17%) and complex biventricular repairs (arterial switch, ventricular septal defect, and arch repair [14%]). More than one half of the MCS patients did not survive to hospital discharge (53.2% vs 2.9% of non-MCS patients; P < .0001). MCS-associated mortality was greatest for truncus arteriosus and Ross-Konno operations (both 71%). The hospital-level MCS rates adjusted for patient characteristics and case mix varied by 15-fold across institutions, with both high- and low-volume hospitals having substantial variation in MCS rates., Conclusions: Perioperative MCS use varied widely across centers. The MCS rates were greatest overall for the Norwood procedure and complex biventricular repairs. Although MCS can be a life-saving therapy, more than one half of MCS patients will not survive to hospital discharge, with mortality >70% for some operations. Future studies aimed at better understanding the appropriate indications, optimal timing, and management of MCS could help to reduce the variation in MCS use across hospitals and improve outcomes., (Copyright © 2014 The American Association for Thoracic Surgery. Published by Mosby, Inc. All rights reserved.)

Objectives: Despite progress with adult ventricular assist devices, limited options exist to support pediatric patients with life-threatening heart disease. Extracorporeal membrane oxygenation remains the clinical standard. To characterize (patho)physiologic responses to different modes of mechanical unloading of the failing pediatric heart, extracorporeal membrane oxygenation was compared to intra-aortic balloon pump, pulsatile-flow ventricular assist device, or continuous-flow ventricular assist device support in a pediatric heart failure model., Design: Experimental., Setting: Large animal laboratory operating room., Subjects: Yorkshire piglets (n = 47; 11.7 ± 2.6 kg)., Interventions: In piglets with coronary ligation-induced cardiac dysfunction, mechanical circulatory support devices were implanted and studied during maximum support., Measurements and Main Results: Left ventricular, right ventricular, coronary, carotid, systemic arterial, and pulmonary arterial hemodynamics were measured with pressure and flow transducers. Myocardial oxygen consumption and total-body oxygen consumption were calculated from arterial, venous, and coronary sinus blood sampling. Blood flow was measured in 17 organs with microspheres. Paired Student t tests compared baseline and heart failure conditions. One-way repeated-measures analysis of variance compared heart failure, device support mode(s), and extracorporeal membrane oxygenation. Statistically significant (p < 0.05) findings included 1) an improved left ventricular blood supply/demand ratio during pulsatile-flow ventricular assist device, continuous-flow ventricular assist device, and extracorporeal membrane oxygenation but not intra-aortic balloon pump support, 2) an improved global myocardial blood supply/demand ratio during pulsatile-flow ventricular assist device and continuous-flow ventricular assist device but not intra-aortic balloon pump or extracorporeal membrane oxygenation support, and 3) diminished pulsatility during extracorporeal membrane oxygenation and continuous-flow ventricular assist device but not intra-aortic balloon pump and pulsatile-flow ventricular assist device support. A profile of systems-based responses was established for each type of support., Conclusions: Each type of pediatric ventricular assist device provided hemodynamic support by unloading the heart with a different mechanism that created a unique profile of physiological changes. These data contribute novel, clinically relevant insight into pediatric mechanical circulatory support and establish an important resource for pediatric device development and patient selection.

Background: There are a wide variety of reported techniques with few comparative trials and no current data available by which surgeons can compare their myopreservation strategies across the specialty. We therefore surveyed congenital heart surgeons to develop a profile of current practice., Methods: One hundred twenty-two members of the Congenital Heart Surgeons' Society were surveyed, and 56 responses were analyzed. The survey focused on cardioplegia formulations, dosage and administration, and perfusion strategies for four age groups: neonates, infants, children, and adolescents. All percentages are expressed as percentage of the entire reporting cohort (n=56)., Results: Eighty-six percent of surgeons use blood-based cardioplegia versus crystalloid cardioplegia. Microplegia is used in 5%. Blood-based cardioplegia additives include del Nido (38%), customized solutions (32%), St. Thomas, Plegisol, or Baxter (11%), and microplegia (5%). Crystalloid cardioplegia types are Custodiol (7%), St. Thomas, Plegisol, or Baxter (5%), and customized solutions (2%). Cold (<10°C) cardioplegia is most common (93%), and "hot shots" are used in 21%. Moderate (26° to 30°C) hypothermic cardiopulmonary bypass is more common in neonates and infants compared with older children and adolescents. Antegrade administration is most common (89%). Longer intervals between cardioplegia doses were associated with surgeons using del Nido and Custodiol solutions, and these solutions were commonly administered with a single dose regardless of aortic cross-clamp time., Conclusions: Myocardial protection techniques still remained highly variable among congenital heart surgeons. This survey demonstrates that there is a perception that del Nido and Custodiol solutions can offer appropriate myocardial protection for longer intervals with decreased repeat dosing. An observational study correlating markers of postoperative myocardial performance with myocardial preservation strategies should be considered., (Copyright © 2013 The Society of Thoracic Surgeons. 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.

