First report from the European registry for anomalous aortic origin of coronary artery (EURO-AAOCA).

OBJECTIVES: Anomalous aortic origin of a coronary artery (AAOCA) is a group of rare congenital heart defects with various clinical presentations. The lifetime-risk of an individual living with AAOCA is unknown, and data from multicentre registries are urgently needed to adapt current recommendations and guide optimal patient management. The European AAOCA Registry (EURO-AAOCA) aims to assess differences with regard to AAOCA management between centres. METHODS: EURO-AAOCA is a prospective, multicentre registry including 13 European centres. Herein, we evaluated differences in clinical presentations and management, treatment decisions and surgical outcomes across centres from January 2019 to June 2023. RESULTS: A total of 262 AAOCA patients were included, with a median age of 33 years (12-53) with a bimodal distribution. One hundred thirty-nine (53.1%) were symptomatic, whereas chest pain (n = 74, 53.2%) was the most common complaint, followed by syncope (n = 21, 15.1%). Seven (5%) patients presented with a myocardial infarction, 2 (1.4%) with aborted sudden cardiac death. Right-AAOCA was most frequent (150, 57.5%), followed by left-AAOCA in 51 (19.5%), and circumflex AAOCA in 20 (7.7%). There were significant differences regarding diagnostics between age groups and across centres. Seventy-four (28.2%) patients underwent surgery with no operative deaths; minor postoperative complications occurred in 10 (3.8%) cases. CONCLUSIONS: Currently, no uniform agreement exists among European centres with regard to diagnostic protocols and clinical management for AAOCA variants. Although surgery is a safe procedure in AAOCA, future longitudinal outcome data will hopefully shed light on how to best decide towards optimal selection of patients undergoing revascularization versus conservative treatment.

The American Association for Thoracic Surgery (AATS) 2024 expert consensus document: Management of neonates and infants with Ebstein anomaly.

OBJECTIVES: Symptomatic neonates and infants with Ebstein anomaly (EA) require complex management. A group of experts was commissioned by the American Association for Thoracic Surgery to provide a framework on this topic focusing on risk stratification and management. METHODS: The EA Clinical Congenital Practice Standards Committee is a multinational and multidisciplinary group of surgeons and cardiologists with expertise in EA. A citation search in PubMed, Embase, Scopus, and Web of Science was performed using key words related to EA. The search was restricted to the English language and the year 2000 or later and yielded 455 results, of which 71 were related to neonates and infants. Expert consensus statements with class of recommendation and level of evidence were developed using a modified Delphi method, requiring 80% of members votes with at least 75% agreement on each statement. RESULTS: When evaluating fetuses with EA, those with severe cardiomegaly, retrograde or bidirectional shunt at the ductal level, pulmonary valve atresia, circular shunt, left ventricular dysfunction, or fetal hydrops should be considered high risk for intrauterine demise and postnatal morbidity and mortality. Neonates with EA and severe cardiomegaly, prematurity (<32 weeks), intrauterine growth restriction, pulmonary valve atresia, circular shunt, left ventricular dysfunction, or cardiogenic shock should be considered high risk for morbidity and mortality. Hemodynamically unstable neonates with a circular shunt should have emergent interruption of the circular shunt. Neonates in refractory cardiogenic shock may be palliated with the Starnes procedure. Children may be assessed for later biventricular repair after the Starnes procedure. Neonates without high-risk features of EA may be monitored for spontaneous closure of the patent ductus arteriosus (PDA). Hemodynamically stable neonates with significant pulmonary regurgitation at risk for circular shunt with normal right ventricular systolic pressure should have an attempt at medical closure of the PDA. A medical trial of PDA closure in neonates with functional pulmonary atresia and normal right ventricular systolic pressure (>20-25 mm Hg) should be performed. Neonates who are hemodynamically stable without pulmonary regurgitation but inadequate antegrade pulmonary blood flow may be considered for a PDA stent or systemic to pulmonary artery shunt. CONCLUSIONS: Risk stratification is essential in neonates and infants with EA. Palliative comfort care may be reasonable in neonates with associated risk factors that may include prematurity, genetic syndromes, other major medical comorbidities, ventricular dysfunction, or sepsis. Neonates who are unstable with a circular shunt should have emergent interruption of the circular shunt. Neonates who are unstable are most commonly palliated with the Starnes procedure. Neonates who are stable should undergo ductal closure. Neonates who are stable with inadequate pulmonary flow may have ductal stenting or a systemic-to-pulmonary artery shunt. Subsequent procedures after Starnes palliation include either single-ventricle palliation or biventricular repair strategies.

