Initial application in the EACTS and STS Congenital Heart Surgery Databases of an empirically derived methodology of complexity adjustment to evaluate surgical case mix and results.

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.

Variation in outcomes for risk-stratified pediatric cardiac surgical operations: an analysis of the STS Congenital Heart Surgery Database.

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.

Quality measures for congenital and pediatric cardiac surgery.

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.

Variation in outcomes for benchmark operations: an analysis of the Society of Thoracic Surgeons Congenital Heart Surgery Database.

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.

Transposition of the great arteries: lessons learned about patterns of practice and outcomes from the congenital heart surgery database of the society of thoracic surgeons.

The Society of Thoracic Surgeons (STS) Congenital Heart Surgery Database contains data about 3258 patients with the diagnosis of transposition of the great arteries (TGA) who underwent surgery during the 4-year time interval from July 1, 2005 to June 30, 2009, inclusive. This cohort includes 2918 patients with concordant atrioventricular connections and discordant ventriculoarterial connections and 341 patients with congenitally corrected TGA (discordant atrioventricular connections and discordant ventriculoarterial connections). The 4 most common operations were the following: (1) arterial switch operation (ASO) for TGA with intact ventricular septum (n = 1196), (2) ASO with ventricular septal defect (VSD) repair for TGA with VSD (n = 420), (3) ASO with VSD repair and aortic arch repair for TGA with VSD and hypoplastic arch (n = 55), and (4) Rastelli operation for TGA with VSD and left ventricular outflow tract obstruction (n = 49). Detailed preoperative, intraoperative, and postoperative data were obtained about patients who underwent these 4 operations. Median age at surgery (days) was as follows: ASO: 6.0; ASO with VSD repair: 7.0; ASO with VSD repair and aortic arch repair: 7.0; and Rastelli: 309.0. Mean age at surgery (days) was as follows: ASO: 22.9; ASO with VSD repair: 24.8; ASO with VSD repair and aortic arch repair: 14.4; and Rastelli: 721.8. Discharge mortality was as follows: ASO: 2.2%; ASO with VSD repair: 5.5%; ASO with VSD repair and aortic arch repair: 7.3%; and Rastelli: 0%. Median length of stay (days) was as follows: ASO: 11.0; ASO with VSD repair: 11.0; ASO with VSD repair and aortic arch repair: 18.0; and Rastelli: 7.0. The sternum was left open in the following: ASO: 24.8%; ASO with VSD repair: 29.5%; ASO with VSD repair and aortic arch repair: 40.0%; and Rastelli: 6.1%. This review of data from the STS Congenital Heart Surgery Database allows for unique documentation of patterns of practice and outcomes. From this review, we learned that although surgery for TGA is often complex and may be associated with morbidity, most patients survive without major complications.

Heterotaxy: lessons learned about patterns of practice and outcomes from the congenital heart surgery database of the society of thoracic surgeons.

According to The International Society for Nomenclature of Pediatric and Congenital Heart Disease (ISNPCHD), "Heterotaxy is synonymous with 'visceral heterotaxy' and 'heterotaxy syndrome'. Heterotaxy is defined as an abnormality where the internal thoraco-abdominal organs demonstrate abnormal arrangement across the left-right axis of the body. By convention, heterotaxy does not include patients with either the expected usual or normal arrangement of the internal organs along the left-right axis, also known as 'situs solitus', or patients with complete mirror-imaged arrangement of the internal organs along the left-right axis also known as `situs inversus'." or patients with complete mirror-image arrangement of the internal organs along the left-right axis, also known as situs inversus. The purpose of this article is to review the data about heterotaxy in the Society of Thoracic Surgeons (STS) Congenital Heart Surgery Database. The investigators examined all index operations in the STS Congenital Heart Surgery Database over 12 years from January 1, 1998 to December 31, 2009, inclusive. This analysis resulted in a cohort of 77 153 total index operations. Of these, 1505 operations (1.95%) were performed in patients with heterotaxy. Of the 1505 index operations performed in patients with heterotaxy, 1144 were in patients with asplenia and 361 were in patients with polysplenia. In every STS -EACTS Congenital Heart Surgery Mortality Category, discharge mortality is higher in patients with heterotaxy compared with patients without heterotaxy (EACTS = European Association for Cardio-Thoracic Surgery). Discharge mortality after systemic to pulmonary artery shunt is 6.6% in a cohort of all single-ventricle patients except those with heterotaxy, whereas it is 10.8% in single-ventricle patients with heterotaxy. Discharge mortality after Fontan is 1.8% in a cohort of all single-ventricle patients except those with heterotaxy, whereas it is 4.2% in single-ventricle patients with heterotaxy. The STS Congenital Heart Surgery Database is largest congenital heart surgery database in North America. This review of data from the STS Congenital Heart Surgery Database allows for unique documentation of practice patterns and outcomes. From this analysis, it is clear that heterotaxy is a challenging problem with increased discharge mortality in most subgroups.