Background: We evaluated outcomes for groups of risk-stratified operations in The Society of Thoracic Surgeons Congenital Heart Surgery Database to provide contemporary benchmarks and examine variation between centers., Methods: Patients undergoing surgery from 2005 to 2009 were included. Centers with more than 10% missing data were excluded. Discharge mortality and postoperative length of stay (PLOS) among patients discharged alive were calculated for groups of risk-stratified operations using the five Society of Thoracic Surgeons-European Association for Cardio-Thoracic Surgery Congenital Heart Surgery mortality categories (STAT Mortality Categories). Power for analyzing between-center differences in outcome was determined for each STAT Mortality Category. Variation was evaluated using funnel plots and Bayesian hierarchical modeling., Results: In this analysis of risk-stratified operations, 58,506 index operations at 73 centers were included. Overall discharge mortality (interquartile range among programs with more than 10 cases) was as follows: STAT Category 1=0.55% (0% to 1.0%), STAT Category 2=1.7% (1.0% to 2.2%), STAT Category 3=2.6% (1.1% to 4.4%), STAT Category 4=8.0% (6.3% to 11.1%), and STAT Category 5=18.4% (13.9% to 27.9%). Funnel plots with 95% prediction limits revealed the number of centers characterized as outliers by STAT Mortality Categories was as follows: Category 1=3 (4.1%), Category 2=1 (1.4%), Category 3=7 (9.7%), Category 4=13 (17.8%), and Category 5=13 (18.6%). Between-center variation in PLOS was analyzed for all STAT Categories and was greatest for STAT Category 5 operations., Conclusions: This analysis documents contemporary benchmarks for risk-stratified pediatric cardiac surgical operations grouped by STAT Mortality Categories and the range of outcomes among centers. Variation was greatest for the more complex operations. These data may aid in the design and planning of quality assessment and quality improvement initiatives., (Copyright © 2012 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Extracorporeal membrane oxygenation (ECMO) is often used to provide cardiopulmonary support for infants experiencing severe levels of cardiac or respiratory failure. In patients with two ventricles and no intra-atrial communication, ECMO is often complicated by limited venous return to the circuit and marked left atrial hypertension. This condition may be treated by performing an atrial septostomy to create an intentional atrial septal defect (ASD). A pediatric mock circulation with a conduit connecting the left and right atrium was used to examine the size of ASD that would relieve left atrial hypertension and restore acceptable hemodynamics in a 4 to 5 kg infant. After creation of left ventricular failure and subsequent institution of ECMO, the ASD was opened in a graded fashion while the resulting hemodynamic changes were recorded. An ASD equivalent to a 6.3 mm diameter hole decreased the left atrial pressure 30 mmHg to an acceptable level with a net left-to-right shunt of 393 ml/min. Further opening of the ASD resulted in additional hemodynamic improvement, but was eventually limited in further effect. This study demonstrates that a mock circulation can be used to investigate the size of an ASD necessary to effect sufficient clinical improvement in a two-ventricle infant on ECMO.

This article presents 21 "Quality Measures for Congenital and Pediatric Cardiac Surgery" that were developed and approved by the Society of Thoracic Surgeons (STS) and endorsed by the Congenital Heart Surgeons' Society (CHSS). These Quality Measures are organized according to Donabedian's Triad of Structure, Process, and Outcome. It is hoped that these quality measures can aid in congenital and pediatric cardiac surgical quality assessment and quality improvement initiatives.