Congenital Heart Surgery Machine Learning-Derived In-Depth Benchmarking Tool.

BACKGROUND: We previously showed that machine learning-based methodologies of optimal classification trees (OCTs) can accurately predict risk after congenital heart surgery and assess case-mix-adjusted performance after benchmark procedures. We extend this methodology to provide interpretable, easily accessible, and actionable hospital performance analysis across all procedures. METHODS: The European Congenital Heart Surgeons Association Congenital Cardiac Database data subset of 172,888 congenital cardiac surgical procedures performed in European centers between 1989 and 2022 was analyzed. OCT models (decision trees) were built predicting hospital mortality (area under the curve [AUC], 0.866), prolonged postoperative mechanical ventilatory support time (AUC, 0.851), or hospital length of stay (AUC, 0.818), thereby establishing case-adjusted benchmarking standards reflecting the overall performance of all participating hospitals, designated as the "virtual hospital." OCT analysis of virtual hospital aggregate data yielded predicted expected outcomes (both aggregate and for risk-matched patient cohorts) for the individual hospital's own specific case-mix, readily available on-line. RESULTS: Raw average rates were hospital mortality, 4.9%; mechanical ventilatory support time, 14.5%; and length of stay, 15.0%. Of 146 participating centers, compared with each hospital's overall case-adjusted predicted hospital mortality benchmark, 20.5% statistically (<90% CI) overperformed and 20.5% underperformed. An interactive tool based on the OCT analysis automatically reveals 14 hospital-specific patient cohorts, simultaneously assessing overperformance or underperformance, and enabling further analysis of cohort strata in any chosen time frame. CONCLUSIONS: Machine learning-based OCT benchmarking analysis provides automatic assessment of hospital-specific case-adjusted performance after congenital heart surgery, not only overall but importantly, also by similar risk patient cohorts. This is a tool for hospital self-assessment, particularly facilitated by the user-accessible online-platform.

Combining Congenital Heart Surgical and Interventional Cardiology Outcome Data in a Single Database: The Development of a Patient-Centered Collaboration of the European Congenital Heart Surgeons Association (ECHSA) and the Association for European Paediatric and Congenital Cardiology (AEPC).

The European Congenital Heart Surgeons Association (ECHSA) Congenital Database (CD) is the second largest clinical pediatric and congenital cardiac surgical database in the world and the largest in Europe, where various smaller national or regional databases exist. Despite the dramatic increase in interventional cardiology procedures over recent years, only scattered national or regional databases of such procedures exist in Europe. Most importantly, no congenital cardiac database exists in the world that seamlessly combines both surgical and interventional cardiology data on an international level; therefore, the outcomes of surgical and interventional procedures performed on the same or similar patients cannot easily be tracked, assessed, and analyzed. In order to fill this important gap in our capability to gather and analyze information on our common patients, ECHSA and The Association for European Paediatric and Congenital Cardiology (AEPC) have embarked on a collaborative effort to expand the ECHSA-CD with a new module designed to capture data about interventional cardiology procedures. The purpose of this manuscript is to describe the concept, the structure, and the function of the new AEPC Interventional Cardiology Part of the ECHSA-CD, as well as the potentially valuable synergies provided by the shared interventional and surgical analyses of outcomes of patients. The new AEPC Interventional Cardiology Part of the ECHSA-CD will allow centers to have access to robust surgical and transcatheter outcome data from their own center, as well as robust national and international aggregate outcome data for benchmarking. Each contributing center or department will have access to their own data, as well as aggregate data from the AEPC Interventional Cardiology Part of the ECHSA-CD. The new AEPC Interventional Cardiology Part of the ECHSA-CD will allow cardiology centers to have access to aggregate cardiology data, just as surgical centers already have access to aggregate surgical data. Comparison of surgical and catheter interventional outcomes could potentially strengthen decision processes. A study of the wealth of information collected in the database could potentially also contribute toward improved early and late survival, as well as enhanced quality of life of patients with pediatric and/or congenital heart disease treated with surgery and interventional cardiac catheterization across Europe and the world.