Congenital heart surgery databases around the world: do we need a global database?

The question posed in the title of this article is: "Congenital Heart Surgery Databases Around the World: Do We Need a Global Database?" The answer to this question is "Yes and No"! Yes--we need to create a global database to track the outcomes of patients with pediatric and congenital heart disease. No--we do not need to create a new "global database." Instead, we need to create a platform that allows for the linkage of currently existing continental subspecialty databases (and continental subspecialty databases that might be created in the future) that will allow for the seamless sharing of multi-institutional longitudinal data across temporal, geographical, and subspecialty boundaries. This review article will achieve the following objectives: (A) Consider the current state of analysis of outcomes of treatments for patients with congenitally malformed hearts. (B) Present some principles that might make it possible to achieve life-long longitudinal monitoring and follow-up. (C) Describe the rationale for the creation of a Global Federated Multispecialty Congenital Heart Disease Database. (D) Propose a methodology for the creation of a Global Federated Multispecialty Congenital Heart Disease Database that is based on linking together currently existing databases without creating a new database. To perform meaningful multi-institutional analyses, any database must incorporate the following six essential elements: (1) Use of a common language and nomenclature. (2) Use of a database with an established uniform core dataset for collection of information. (3) Incorporation of a mechanism to evaluate the complexity of cases. (4) Implementation of a mechanism to assure and verify the completeness and accuracy of the data collected. (5) Collaboration between medical and surgical subspecialties. (6) Standardization of protocols for life-long longitudinal follow-up. Analysis of outcomes must move beyond recording 30-day or hospital mortality, and encompass longer-term follow-up, including cardiac and non-cardiac morbidities, and importantly, those morbidities impacting health-related quality of life. Methodologies must be implemented in our databases to allow uniform, protocol-driven, and meaningful long-term follow-up. We need to create a platform that allows for the linkage of currently existing continental subspecialty databases (and continental subspecialty databases that might be created in the future) that will allow for the seamless sharing of multi-institutional longitudinal data across temporal, geographical, and subspecialty boundaries. This "Global Federated Multispecialty Congenital Heart Disease Database" will not be a new database, but will be a platform that effortlessly links multiple databases and maintains the integrity of these extant databases. Description of outcomes requires true multi-disciplinary involvement, and should include surgeons, cardiologists, anesthesiologists, intensivists, perfusionists, neurologists, educators, primary care physicians, nurses, and physical therapists. Outcomes should determine primary therapy, and as such must be monitored life-long. The relatively small numbers of patients with congenitally malformed hearts requires multi-institutional cooperation to accomplish these goals. The creation of a Global Federated Multispecialty Congenital Heart Disease Database that links extant databases from pediatric cardiology, pediatric cardiac surgery, pediatric cardiac anesthesia, and pediatric critical care will create a platform for improving patient care, research, and teaching related to patients with congenital and pediatric cardiac disease.

Atrioventricular septal defects: lessons learned about patterns of practice and outcomes from the congenital heart surgery database of the society of thoracic surgeons.