Background: We evaluated outcomes for common operations in The Society of Thoracic Surgeons Congenital Heart Surgery Database (STS-CHSDB) to provide contemporary benchmarks and examine variation between centers., Methods: Patients undergoing surgery from 2005 to 2009 were included. Centers with greater than 10% missing data were excluded. Discharge mortality and postoperative length of stay (PLOS) among patients discharged alive were calculated for 8 benchmark operations of varying complexity. Power for analyzing between-center variation in outcome was determined for each operation. Variation was evaluated using funnel plots and Bayesian hierarchical modeling., Results: Eighteen thousand three hundred seventy-five index operations at 74 centers were included in the analysis of 8 benchmark operations. Overall discharge mortality was: ventricular septal defect (VSD) repair = 0.6% (range, 0% to 5.1%), tetralogy of Fallot (TOF) repair = 1.1% (range, 0% to 16.7%), complete atrioventricular canal repair (AVC) = 2.2% (range, 0% to 20%), arterial switch operation (ASO) = 2.9% (range, 0% to 50%), ASO + VSD = 7.0% (range, 0% to 100%), Fontan operation = 1.3% (range, 0% to 9.1%), truncus arteriosus repair = 10.9% (0% to 100%), and Norwood procedure = 19.3% (range, 0% to 100%). Funnel plots revealed that the number of centers characterized as outliers were VSD = 0, TOF = 0, AVC = 1, ASO = 3, ASO + VSD = 1, Fontan operation = 0, truncus arteriosus repair = 4, and Norwood procedure = 11. Power calculations showed that statistically meaningful comparisons of mortality rates between centers could be made only for the Norwood procedure, for which the Bayesian-estimated range (95% probability interval) after risk-adjustment was 7.0% (3.7% to 10.3%) to 41.6% (30.6% to 57.2%). Between-center variation in PLOS was analyzed for all operations and was larger for more complex operations., Conclusions: This analysis documents contemporary benchmarks for common pediatric cardiac surgical operations and the range of outcomes among centers. Variation was most prominent for the more complex operations. These data may aid in quality assessment and quality improvement initiatives., (Copyright © 2011 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Objective: Outcomes data for adults undergoing congenital heart surgery are limited. Previous analyses used administrative data or focused on single-center outcomes. We describe the most common operations, patient characteristics, and postoperative outcomes using a multicenter clinical database., Methods: The study included adults (aged ≥ 18 years) listed in the Society of Thoracic Surgeons Congenital Heart Surgery Database (2000-2009). We describe patient characteristics and morbidity and mortality, and examine congenital procedures in the Society of Thoracic Surgeons Adult Cardiac Surgery Database to permit consideration of the primary dataset within a broader context., Results: A total of 5265 patients (68 centers) from the Society of Thoracic Surgeons Congenital Heart Surgery Database were included. Patients' median age was 25 years (interquartile range, 20-35). Common preoperative risk factors included noncardiac abnormalities (17%) and arrhythmia (14%). Overall, in-hospital mortality was 2.1%, 27% had 1 or more complication, and median length of stay was 5 days. Common operations included right ventricular outflow tract procedures (21%) and pacemaker/arrhythmia procedures (20%). We further evaluated cardiopulmonary bypass procedures in more than 100 patients. Mortality ranged from 0% (atrial septal defect repair) to 11% (Fontan revision/conversion). Separate evaluation of the Society of Thoracic Surgeons Adult Cardiac Surgery Database revealed 39,872 adults undergoing congenital heart operations., Conclusions: Most adult congenital heart operations listed in the Society of Thoracic Surgeons Congenital Heart Surgery Database are performed in the third to fourth decades of life; approximately half are for right heart pathology or arrhythmia. Many patients have complications, but mortality is low with the exception of those undergoing Fontan revision/conversion. Many more adults undergoing congenital heart surgery are entered into the Society of Thoracic Surgeons Adult Cardiac Surgery Database., (Copyright © 2011 The American Association for Thoracic Surgery. Published by Mosby, Inc. All rights reserved.)

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.)

Q fever is a rare systemic infection caused by Coxiella Burnetii. The presentation with endocarditis is insidious, with negative blood cultures, and oftentimes it is not obvious in diagnostic imaging studies until hemodynamic changes or valve destruction is reached. We report a case of Q fever endocarditis involving the tricuspid and aortic valves and a congenital ventricular septal defect. Surgical treatment and distinct aspects of this unusual case are herein described.

In view of the existing controversy concerning the best perfusion technique during deep hypothermic circulatory arrest (DHCA) for neonatal heart operations, we examined intraoperative rSO2 to help define an optimal interval for an intermittent antegrade cerebral perfusion (IACP) strategy. Records of patients undergoing stage 1 palliation (S1P) and repair of total anomalous pulmonary venous return (rTAPVR) from 1996 to 2004 were reviewed. A total of 16 patients were identified (11 S1P, 5 rTAPVR) with complete data and long periods of DHCA. A decline in rSO2 of either 20% or below a value of 50 was considered significant. The rSO2 for all patients was evaluated after 5, 10, 15, and 20 minutes of DHCA for significant cerebral desaturation. The average rSO2 at the start of DHCA ranged from 45 to 89 for S1P and 35-86 for rTAPVR. Significant cerebral desaturation was observed in 25%-31% of patients after 5 minutes; 42%-44% of patients after 10 minutes; 58%-69% after 15 minutes; and 75%-83% after 20 minutes. Each neonate has a unique baseline cerebral saturation. Also, the response to DHCA varies among subjects as the rate of decrease of rSO2 was not uniform. Universally applying the same interval after which to perfuse the brain permits significant cerebral desaturation in a large percentage of patients. Cerebral oximetry may provide a guide for developing an individualized cerebral perfusion strategy.