Combining Congenital Heart Surgical and Interventional Cardiology Outcome Data in a Single Database: The Development of a Patient-Centered Collaboration of the European Congenital Heart Surgeons Association (ECHSA) and the Association for European Paediatric and Congenital Cardiology (AEPC).

The European Congenital Heart Surgeons Association (ECHSA) Congenital Database (CD) is the second largest clinical pediatric and congenital cardiac surgical database in the world and the largest in Europe, where various smaller national or regional databases exist. Despite the dramatic increase in interventional cardiology procedures over recent years, only scattered national or regional databases of such procedures exist in Europe. Most importantly, no congenital cardiac database exists in the world that seamlessly combines both surgical and interventional cardiology data on an international level; therefore, the outcomes of surgical and interventional procedures performed on the same or similar patients cannot easily be tracked, assessed, and analyzed. In order to fill this important gap in our capability to gather and analyze information on our common patients, ECHSA and The Association for European Paediatric and Congenital Cardiology (AEPC) have embarked on a collaborative effort to expand the ECHSA-CD with a new module designed to capture data about interventional cardiology procedures. The purpose of this manuscript is to describe the concept, the structure, and the function of the new AEPC Interventional Cardiology Part of the ECHSA-CD, as well as the potentially valuable synergies provided by the shared interventional and surgical analyses of outcomes of patients. The new AEPC Interventional Cardiology Part of the ECHSA-CD will allow centers to have access to robust surgical and transcatheter outcome data from their own center, as well as robust national and international aggregate outcome data for benchmarking. Each contributing center or department will have access to their own data, as well as aggregate data from the AEPC Interventional Cardiology Part of the ECHSA-CD. The new AEPC Interventional Cardiology Part of the ECHSA-CD will allow cardiology centers to have access to aggregate cardiology data, just as surgical centers already have access to aggregate surgical data. Comparison of surgical and catheter interventional outcomes could potentially strengthen decision processes. A study of the wealth of information collected in the database could potentially also contribute toward improved early and late survival, as well as enhanced quality of life of patients with pediatric and/or congenital heart disease treated with surgery and interventional cardiac catheterization across Europe and the world.

TGA + IVS + LVOTO: patterns of practice and outcomes.

PURPOSE: Transposition of great arteries with intact ventricular septum and left ventricular outflow tract obstruction (TGA + IVS + LVOTO) is uncommon. We reviewed operations performed in patients with TGA + IVS + LVOTO in the European Congenital Heart Surgeons Association Congenital Database (ECHSA-CD). METHODS: All 109 patients with a diagnosis of TGA + IVS + LVOTO in ECHSA-CD who underwent cardiac surgery during a 21-year period (01/2000-02/2021, inclusive) were included. Preoperative variables, operative data, and postoperative outcomes were collected. RESULTS: These 109 patients underwent 176 operations, including 37 (21.0%) arterial switch operations (ASO), 26 (14.2%) modified Blalock-Taussig-Thomas shunts (MBTTS), 11 (6.2%) Rastelli operations, and 13 (7.3%) other palliative operations (8 superior cavopulmonary anastomosis[es], 4 Fontan, and 1 other palliative procedure). Of 37 patients undergoing ASO, 22 had a concomitant procedure.There were 68 (38.6%) reoperations, including 11 pacemaker procedures and 8 conduit operations. After a systemic-to-pulmonary artery shunt, reoperations included shunt reoperation (n = 4), Rastelli (n = 4), and superior cavopulmonary anastomosis (n = 3).Overall Operative Mortality was 8.2% (9 deaths), including three following ASO, two following "Nikaidoh, Kawashima, or LV-PA conduit" procedures, and two following Rastelli. Postoperative complications occurred after 36 operations (20.4%). The most common complications were delayed sternal closure (n = 11), postoperative respiratory insufficiency requiring mechanical ventilation >7 days (n = 9), and renal failure requiring temporary dialysis (n = 8). CONCLUSION: TGA + IVS + LVOTO is rare (109 patients in ECHSA-CD over 21 years). ASO, MBTTS, and Rastelli are the most common operations performed for TGA + IVS + LVOTO. Larger international studies with long-term follow-up are needed to better define the anatomy of the LVOTO and to determine the optimal surgical strategy.

