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Introduction: We sought to determine the surgical outcomes of patients with anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) enrolled in the European Congenital Heart Surgeons Association (ECHSA) database.Materials and Methods: From 1999 to 2019, 907 patients with ALCAPA underwent surgical repair and were included in the current study. The primary outcome was in-hospital mortality. Secondary outcomes included frequency and results of concomitant mitral valve surgery and postoperative mechanical circulatory support (MCS).Results: The overall in-hospital mortality was 6% (54/907) and was significantly higher in neonates (p = .01), patients with lower body surface area (BSA) (p = .01), and those requiring postoperative MCS (p = .001). Associated mitral valve surgery was performed in 144 patients (15.9%) and was associated with longer cardiopulmonary bypass (CPB) and aortic cross-clamp times (AOX) (p ≤ .0001) but was not significantly related to an increase in in-hospital mortality. Postoperative MCS was required in 66 patients (7.3%). These patients were younger (p ≤ .001), had a lower BSA (p ≤ .001), and required a longer CPB (p ≤ .001) and AOX time (p ≤ .001).Conclusions: ALCAPA repair can be achieved successfully, and with low surgical risk. Concomitant mitral valve procedures can be performed without increasing operative mortality. The use of MCS remains a valuable option, especially in younger patients. [ABSTRACT FROM AUTHOR]

OBJECTIVES A second paediatric report has been generated from the European Registry for Patients with Mechanical Circulatory Support (EUROMACS). The purpose of EUROMACS, which is operated by the European Association for Cardio-Thoracic Surgery, is to gather data related to durable mechanical circulatory support for scientific purposes and to publish reports with respect to the course of mechanical circulatory support therapy. Since the first report issued, efforts to increase compliance and participation have been extended. Additionally, the data provided the opportunity to analyse patients of younger age and lower weight. METHODS Participating hospitals contributed pre-, peri- and long-term postoperative data on mechanical circulatory support implants to the registry. Data for all implants in paediatric patients (≤19 years of age) performed from 1 January 2000 to 1 July 2019 were analysed. This report includes updates of patient characteristics, implant frequency, outcome (including mortality rates, transplants and recovery rates) as well as adverse events including neurological dysfunction, device malfunction, major infection and bleeding. RESULTS Twenty-nine hospitals contributed 398 registered implants in 353 patients (150 female, 203 male) to the registry. The most frequent aetiology of heart failure was any form of cardiomyopathy (61%), followed by congenital heart disease and myocarditis (16.4% and 16.1%, respectively). Competing outcomes analysis revealed that a total of 80% survived to transplant or recovery or are ongoing; at the 2-year follow-up examination, 20% died while on support. At 12 months, 46.7% received transplants, 8.7% were weaned from their device and 18.5% died. The 3-month adverse events rate was 1.69 per patient-year for device malfunction including pump exchange, 0.48 for major bleeding, 0.64 for major infection and 0.78 for neurological events. CONCLUSIONS The overall survival rate was 81.5% at 12 months following ventricular assist device implant. The comparison of survival rates of the early and later eras shows no significant difference. A focus on specific subgroups showed that survival was less in patients of younger age (<1 year of age) (P  = 0.01) and lower weight (<20 kg) (P  = 0.015). Transplant rates at 6 months continue to be low (33.2%) The fact that the EUROMACS registry is embedded within the European Association for Cardio-Thoracic Surgery Quality Improvement Programme offers opportunities to focus on improving outcomes. [ABSTRACT FROM AUTHOR]

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OBJECTIVES EUROMACS is a registry of the European Association for Cardio-Thoracic Surgery (EACTS) whose purpose is to gather clinical data related to durable mechanical circulatory support for scientific purposes and to publish annual reports. Because the treatment of children with end-stage heart failure has several significantly different characteristics than the treatment of adults, data and outcomes of interventions are analysed in this dedicated paediatric report. METHODS Participating hospitals contributed pre-, peri- and long-term postoperative data on mechanical circulatory support implants to the registry. Data for all implants in paediatric patients (≤19 years of age) performed from 1 January 2000 to 31 December 2017 were analysed. This report includes updates of patient characteristics, implant frequency, outcome (including mortality rates, transplants and recovery rates) as well as adverse events. RESULTS Twenty-five hospitals contributed 237 registered implants in 210 patients (81 ♀, 129 ♂) to the registry. The most frequent diagnosis was any form of cardiomyopathy (71.4%) followed by congenital heart disease (18.6%). Overall mean support time on a device was 11.6 months (±16.5 standard deviation). A total of 173 children (82.4%) survived to transplant, recovery or are ongoing; 37 patients (17.6%) died while on support within the observed follow-up time. At 12 months 38% of patients received transplants, 7% were weaned from their device and 15% died. At 24 months, 51% of patients received transplants, 17% died while on support, 22% were on a device and 9% were explanted due to myocardial recovery. The adverse events rate per 100 patient-months was 0.2 for device malfunction, 0.05 for major bleeding, 0.06 for major infection and 0.03 for neurological events within the first 3 months after implantation. CONCLUSIONS The first paediatric EUROMACS report reveals a low transplant rate in European countries within the first 2 years of implantation compared to US data. The 1-year survival rate seems to be satisfactory. Device malfunction including pump chamber changes due to thrombosis was the most frequent adverse event. [ABSTRACT FROM AUTHOR]