The population of patients with adult congenital heart disease is increasing. A significant number of these patients already have or will require placement of either a transvenous pacemaker or implantable cardioverter defibrillator. In addition to this, some with right ventricular dysfunction might benefit from volume unloading of the right ventricle by the construction of a superior cavopulmonary anastomosis. The usual technique for the bidirectional Glenn anastomosis precludes the presence of upper extremity transvenous hardware. We present a modified technique for the superior cavopulmonary anastomosis when pacing or cardioverter defibrillator leads are present.

Background: Reports of somatic mutations found in hearts with cardiac septal defects have suggested that these mutations are aetiologic in pathologic cardiac development. However, the hearts in these reports had been fixed in formalin for over 22 years. Because of the profound implication of this finding, we attempted to replicate it using fresh frozen tissue obtained in the current era from 28 patients with septal defects who underwent cardiac surgery and who were enrolled in our congenital heart disease tissue bank., Methods: Our cohort included patients with atrial septal defects (ASD, n = 13), ventricular septal defects (VSD, n = 5), and atrioventricular canal defects (AVCD, n = 10). Cardiac tissue samples were collected both from diseased tissue located immediately adjacent to the defect and from anatomically normal tissue located at a site remote from the defect (right atrial appendage). Tissue samples were immediately frozen in liquid nitrogen and stored at -80 degrees C. Genomic DNA was isolated and amplified using the same methodology described in the previously published reports. 42 pathologic cardiac tissue samples were sequenced., Results: One non-synonymous germline sequence variant was identified in one patient. Two synonymous germline sequence variants were identified in two separate patients. A common single nucleotide polymorphism (SNP) was identified in 16 patients. Based on the incidence of somatic mutations described in the previously published reports, our study was adequately powered to replicate the previous studies. No evidence of somatic mutations was found in this study., Conclusion: Somatic mutations in NKX2-5 do not represent an important aetiologic pathway in pathologic cardiac development in patients with cardiac septal defects.

Mycotic endoaortitis is a rarely occurring infectious entity with an extremely high mortality rate. We report an uncommon case of Phialemonium endoaortits with its management. This large fungal thrombus in the ascending aorta caused significant impediment to the blood flow.

We describe a case of thrombus formation within a right ventricle-to-pulmonary artery conduit (Sano shunt) in a patient with hypoplastic left heart syndrome with resulting acute cyanosis and hypotension requiring emergency balloon angioplasty.

We describe a case of a patient with hypoplastic left heart syndrome (HLHS) who developed protein-losing enteropathy (PLE) following autologous pericardial extracardiac Fontan (APEF) operation with successful resolution of PLE following transcatheter fenestration via the transhepatic approach.

Background: Although improved perioperative outcomes with growth potential of the extracardiac pedicled pericardial Fontan (EPPF) operation have been suggested, no advantage has been demonstrated., Methods: We retrospectively reviewed our institutional experience of 54 consecutive patients undergoing EPPF between June 1996 and August 2003. Clinical and echocardiographic follow-up was obtained yearly with a mean follow-up of 2.8 +/- 2.0 years., Results: There were 29 males, median age 3.3 years (2-6.8). Median cardiopulmonary bypass time was 79 min (39-295). Fibrillatory arrest was used briefly in 9 patients, of which 6 were for fenestration. One Fontan required takedown (1.8%) and there was 1 death (1.8%) from Candida mediastinitis. Median intensive care unit stay, hospital length of stay, and chest tube drainage were 4 days, 12 days, and 8 days, respectively. Arrhythmias occurred in 7 patients. Three (5.6%) of these had preexisting Holter abnormalities requiring permanent pacemaker implantation. Freedom from thromboembolic events, reoperation, and death at 2.8 years after discharge were 96.2%, 98.1%, and 100%, respectively. All patients were New York Heart Association class I-II, with median oxygen saturation of 94 %. Only 5 patients (9.4%) had mild self-restricted activities. Echocardiographic evaluation revealed excellent ventricular function and flow dynamics., Conclusions: At midterm follow-up this technique yields outcomes as good as the other Fontan techniques and with further follow-up may prove to be superior. However, at this point no clear advantage has been demonstrated. The low rate of complications and potential for growth are appealing features of this procedure.

The technology of transcranial near-infrared spectroscopy (NIRS) for the measurement of cerebral oxygen balance was introduced 25 years ago. Until very recently, there has been only occasional interest in its use during surgical monitoring. Now, however, substantial technologic advances and numerous clinical studies have, at least partly, succeeded in overcoming long-standing and widespread misunderstanding and skepticism regarding its value. Our goals are to clarify common misconceptions about near-infrared spectroscopy and acquaint the reader with the substantial literature that now supports cerebral oximetric monitoring in cardiac and major vascular surgery.

We describe two patients who required fenestration of an autologous pericardial extracardiac Fontan (APEF), short-medium term angiographic follow-up, and subsequent successful transcatheter closure of the fenestration.