Ten Years of Data Verification: The European Congenital Heart Surgeons Association Congenital Database Audits.

BACKGROUND: Congenital heart surgery databases are tools for internal programmatic evaluation, benchmarking institutional results to multi-institutional aggregate data, and research. Therefore, it is essential to ensure the completeness and accuracy of data. This study analyzes the results of ten years of on-site source data verification of the European Congenital Heart Surgeons Association Congenital Heart Surgery Database (ECHSA CHSD). METHODS: All data forms verified between 2009 and 2018 were analyzed. The data form consists of 12 data elements: dates of birth, admission, surgery, discharge, and death; weight; case category; cardiopulmonary bypass time; aortic cross-clamp time; validation rules; diagnoses; and procedures. Descriptive data calculation and rates of completeness and accuracy were determined. The trend of error rate of seven centers with ≥5 visits was analyzed. RESULTS: Sixty-nine on-site verification visits took place at 17 centers. A total of 26,245 cases were verified; 2,841 of these 26,245 cases (10.8%) showed an error. The total mean error rate of centers for all years was 12.3 ± 2.1%. Rates of completeness and accuracy were 99% and 89.2%, respectively. Coded diagnoses and procedure analysis revealed that 716 (2.7%) and 456 (1.7%) datasets were incorrect, respectively. Rates of completeness and accuracy of dates were 100%, and 97.1%, respectively. Validation fields showed no errors. CONCLUSION: Source data verification is an appropriate tool to determine completeness and accuracy of data. The ECHSA CHSD verification analysis of a ten-year period showed a high level of completeness and accuracy. The verified data of the ECHSA CHSD are well-suited for benchmarking and research.

Benchmarking in Congenital Heart Surgery Using Machine Learning-Derived Optimal Classification Trees.

Background: We have previously shown that the machine learning methodology of optimal classification trees (OCTs) can accurately predict risk after congenital heart surgery (CHS). We have now applied this methodology to define benchmarking standards after CHS, permitting case-adjusted hospital-specific performance evaluation. Methods: The European Congenital Heart Surgeons Association Congenital Database data subset (31 792 patients) who had undergone any of the 10 "benchmark procedure group" primary procedures were analyzed. OCT models were built predicting hospital mortality (HM), and prolonged postoperative mechanical ventilatory support time (MVST) or length of hospital stay (LOS), thereby establishing case-adjusted benchmarking standards reflecting the overall performance of all participating hospitals, designated as the "virtual hospital." These models were then used to predict individual hospitals' expected outcomes (both aggregate and, importantly, for risk-matched patient cohorts) for their own specific cases and case-mix, based on OCT analysis of aggregate data from the "virtual hospital." Results: The raw average rates were HM = 4.4%, MVST = 15.3%, and LOS = 15.5%. Of 64 participating centers, in comparison with each hospital's specific case-adjusted benchmark, 17.0% statistically (under 90% confidence intervals) overperformed and 26.4% underperformed with respect to the predicted outcomes for their own specific cases and case-mix. For MVST and LOS, overperformers were 34.0% and 26.4%, and underperformers were 28.3% and 43.4%, respectively. OCT analyses reveal hospital-specific patient cohorts of either overperformance or underperformance. Conclusions: OCT benchmarking analysis can assess hospital-specific case-adjusted performance after CHS, both overall and patient cohort-specific, serving as a tool for hospital self-assessment and quality improvement.