Background The optimal management and prognostic factors of postsurgical pulmonary vein stenosis remain controversial. We sought to determine current postsurgical pulmonary vein stenosis outcomes and prognostic factors in a multicentric study in the current era. Methods Seventy-five patients with postsurgical pulmonary vein stenosis who underwent 103 procedures in 14 European/North American centers (2000-2012) were included retrospectively. A specific pulmonary vein stenosis severity score was developed on the basis of the assessment of each pulmonary vein. End points were death, pulmonary vein reintervention, and restenosis. A univariate and multivariate risk analysis was performed. Results Some 76% of postsurgical pulmonary vein stenosis occurred after repair of a total anomalous pulmonary venous return. Sutureless repair was used in 42 of 103 procedures (41%), patch veinoplasty was used in 28 procedures (27%), and endarterectomy was used in 16 procedures (16%). Overall pulmonary vein restenosis, reintervention, and mortality occurred in 56% (n = 58/103), 49% (n = 50/103), and 27% (n = 20/75), respectively. Sutureless repair was associated with less restenosis (40% vs 67%; P = .007) and less reintervention (31% vs 61%; P = .003). Mortality after sutureless repair (20%; 7/35) tends to be lower than after nonsutureless repair (33%; 13/40) ( P = .22). A high postoperative residual pulmonary vein stenosis score at the time of hospital discharge was an independent risk factor for restenosis (hazard ratio [HR], 1.55; P < 10 −4 ), reintervention (HR, 1.33; P < 10 −4 ), and mortality (HR, 1.37; P < 10 −4 ). The sutureless technique was an independent protective factor against restenosis (HR, 0.27; P = .006). Conclusions Postsurgical pulmonary vein stenosis still has a guarded prognosis in the current era. The sutureless technique is an independent protective factor against restenosis. The severity of the residual disease evaluated by a new severity score is an independent risk factor for poor outcomes regardless of surgical technique. [ABSTRACT FROM AUTHOR]

OBJECTIVES: Our goal was to evaluate the early and late results of the surgical management of congenital supravalvular aortic stenosis (SVAS). METHODS: We performed a retrospective, multicentre study using data from the European Congenital Heart Surgeons Association. Exclusion criteria were age >18 years, operation before 1990 and redo supravalvular aortic stenosis operations. Multivariate Cox regression analysis was performed to detect independent predictors of adverse events. RESULTS: Of a total of 301 patients (male/female = 194/107; median age 3.9 years, range 13 days-17.9 years), 17.6% had a prior surgical or interventional procedure. Pulmonary artery stenosis was present in 41.5% and coronary anomalies in 13.6%. The operation consisted of a single patch repair in 36.7%, a pantaloon-shaped patch in 36.7%, a 3-patch technique in 14.3% and other techniques in 11.7%. Postoperative complications occurred in 14.9%, and the early mortality rate was 5%. At a median follow-up of 13 years (interquartile range 3.5-7.8; follow-up completed 79.1%), there were 10 late deaths (4.2%). A surgical reoperation or an interventional cardiology procedure occurred in 12.6% and 7.2%, respectively. No significant differences in outcomes between the techniques were found. Age at repair <12 months and pulmonary artery stenosis were associated with an increased risk of early (P = 0.0001) and overall mortality (P = 0.025), respectively. Having an operation after 2005 and co-existing pulmonary artery stenosis were significant predictors of late reintervention (P = 0.0110 and P = 0.001, respectively). CONCLUSIONS: Surgical repair of congenital stenosis is an effective procedure with acceptable surgical risk and good late survival, but late morbidity is not negligible, especially in infants and when associated pulmonary artery stenosis is present. [ABSTRACT FROM AUTHOR]