During the 4-year time interval of 2005 through 2008, the Society of Thoracic Surgeons Congenital Heart Surgery Database documented data about 2882 operations to repair atrioventricular (AV) canal defects: partial, 623 (21.5%); intermediate, 342 (11.8%);. complete, 1917 (66.3%). Mean age at complete repair (years) was as follows: partial, 6.1; intermediate, 2.9; complete, 0.6. Median age at complete repair (years) was as follows: partial, 2.6; intermediate, 0.9; complete, 0.4. Down syndrome was present in 1767 patients (61.1%). Debanding of the pulmonary artery was rarely performed: partial, 1 (0.2%); intermediate, 0 (0.0%); complete, 66 (3.4%). Deep hypothermic circulatory arrest was rarely used: partial, 6 (1.0%); intermediate, 5 (1.5%); complete, 52 (2.7%). Discharge mortality was low: partial, 2 (0.3%); intermediate, 3 (0.9%); complete, 38 (2.0%). Atrioventricular block requiring permanent pacemaker occurred but was uncommon: partial, 6 (1.0%); intermediate, 2 (0.6%); complete, 29 (1.5%). Unplanned reoperation prior to hospital discharge occurred in 3.9% of complete AV canal repairs. The sternum was left open in 3.0% of complete AV canal repairs. Postoperative cardiac arrest occurred in 1.9% of complete AV canal repairs. Mean postoperative length of stay (days) was as follows: partial, 5.2; intermediate, 7; complete, 13.1. Median postoperative length of stay (days) was as follows: partial, 4; intermediate, 4; complete, 7. This review of data from the Society of Thoracic Surgeons Congenital Heart Surgery Database allows for unique documentation of patterns of practice and outcomes. From this review, we learned that 98% to 99% of patients survive complete repair of AV canal and 96% to 97% survive complete repair of AV canal with no major complications.

An empirically based tool for analyzing mortality associated with congenital heart surgery.

OBJECTIVE: Analysis of congenital heart surgery results requires a reliable method of estimating the risk of adverse outcomes. Two major systems in current use are based on projections of risk or complexity that were predominantly subjectively derived. Our goal was to create an objective, empirically based index that can be used to identify the statistically estimated risk of in-hospital mortality by procedure and to group procedures into risk categories. METHODS: Mortality risk was estimated for 148 types of operative procedures using data from 77,294 operations entered into the European Association for Cardiothoracic Surgery (EACTS) Congenital Heart Surgery Database (33,360 operations) and the Society of Thoracic Surgeons (STS) Congenital Heart Surgery Database (43,934 patients) between 2002 and 2007. Procedure-specific mortality rate estimates were calculated using a Bayesian model that adjusted for small denominators. Each procedure was assigned a numeric score (the STS-EACTS Congenital Heart Surgery Mortality Score [2009]) ranging from 0.1 to 5.0 based on the estimated mortality rate. Procedures were also sorted by increasing risk and grouped into 5 categories (the STS-EACTS Congenital Heart Surgery Mortality Categories [2009]) that were chosen to be optimal with respect to minimizing within-category variation and maximizing between-category variation. Model performance was subsequently assessed in an independent validation sample (n = 27,700) and compared with 2 existing methods: Risk Adjustment for Congenital Heart Surgery (RACHS-1) categories and Aristotle Basis Complexity scores. RESULTS: Estimated mortality rates ranged across procedure types from 0.3% (atrial septal defect repair with patch) to 29.8% (truncus plus interrupted aortic arch repair). The proposed STS-EACTS score and STS-EACTS categories demonstrated good discrimination for predicting mortality in the validation sample (C-index = 0.784 and 0.773, respectively). For procedures with more than 40 occurrences, the Pearson correlation coefficient between a procedure's STS-EACTS score and its actual mortality rate in the validation sample was 0.80. In the subset of procedures for which RACHS-1 and Aristotle Basic Complexity scores are defined, discrimination was highest for the STS-EACTS score (C-index = 0.787), followed by STS-EACTS categories (C-index = 0.778), RACHS-1 categories (C-index = 0.745), and Aristotle Basic Complexity scores (C-index = 0.687). When patient covariates were added to each model, the C-index improved: STS-EACTS score (C-index = 0.816), STS-EACTS categories (C-index = 0.812), RACHS-1 categories (C-index = 0.802), and Aristotle Basic Complexity scores (C-index = 0.795). CONCLUSION: The proposed risk scores and categories have a high degree of discrimination for predicting mortality and represent an improvement over existing consensus-based methods. Risk models incorporating these measures may be used to compare mortality outcomes across institutions with differing case mixes.

Stratification of complexity improves the utility and accuracy of outcomes analysis in a Multi-Institutional Congenital Heart Surgery Database: Application of the Risk Adjustment in Congenital Heart Surgery (RACHS-1) and Aristotle Systems in the Society of Thoracic Surgeons (STS) Congenital Heart Surgery Database.