Current Status of Training and Certification for Congenital Heart Surgery Around the World: Proceedings of the Meetings of the Global Council on Education for Congenital Heart Surgery of the World Society for Pediatric and Congenital Heart Surgery.

The optimal training of the highly specialized congenital heart surgeon is a long and complex process, which is a significant challenge in most parts of the world. The World Society for Pediatric and Congenital Heart Surgery (WSPCHS) has established the Global Council on Education for Congenital Heart Surgery as a nonprofit organization with the goal of assessing current training and certification and ultimately establishing standardized criteria for the training, evaluation, and certification of congenital heart surgeons around the world. The Global Council and the WSPCHS have reviewed the present status of training and certification for congenital cardiac surgery around the world. There is currently lack of consensus and standardized criteria for training in congenital heart surgery, with significant disparity between continents and countries. This represents significant obstacles to international job mobility of competent congenital heart surgeons and to the efforts to improve the quality of care for patients with Congenital Heart Disease worldwide. The purpose of this article is to summarize and document the present state of training and certification in congenital heart surgery around the world.

Adverse Outcomes Prediction for Congenital Heart Surgery: A Machine Learning Approach.

OBJECTIVE: Risk assessment tools typically used in congenital heart surgery (CHS) assume that various possible risk factors interact in a linear and additive fashion, an assumption that may not reflect reality. Using artificial intelligence techniques, we sought to develop nonlinear models for predicting outcomes in CHS. METHODS: We built machine learning (ML) models to predict mortality, postoperative mechanical ventilatory support time (MVST), and hospital length of stay (LOS) for patients who underwent CHS, based on data of more than 235,000 patients and 295,000 operations provided by the European Congenital Heart Surgeons Association Congenital Database. We used optimal classification trees (OCTs) methodology for its interpretability and accuracy, and compared to logistic regression and state-of-the-art ML methods (Random Forests, Gradient Boosting), reporting their area under the curve (AUC or c-statistic) for both training and testing data sets. RESULTS: Optimal classification trees achieve outstanding performance across all three models (mortality AUC = 0.86, prolonged MVST AUC = 0.85, prolonged LOS AUC = 0.82), while being intuitively interpretable. The most significant predictors of mortality are procedure, age, and weight, followed by days since previous admission and any general preoperative patient risk factors. CONCLUSIONS: The nonlinear ML-based models of OCTs are intuitively interpretable and provide superior predictive power. The associated risk calculator allows easy, accurate, and understandable estimation of individual patient risks, in the theoretical framework of the average performance of all centers represented in the database. This methodology has the potential to facilitate decision-making and resource optimization in CHS, enabling total quality management and precise benchmarking initiatives.

Anomalous left coronary artery from pulmonary artery repair: Outcomes from the European Congenital Heart Surgeons Association Database.

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.

An eight-year-old girl with tracheal mass treated as a difficult asthma case.

INTRODUCTION: Endobronchial masses such as mucoepidermoid carcinomas or carcinoid tumors are extremely rare in children and they usually originate from large bronchi. These lesions may cause wheezing and dyspnea with poor response to bronchodilators and mimic the airway obstruction caused by asthma. CASE STUDY: We present the case of an 8-year-old girl with tracheal mucoepidermoid carcinoma who was treated as a difficult asthma case with high dose of inhaled corticosteroids. RESULTS: The characteristic stridor, the lack of response to bronchodilators and to inhaled corticosteroid treatment, combined with the characteristic flow loop in spirometry and the hyperinflation seen on the chest radiograph, all raised the clinical suspicion of a tracheal lesion and indicated the need for flexible bronchoscopy. The bronchoscopy revealed a large lesion obstructing totally the trachea lumen. The latter finding was confirmed by chest high resolution CT. The mass was completely excised via sternotomy under cardiopulmonary bypass, and the pathologic examination showed a low-grade mucoepidermoid carcinoma of the trachea. One month after the surgery she was free of symptoms and her spirometry was normal. CONCLUSION: Tracheal lesions mimic the symptoms of airway obstruction caused by asthma and should be always be part of the differential diagnosis in young patients with no response to asthma treatment.