Background: A multivariable risk estimation model, in which the primary outcome was major infection, was recently developed and published using The Society of Thoracic Surgeons (STS) Congenital Heart Surgery Database. We have applied this risk estimation model to our congenital heart surgery program over a 16-year time interval to validate this risk estimation model and verify its specific risk factors for major infection. Methods: Using complete and verified data, we selected patients in whom major procedures had been classified using both Aristotle Basic Score and Risk Adjustment for Congenital Heart Surgery (RACHS-1) and created a multivariable model in which primary outcome was major infection (septicemia, mediastinitis, or endocarditis). We checked the STS risk estimation model for major infection. We also assessed the significance of the STS risk factors in our program. Results: A total of 6,314 patients were analyzed. We identified 197 (3.1%) major infections (septicemia 3%, endocarditis 0.015%, mediastinitis 0.09%). Hospital mortality, ventilation time, and length of stay were greater in patients with major infections. The following preoperative risk factors identified by the STS risk estimation model were significant in multivariate analysis in our patients: young age, high complexity, medium complexity, previous operation, and preoperative ventilation (p < 0.0001). Estimated infection risk ranged from 0.32% to 11.58%. The model discrimination was good (c index, 0.808). Risks of infections after most common congenital heart surgery procedures were similar in both studies (r s = 0.952, p = 0.0003). Conclusions: Our external validation study confirmed that the STS model can be used as a preoperative risk stratification tool for major infection risk at the single institutional level. [ABSTRACT FROM AUTHOR]

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. [Copyright &y& Elsevier]

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. [Copyright &y& Elsevier]

The World Society for Pediatric and Congenital Heart Surgery (WSPCHS) was established in 2006 to assemble pediatric and congenital heart surgeons from all continents and regions of the world and their colleagues from related specialties dealing with pediatric and congenital heart disease. Since its birth, it has held a highly successful inaugural scientific meeting in 2007 in Washington, DC, and a World Summit on Pediatric and Congenital Heart Surgery Services, Education, and Cardiac Care for Children and Adults with Congenital Heart Disease in 2008 in Montreal. It currently has 549 members from 71 countries and in a short period of time has become the largest organization in the world of pediatric and congenital heart surgeons. Its brief history already seems to be a guarantee of a promising future. Projects in the areas of research, training and education, patient care, and community service will allow the Society to reach its goals. By bringing together professionals from every region of the world, the WSPCHS should play a significant role in the improvement of care for children and adults with congenital heart disease around the world. [Copyright &y& Elsevier]

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. [Copyright &y& Elsevier]

Background: Surgery for congenital heart disease (CHD) has changed considerably during the last three decades. The results of primary repair have steadily improved, to allow treating almost all patients within the pediatric age; nonetheless an increasing population of adult patients requires surgical treatment. The objective of this study is to present the early surgical results of patients who require surgery for CHD in the adult population within a multicentered European study population. Methods: Data relative to the hospital course of 2,012 adult patients (age ≥18 years) who required surgical treatment for CHD from January 1, 1997 through December 31, 2004 were reviewed. Nineteen cardiothoracic centers from 13 European countries contributed to the data collection. Results: Mean age at surgery was 34.4 ± 14.53 years. Most of the operations were corrective procedures (1,509 patients, 75%), followed by reoperations (464 patients, 23.1%) and palliative procedures (39 patients, 1.9%). Six hundred forty-nine patients (32.2%) required surgical closure of an isolated ostium secundum atrial septal defect. Overall hospital mortality was 2%. Preoperative cyanosis, arrhythmias, and NYHA class III-IV, proved significant risk factors for hospital mortality. Follow-up data were available in 1,342 of 1,972 patients (68%) who were discharged home. Late deaths occurred in 6 patients (0.5%). Overall survival probability was 97% at 60 months, which is higher for corrective procedures (98.2%) if compared with reoperations (94.1%) and palliations (86.1%). Conclusions: Surgical treatment of CHD in adult patients, in specialized cardiac units, proved quite safe, beneficial, and low-risk. [Copyright &y& Elsevier]