Quality-of-care evaluation must take into account variations in "ase mix."This study reviewed the application of two case-mix complexity-adjustment tools in the Society of Thoracic Surgeons (STS) Congenital Heart Surgery Database: the Aristotle Basic Complexity (ABC) score and the Risk Adjustment in Congenital Heart Surgery (RACHS-1) method. The 2006 STS Congenital Heart Surgery Database Report, the first STS report to incorporate both methods, included 45,635 operations from 47 centers. Each operation was assigned an ABC score in a range from 1.5 (lowest complexity) to 15 (highest complexity), an ABC level in a range from 1 (lowest complexity) to 4 (highest complexity), and a RACHS-1 category in a range from 1 (lowest risk) to 6 (highest risk). The overall discharge mortality was 3.9% (1,222/31,719 eligible cardiac index operations). Of the eligible cardiac index operations, 85.8% (27,202/31,719) were eligible for analysis by the RACHS-1 method, and 94.0% (29,813/31,719) were eligible for analysis by the ABC approach. With both RACHS-1 and ABC, as complexity increases, discharge mortality also ncreases. The ABC approach allows classification of more operations, whereas the RACHS-1 discriminates better at the higher end of complexity. Complexity stratification is a useful method for analyzing the impact of case mix on pediatric cardiac surgical outcomes. Both the RACHS-1 and ABC methods facilitate complexity stratification in the STS database.

An artificial right ventricle for failing fontan: in vitro and computational study.

BACKGROUND: The aim of this study is to develop a destination low-pressure artificial right ventricle (ARV) to correct the impaired hemodynamics in the failing Fontan circulation. METHODS: An in vitro model circuit of the Fontan circulation was created to reproduce the hemodynamics of the failing Fontan and test ARV performance under various central venous pressures (CVP) and flows. A novel geometry of the extracardiac conduit was designed to adapt to the need of the pump. The ARV was a low-pressure axial flow pump designed to produce a low suction inflow pressure and moderate outflow increase. With the power off, the passive forward gradient across the propeller is 2 mm Hg at 4.5 L/min. The ARV would require 4 watts at a rotation of 5000 rpm. To examine the shear loading on the red blood cells, virtual particles were injected upstream of the ARV inducer and tracked by computerized modeling. RESULTS: The effect of the ARV on the failing Fontan was studied at various CVP pressures and flows, and under constant values of lung resistances and left atrial pressure set respectively to 2.5 Woods Units and 7 mm Hg. The CVP pressures decreased respectively from 25, 22.5, 20, 17.5, 15, and 10 mm Hg to a minimal value of 2 to 5 mm Hg with a pump speed varying from 1700 to 4500 rpm. The pulmonary artery pressures increased moderately between 12.5 and 25 mm Hg at 4500 rpm. Cardiac output at 4500 rpm was increased by an average gain of 2 L/min. The average blood damage index was 0.92%, far below the 5% value considered to cause hemolysis. The flow structure produced by the pump was suitable. CONCLUSIONS: The performance of this novel low-pressure ARV was satisfactory, showing good decrease of CVP pressures, a moderate increase of pulmonary artery pressures, adequate increase of cardiac output, and minimal hemolysis. The use of a mock Fontan model circuit facilitates device prototyping and design to a far greater extent than can be achieved using animal studies, and is an essential first step for rapid design iteration of a novel ARV device. The next steps are the manufacturing of this device, including an electromagnetic engine, a regulatory system, and further testing the device in a survival animal experiment.

Implications of incising the ventricular septum in double outlet right ventricle and in the Ross-Konno operation.

OBJECTIVE: Incision into the ventricular septum in complex biventricular repair is controversial, and has been blamed for impairing left ventricular function. This retrospective study evaluates the risk of a ventricular septal incision in patients undergoing double outlet right ventricle (DORV) repair and Ross-Konno procedure. METHODS: From January 2003 to September 2007, 11 patients with DORV had a ventricular septum (VS) incision and 12 DORV patients did not. Sixteen patients had a Ross-Konno, and 16 had an isolated Ross procedure. The ventricular septal incision was made to match at least the diameter of a normal aortic annulus. In DORV, the VSD was enlarged superiorly and to the left. In the Ross-Konno, the aortic annulus was enlarged towards the septum posteriorly and to the left. RESULTS: The median follow-up for the study is 19 months (1 month-4 years). For DORV, there were no significant differences in discharge mortality (p=0.22), late mortality (p=0.48), or late mortality plus heart transplant (p=0.093). Although patients with DORV and VSD enlargement have a more complex postoperative course, there were no differences in ECMO use (p=0.093), occurrence of permanent AV block (p=0.55), left ventricular ejection fraction (LVEF) (p=0.40), or shortening fraction (LVSF) (p=0.50). Similarly, for the Ross-Konno there were no significant differences in discharge mortality (p=0.30), late mortality (p=NS), LVEF (p=0.90) and LVSF (p=0.52) compared to the Ross, even though the Ross-Konno patients were significantly younger (p<0.0001). CONCLUSION: Making a ventricular septal incision in DORV repair and in the Ross-Konno operation does not increase mortality and does not impair the LV function. The restriction of the VSD remains an important issue in the management of complex DORV. These encouraging results need to be confirmed by larger series.

