Nomenclature for Pediatric and Congenital Cardiac Care: Unification of Clinical and Administrative Nomenclature - The 2021 International Paediatric and Congenital Cardiac Code (IPCCC) and the Eleventh Revision of the International Classification of Diseases (ICD-11).

Substantial progress has been made in the standardization of nomenclature for paediatric and congenital cardiac care. In 1936, Maude Abbott published her Atlas of Congenital Cardiac Disease, which was the first formal attempt to classify congenital heart disease. The International Paediatric and Congenital Cardiac Code (IPCCC) is now utilized worldwide and has most recently become the paediatric and congenital cardiac component of the Eleventh Revision of the International Classification of Diseases (ICD-11). The most recent publication of the IPCCC was in 2017. This manuscript provides an updated 2021 version of the IPCCC.The International Society for Nomenclature of Paediatric and Congenital Heart Disease (ISNPCHD), in collaboration with the World Health Organization (WHO), developed the paediatric and congenital cardiac nomenclature that is now within the eleventh version of the International Classification of Diseases (ICD-11). This unification of IPCCC and ICD-11 is the IPCCC ICD-11 Nomenclature and is the first time that the clinical nomenclature for paediatric and congenital cardiac care and the administrative nomenclature for paediatric and congenital cardiac care are harmonized. The resultant congenital cardiac component of ICD-11 was increased from 29 congenital cardiac codes in ICD-9 and 73 congenital cardiac codes in ICD-10 to 318 codes submitted by ISNPCHD through 2018 for incorporation into ICD-11. After these 318 terms were incorporated into ICD-11 in 2018, the WHO ICD-11 team added an additional 49 terms, some of which are acceptable legacy terms from ICD-10, while others provide greater granularity than the ISNPCHD thought was originally acceptable. Thus, the total number of paediatric and congenital cardiac terms in ICD-11 is 367. In this manuscript, we describe and review the terminology, hierarchy, and definitions of the IPCCC ICD-11 Nomenclature. This article, therefore, presents a global system of nomenclature for paediatric and congenital cardiac care that unifies clinical and administrative nomenclature.The members of ISNPCHD realize that the nomenclature published in this manuscript will continue to evolve. The version of the IPCCC that was published in 2017 has evolved and changed, and it is now replaced by this 2021 version. In the future, ISNPCHD will again publish updated versions of IPCCC, as IPCCC continues to evolve.

The importance of patient-specific preoperative factors: an analysis of the society of thoracic surgeons congenital heart surgery database.

BACKGROUND: The most common forms of risk adjustment for pediatric and congenital heart surgery used today are based mainly on the estimated risk of mortality of the primary procedure of the operation. The goals of this analysis were to assess the association of patient-specific preoperative factors with mortality and to determine which of these preoperative factors to include in future pediatric and congenital cardiac surgical risk models. METHODS: All index cardiac operations in The Society of Thoracic Surgeons Congenital Heart Surgery Database (STS-CHSD) during 2010 through 2012 were eligible for inclusion. Patients weighing less than 2.5 kg undergoing patent ductus arteriosus closure were excluded. Centers with more than 10% missing data and patients with missing data for discharge mortality or other key variables were excluded. Rates of discharge mortality for patients with or without specific preoperative factors were assessed across age groups and were compared using Fisher's exact test. RESULTS: In all, 25,476 operations were included (overall discharge mortality 3.7%, n=943). The prevalence of common preoperative factors and their associations with discharge mortality were determined. Associations of the following preoperative factors with discharge mortality were all highly significant (p<0.0001) for neonates, infants, and children: mechanical circulatory support, renal dysfunction, shock, and mechanical ventilation. CONCLUSIONS: Current STS-CHSD risk adjustment is based on estimated risk of mortality of the primary procedure of the operation as well as age, weight, and prematurity. The inclusion of additional patient-specific preoperative factors in risk models for pediatric and congenital cardiac surgery could lead to increased precision in predicting risk of operative mortality and comparison of observed to expected outcomes.

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.

Successful linking of the Society of Thoracic Surgeons database to social security data to examine survival after cardiac operations.

BACKGROUND: Long-term evaluation of cardiothoracic surgical outcomes is a major goal of The Society of Thoracic Surgeons (STS). Linking the STS Database to the Social Security Death Master File (SSDMF) allows for the verification of "life status." This study demonstrates the feasibility of linking the STS Database to the SSDMF and examines longitudinal survival after cardiac operations. METHODS: For all operations in the STS Adult Cardiac Surgery Database performed in 2008 in patients with an available Social Security Number, the SSDMF was searched for a matching Social Security Number. Survival probabilities at 30 days and 1 year were estimated for nine common operations. RESULTS: A Social Security Number was available for 101,188 patients undergoing isolated coronary artery bypass grafting, 12,336 patients undergoing isolated aortic valve replacement, and 6,085 patients undergoing isolated mitral valve operations. One-year survival for isolated coronary artery bypass grafting was 88.9% (6,529 of 7,344) with all vein grafts, 95.2% (84,696 of 88,966) with a single mammary artery graft, 97.4% (4,422 of 4,540) with bilateral mammary artery grafts, and 95.6% (7,543 of 7,890) with all arterial grafts. One-year survival was 92.4% (11,398 of 12,336) for isolated aortic valve replacement (95.6% [2,109 of 2,206] with mechanical prosthesis and 91.7% [9,289 of 10,130] with biologic prosthesis), 86.5% (2,312 of 2,674) for isolated mitral valve replacement (91.7% [923 of 1,006] with mechanical prosthesis and 83.3% [1,389 of 1,668] with biologic prosthesis), and 96.0% (3,275 of 3,411) for isolated mitral valve repair. CONCLUSIONS: Successful linkage to the SSDMF has substantially increased the power of the STS Database. These longitudinal survival data from this large multi-institutional study provide reassurance about the durability and long-term benefits of cardiac operations and constitute a contemporary benchmark for survival after cardiac operations.