Abstract: Objective: The multi-national and multi-institutional collection of data on outcomes in Congenital Heart Surgery (CHS) provides the possibility of analysis of results of treatment and may result in further improvement of the quality of care. The verification of data as far as the completeness and accuracy is necessary to give confidence to all sides—the patients, centers and regulatory authorities. The source data verification (SDV) although difficult, appears possible even in such a large-scale database. Methods: Out of 5.274 patients and 5.612 procedures data of 2003, collected in the database, 1.703 (32.3%) and 1.895 (33.8%), respectively, have been verified at five sites on following fields: IPPV time, date of birth, date of admission, date of surgery, date of discharge/mortality, body weight, case category, CPB time, AoX time, Circulatory arrest time. SDV was performed at five sites by two database officers using the sources of information different to the local copy of the database (patients'' files, operation notes, perfusion charts, OR Books). Verification was performed between June 1st and July 31st 2004. Statistical analysis was performed using R-project software, ver. 2.0.0. and Welch''s t-test for comparison of continuous variables. P-value >0.05 was used as statistically significant difference between groups. Results: Pre- and post-verification mortalities in all groups showed no significant differences although seven deaths out of 68 (10.27%) were missed. None of the other verified fields showed significant differences after verification. Conclusions: Source Data Verification showed no statistically significant differences between verified and non-verified data on 30 days mortality, LOS, age, body weight, CPB time, AoX and Circulatory arrest time. IPPV time was not available in 58.6% procedures. [Copyright &y& Elsevier]

The first report of The Society of Thoracic Surgeons (STS) National Congenital Heart Surgery Database in 1998 reported the clinical features of 18 congenital heart categories. The report provided a significant amount of important information and also highlighted the strengths and weaknesses of the existing database. Following this report the STS Congenital Heart Surgery Committee in cooperation with the European Association of Cardio-Thoracic Surgery and the European Congenital Heart Surgeons Foundation initiated the International Congenital Heart Surgery Nomenclature and Database Project. The goal was to begin the standardization of nomenclature reporting strategies and establish the foundations for an international congenital heart surgery database. The first report of the International Congenital Heart Surgery Nomenclature Project was published in The Annals of Thoracic Surgery in April 2000. The current report outlines modifications to the minimum dataset as well as the diagnoses and procedure short lists. Plans for the next STS National Congenital Heart Surgery Database harvest are also presented. [Copyright &y& Elsevier]

Background--To evaluate indications and results of surgery for primary cardiac tumors in children. Methods and Results--Eighty-nine patients aged ≤18 years undergoing surgery for cardiac tumor between 1990 and 2005 from 16 centers were included retrospectively (M/F=41/48; median age 4.3 months, range 1 day to 18 years). Sixty-three patients (68.5%) presented with symptoms. Surgery consisted of complete resection in 62 (69.7%) patients, partial resection in 21 (23.6%), and cardiac transplant in 4 (4.5%). Most frequent histotypes (93.2%) were benign (rhabdomyoma, myxoma, teratoma, fibroma, and hemangioma). Postoperative complications occurred in 29.9%. Early and late mortality were 4.5% each (mean follow-up, 6.3±4.4 years); major adverse events occurred in 28.2% of the patients; 90.7% of patients are in New York Heart Association class I. There were no statistically significant differences in survival, postoperative complications, or adverse events after complete and partial resection in benign tumors other than myxomas. Cardiac transplant was associated significantly with higher mortality rate (f=0.006). Overall mortality was associated to malignancy (P=0.0008), and adverse events during follow-up (P=0.005) Conclusions--Surgery for primary cardiac tumors in children has good early and long-term outcomes, with low recurrence rate. Rhabdomyomas are the most frequent surgical histotypes. Malignant tumors negatively affect early and late survival. Heart transplant is indicated when conservative surgery is not feasible. Lack of recurrence after partial resection of benign cardiac tumors indicates that a less risky tumor debulking is effective for a subset of histotypes such as rhabdomyomas and fibromas. [ABSTRACT FROM AUTHOR]

Abstract: Significant advances have been made in the care of children with cardiac disease. Most advances have been guided by anatomical and functional understanding of the changes from normal imposed by the cardiac malformations. As survival has increased, it has become rare to obtain untreated and unaltered hearts for morphologic examination. This limited availability of new specimens, coupled with the inevitable deterioration of currently available specimens, means that alternative methods of “preservation” become increasingly important. In this review, we propose and outline a format for the development of a Global Virtual Museum of Congenital Cardiac Pathology, based on established principles of anatomical analysis, classification based on an internationally accepted nomenclature (The International Pediatric and Congenital Cardiac Code or IPCCC), and the use of digital methods of storage of images and videos. In this fashion, we hope to preserve, in a virtual sense, the currently available anatomical specimens. [Copyright &y& Elsevier]