Nomenclature and databases for the surgical treatment of congenital cardiac disease--an updated primer and an analysis of opportunities for improvement.

This review discusses the historical aspects, current state of the art, and potential future advances in the areas of nomenclature and databases for the analysis of outcomes of treatments for patients with congenitally malformed hearts. We will consider the current state of analysis of outcomes, lay out some principles which might make it possible to achieve life-long monitoring and follow-up using our databases, and describe the next steps those involved in the care of these patients need to take in order to achieve these objectives. In order to perform meaningful multi-institutional analyses, we suggest that any database must incorporate the following six essential elements: use of a common language and nomenclature, use of an established uniform core dataset for collection of information, incorporation of a mechanism of evaluating case complexity, availability of a mechanism to assure and verify the completeness and accuracy of the data collected, collaboration between medical and surgical subspecialties, and standardised protocols for life-long follow-up. During the 1990s, both The European Association for Cardio-Thoracic Surgery and The Society of Thoracic Surgeons created databases to assess the outcomes of congenital cardiac surgery. Beginning in 1998, these two organizations collaborated to create the International Congenital Heart Surgery Nomenclature and Database Project. By 2000, a common nomenclature, along with a common core minimal dataset, were adopted by The European Association for Cardio-Thoracic Surgery and The Society of Thoracic Surgeons, and published in the Annals of Thoracic Surgery. In 2000, The International Nomenclature Committee for Pediatric and Congenital Heart Disease was established. This committee eventually evolved into the International Society for Nomenclature of Paediatric and Congenital Heart Disease. The working component of this international nomenclature society has been The International Working Group for Mapping and Coding of Nomenclatures for Paediatric and Congenital Heart Disease, also known as the Nomenclature Working Group. By 2005, the Nomenclature Working Group crossmapped the nomenclature of the International Congenital Heart Surgery Nomenclature and Database Project of The European Association for Cardio-Thoracic Surgery and The Society of Thoracic Surgeons with the European Paediatric Cardiac Code of the Association for European Paediatric Cardiology, and therefore created the International Paediatric and Congenital Cardiac Code, which is available for free download from the internet at [http://www.IPCCC.NET]. This common nomenclature, the International Paediatric and Congenital Cardiac Code, and the common minimum database data set created by the International Congenital Heart Surgery Nomenclature and Database Project, are now utilized by both The European Association for Cardio-Thoracic Surgery and The Society of Thoracic Surgeons. Between 1998 and 2007 inclusive, this nomenclature and database was used by both of these two organizations to analyze outcomes of over 150,000 operations involving patients undergoing surgical treatment for congenital cardiac disease. Two major multi-institutional efforts that have attempted to measure the complexity of congenital heart surgery are the Risk Adjustment in Congenital Heart Surgery-1 system, and the Aristotle Complexity Score. Current efforts to unify the Risk Adjustment in Congenital Heart Surgery-1 system and the Aristotle Complexity Score are in their early stages, but encouraging. Collaborative efforts involving The European Association for Cardio-Thoracic Surgery and The Society of Thoracic Surgeons are under way to develop mechanisms to verify the completeness and accuracy of the data in the databases. Under the leadership of The MultiSocietal Database Committee for Pediatric and Congenital Heart Disease, further collaborative efforts are ongoing between congenital and paediatric cardiac surgeons and other subspecialties, including paediatric cardiac anaesthesiologists, via The Congenital Cardiac Anesthesia Society, paediatric cardiac intensivists, via The Pediatric Cardiac Intensive Care Society, and paediatric cardiologists, via the Joint Council on Congenital Heart Disease and The Association for European Paediatric Cardiology. In finalizing our review, we emphasise that analysis of outcomes must move beyond mortality, and encompass longer term follow-up, including cardiac and non cardiac morbidities, and importantly, those morbidities impacting health related quality of life. Methodologies must be implemented in these databases to allow uniform, protocol driven, and meaningful, long term follow-up.