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.

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 nomenclature of safety and quality of care for patients with congenital cardiac disease: a report of the Society of Thoracic Surgeons Congenital Database Taskforce Subcommittee on Patient Safety.

A large body of literature devoted to "patient safety" and error prevention exists and utilizes a nomenclature that can be applied specifically to the field of congenital cardiac disease and aid in the goals of increasing the safety of patients, decreasing medical error, minimizing mortality and morbidity, and evaluating quality of care. The purpose of this manuscript is to suggest and document a quality of health care taxonomy and the appropriate application of this nomenclature of "patient safety" to the specialty of congenital cardiac disease, with special emphasis on the following ten terms: morbidity, complication, medical error, adverse event, harm, near miss, iatrogenesis, iatrogenic complication, medical injury, and sentinel event. Each of these terms is commonly utilized in the medical literature without universal agreement on their meaning and relationship. It is our hope that the standardization of the definitions of these terms, as they are applied to the analysis of outcomes of the treatments applied to patients with congenital and paediatric cardiac disease, will facilitate improved methodologies to assess and improve quality of care in our profession.

Databases for assessing the outcomes of the treatment of patients with congenital and paediatric cardiac disease--the perspective of cardiac surgery.

This review includes a brief discussion, from the perspective of cardiac surgeons, of the rationale for creation and maintenance of multi-institutional databases of outcomes of congenital heart surgery, together with a history of the evolution of such databases, a description of the current state of the art, and a discussion of areas for improvement and future expansion of the concept. Five fundamental areas are reviewed: nomenclature, mechanism of data collection and storage, mechanisms for the evaluation and comparison of the complexity of operations and stratification of risk, mechanisms to ensure the completeness and accuracy of the data, and mechanisms for expansion of the current capabilities of databases to include comparison and sharing of data between medical subspecialties. This review briefly describes several European and North American initiatives related to databases for pediatric and congenital cardiac surgery the Congenital Database of The European Association for Cardio-Thoracic Surgery, the Congenital Database of The Society of Thoracic Surgeons, the Pediatric Cardiac Care Consortium, and the Central Cardiac Audit Database in the United Kingdom. Potential means of approaching the ultimate goal of acquisition of long-term follow-up data, and input of this data over the life of the patient, are also considered.

Stratification of complexity: the Risk Adjustment for Congenital Heart Surgery-1 method and the Aristotle Complexity Score--past, present, and future.

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

Cardiac surgery in infants with low birth weight is associated with increased mortality: analysis of the Society of Thoracic Surgeons Congenital Heart Database.

OBJECTIVE: The evaluation of operative mortality risk for cardiac surgery in infants with low weight is limited. To determine whether low weight is a risk factor for increased mortality, we reviewed the experience within the Society of Thoracic Surgeons Congenital Heart Surgery Database of infants who have undergone surgical correction or palliation for congenital heart disease. METHODS: We analyzed mortality in 3022 infants ages 0 to 90 days weighing 1 to 2.5 kg (n = 517) and greater than 2.5 to 4 kg (n = 2505) who underwent cardiac surgery from 2002 through 2004 at 32 participating centers. Patients were grouped according to the primary procedure performed and analyzed according to their weight at the time of surgical intervention. Patients were also analyzed according to Risk Adjustment for Congenital Heart Surgery-1 and Aristotle Basic Complexity scores. RESULTS: Compared with infants weighing 2.5 to 4 kg, infants weighing less than 2.5 kg had a significantly higher mortality for the following operations: repair of coarctation of the aorta, total anomalous pulmonary venous connection repair, arterial switch procedure, systemic to pulmonary artery shunt, and the Norwood procedure. Lower infant weight remained strongly associated with mortality risk after stratifying the population by Risk Adjustment for Congenital Heart Surgery-1 levels 2 through 6 and Aristotle Basic Complexity levels 2 through 4. CONCLUSIONS: Low weight at the time of surgical intervention is associated with increased mortality in patients undergoing several types of cardiovascular procedures. These data do not allow assessment of specific risks or benefits of any particular treatment strategy. However, they do support the need for prospective analysis of specific treatment strategies for these high-risk patients.

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.

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.