The importance of nomenclature for congenital cardiac disease: implications for research and evaluation.

BACKGROUND: Administrative databases are often used for congenital cardiac disease research and evaluation, with little validation of the accuracy of the diagnostic codes. METHODS: Metropolitan Atlanta Congenital Defects Program surveillance records were reviewed and classified using a version of the International Pediatric and Congenital Cardiac Code. Using this clinical nomenclature as the referent, we report the sensitivity and false positive fraction (1 - positive predictive value) of the International Classification of Diseases, Ninth Revision, Clinical Modification diagnosis codes for tetralogy of Fallot, transposition of the great arteries, and hypoplastic left heart syndrome. RESULTS: We identified 4918 infants and foetuses with congenital cardiac disease from the surveillance records. Using only the International Classification of Diseases diagnosis codes, there were 280 records with tetralogy, 317 records with transposition, and 192 records with hypoplastic left heart syndrome. Based on the International Pediatric and Congenital Cardiac Code, 330 records were classified as tetralogy, 163 records as transposition, and 179 records as hypoplastic left heart syndrome. The sensitivity of International Classification of Diseases diagnosis codes was 83% for tetralogy, 100% for transposition, and 95% for hypoplastic left heart syndrome. The false positive fraction was 2% for tetralogy, 49% for transposition, and 11% for hypoplastic left heart syndrome. CONCLUSIONS: Analyses based on International Classification of Diseases diagnosis codes may have substantial misclassification of congenital heart disease. Isolating the major defect is difficult, and certain codes do not differentiate between variants that are clinically and developmentally different.

Accuracy of the aristotle basic complexity score for classifying the mortality and morbidity potential of congenital heart surgery operations.

BACKGROUND: The Aristotle Basic Complexity Score (ABC score) was derived by consensus of an international surgeon panel to facilitate assessment of surgical performance for quality improvement in congenital heart surgery. The utility of the ABC score depends on its ability to correctly classify procedures according to their potential for morbidity, mortality, and technical difficulty. This collaborative study combined two multiinstitution databases to assess how well the ABC score predicts the actual morbidity and mortality potential of 131 congenital heart surgery procedures. METHODS: Data from the European Association of Cardiothoracic Surgery (EACTS) congenital database (17,838 operations, 56 centers) and the Society of Thoracic Surgeons (STS) congenital database (18,024 operations, 32 centers) were analyzed. Discrimination of the ABC score for predicting in-hospital mortality and postoperative length of stay (PLOS) of more than 21 days was quantified by the C statistic. Procedure-specific rates of mortality and prolonged PLOS were compared with predictions from a logistic regression model, and an exact binomial test was used to identify procedures that were mortality and morbidity outliers. RESULTS: There was a significant positive correlation between the ABC score of a procedure and its observed procedure-specific risk of mortality (C = 0.70) and prolonged PLOS (C = 0.67). Several individual procedures were identifed as mortality and morbidity outliers. CONCLUSIONS: The ABC score generally discriminates between low-risk and high-risk congenital procedures making it a potentially useful covariate for case-mix adjustment in congenital heart surgery outcomes analysis. Planned revisions of the ABC score will incorporate empirical data and will benefit from the large sample sizes of the STS and EACTS databases.

Nomenclature and databases - the past, the present, and the future : a primer for the congenital heart surgeon.

This review discusses the historical aspects, current state of the art, and potential future advances in the areas of nomenclature and databases for congenital heart disease. Five areas will be reviewed: (1) common language = nomenclature, (2) mechanism of data collection (database or registry) with an established uniform core data set, (3) mechanism of evaluating case complexity, (4) mechanism to ensure and verify data completeness and accuracy, and (5) collaboration between medical subspecialties. During the 1990s, both the Society of Thoracic Surgeons (STS) and the European Association for Cardiothoracic Surgery (EACTS) created congenital heart surgery outcomes databases. Beginning in 1998, the EACTS and STS collaborated in the work of the International Congenital Heart Surgery Nomenclature and Database Project. By 2000, a common congenital heart surgery nomenclature, along with a common core minimal data set, were adopted by the EACTS and the STS and published in the Annals of Thoracic Surgery. In 2000, the International Nomenclature Committee for Pediatric and Congenital Heart Disease was established; this committee eventually evolved into the International Society for Nomenclature of Paediatric and Congenital Heart Disease (ISNPCHD). The working component of ISNPCHD is the International Working Group for Mapping and Coding of Nomenclatures for Paediatric and Congenital Heart Disease, also known as the Nomenclature Working Group (NWG). By 2005, the NWG cross-mapped the EACTS-STS nomenclature with the European Paediatric Cardiac Code of the Association for European Paediatric Cardiology and created the International Paediatric and Congenital Cardiac Code (IPCCC) ( http://www.IPCCC.NET ). This common nomenclature (IPCCC), and the common minimum database data set created by the International Congenital Heart Surgery Nomenclature and Database Project, are now utilized by both EACTS and STS; since 1998, this nomenclature and database have been used by both the STS and EACTS to analyze outcomes of more than 75,000 patients. Two major multi-institutional efforts have attempted to measure case complexity; the Risk Adjustment in Congenital Heart Surgery-1 and the Aristotle Complexity Score. Efforts to unify these two scoring systems are in their early stages but are encouraging. Collaborative efforts involving the EACTS and STS are under way to develop mechanisms to verify data completeness and accuracy. Further collaborative efforts are also ongoing between pediatric and congenital heart surgeons and other subspecialties, including pediatric cardiac anesthesiologists (via the Congenital Cardiac Anesthesia Society), pediatric cardiac intensivists (via the Pediatric Cardiac Intensive Care Society), and pediatric cardiologists (via the Joint Council on Congenital Heart Disease). Clearly, methods of congenital heart disease outcomes analysis continue to evolve, with continued advances in five areas: nomenclature, database, complexity adjustment, data verification, and subspecialty collaboration.

Initial experience with the development and numerical and in vitro studies of a novel low-pressure artificial right ventricle for pediatric Fontan patients.

The Fontan operation, an efficient palliative surgery, is performed for patients with single-ventricle pathologies. The total cavopulmonary connection is a preferred Fontan procedure in which the superior and inferior vena cava are connected to the left and right pulmonary artery. The overall goal of this work is to develop an artificial right ventricle that can be introduced into the inferior vena cava, which would act to reverse the deleterious hemodynamics in post-Fontan patients. We present the initial design and computational analysis of a micro-axial pump, designed with the particular hemodynamics of Fontan physiology in mind. Preliminary in vitro data on a prototype pump are also presented. Computational studies showed that the new design can deliver a variety of advantageous operating conditions, including decreased venous pressure through proximal suction, increased pressure rise across the pump, increased pulmonary flows, and minimal changes in superior vena cava pressures. In vitro studies on a scaled prototype showed trends similar to those seen computationally. We conclude that a micro-axial flow pump can be designed to operate efficiently within the low-pressure, low-flow environment of cavopulmonary flows. The results provide encouragement to pursue this design to for in vitro studies and animal studies.

What is operative mortality? Defining death in a surgical registry database: a report of the STS Congenital Database Taskforce and the Joint EACTS-STS Congenital Database Committee.

The most concrete and universal outcome measure used in databases, whether governmental, professional society, research, or third-party payer, is operative mortality. To assure congruous data entry by multiple users of The Society of Thoracic Surgeons and the European Association for Cardiothoracic Surgery congenital heart surgery databases, operative mortality must be clearly defined. Traditionally, operative mortality has been defined as any death, regardless of cause, occurring (1) within 30 days after surgery in or out of the hospital, and (2) after 30 days during the same hospitalization subsequent to the operation. Differing hospital practices result in problems in use of the latter part of the definition (eg, the pediatric hospital that provides longer-term care will have higher mortality rates than one which transfers patients to another institution for such care). In addition, because of the significant number of pediatric multiple operation hospitalizations, issues of assignment of mortality to a specific operation within the hospitalization, calculation of operative mortality rates (operation based vs patient admission based), and discharge other than to home must be addressed and defined. We propose refinements to the definition of operative mortality which specifically meet the needs of our professional societies' multi-institutional registry databases, and at the same time are relevant and appropriate with respect to the goals and purposes of administrative databases, government agencies, and the general public.