Early Impact of the COVID-19 Pandemic on Congenital Heart Surgery Programs Across the World: Assessment by a Global Multi-Societal Consortium.

The coronavirus disease 2019 (COVID-19) pandemic currently gripping the globe is impacting the entire health care system with rapidly escalating morbidities and mortality. Although the infectious risk to the pediatric population appears low, the effects on children with congenital heart disease (CHD) remain poorly understood. The closure of congenital heart surgery programs worldwide to address the growing number of infected individuals could have an unintended impact on future health for COVID-19-negative patients with CHD. Pediatric and congenital heart surgeons, given their small numbers and close relationships, are uniquely positioned to collectively assess the impact of the pandemic on surgical practice and care of children with CHD. We present the results of an international survey sent to pediatric and congenital heart surgeons characterizing the early impact of COVID-19 on the care of patients with CHD.

Surgery for Adult Patients with Congenital Heart Disease: Results from the European Database.

Adults with congenital heart disease (ACHD) represent a growing population. To evaluate frequency, type and outcomes of cardiac surgery in ACHD, we gathered data from the European Congenital Heart Surgeons Association Database of 20,602 adult patients (≥18 years) with a diagnosis of congenital heart disease who underwent cardiac surgery, between January 1997 and December 2017. We demonstrated that overall surgical workload (as absolute frequencies of surgical procedures per year) for this specific subset of patients increased steadily during the study period. The most common procedural groups included septal defects repair (n = 5740, 28%), right-heart lesions repair (n = 5542, 27%) and left-heart lesions repair (n = 4566, 22%); almost one-third of the procedures were re-operations (n = 5509, 27%). When considering the year-by-year relative frequencies of the main procedural groups, we observed a variation of the surgical scenario during the last two decades, characterized by a significant increase over time for right and left-heart lesions repair (p < 0.0001, both); while a significant decrease was seen for septal defects repair (p < 0.0001) and transplant (p = 0.03). Overall hospital mortality was 3% (n = 622/20,602 patients) and was stable over time. An inverse relationship between mortality and the number of patients operated in each center (p < 0.0001) was observed.

COVID-19: FAQs-Congenital Heart Surgery Recovery and Defining a "New Normal".

As recovery of congenital heart surgery programs begins during this COVID-19 pandemic, we review key considerations such as screening, protection of patients and health care workers (HCWs), case prioritization, barriers to reactivation, redesign of patient care teams, contribution of telemedicine, modification of trainees' experiences, preparation for potential resurgence, and strategies to maintain HCW wellness. COVID-19 has tested the resolve and grit of our specialty and we have an opportunity to emerge more refined.

Surgical Management of Aorto-Ventricular Tunnel. A Multicenter Study.

Aorto-ventricular tunnel (AoVT), a rare congenital anomaly, is a channel originating in the ascending aorta just above the sinotubular junction and leading to the cavity of the left ventricle (AoLVT), or, rarely, the right (AoRVT). This study reviews our collective 30-year experience with the surgical treatment of AoVT. Data were submitted by 15 participating centers on 42 patients who underwent correction of AoVT between 1987 and 2018. Of these, 36 had AoLVT, and 6 AoRVT. The tunnel originated above the right coronary sinus in 28 (77.8%) patients. For AoLVT, most operations were performed early (median age 25 days, range: 1 day-25 years). In contrast, AoRVT was diagnosed and repaired later (median age 6 years, range: 1 month-12 years). Surgically important coronary ostial displacement was common. Patch closure of the aortic orifice only was the commonest surgical repair for AoLVT (23 patients), while in AoRVT, both orifices or only the ventricular one was closed. Aortic valvar insufficiency, severe or moderate, coexisted in 11 (30.5%) patients with AoLVT, and aortic valvuloplasty was performed in 8, mainly due to aortic valve stenosis. Aortic valvar insufficiency at discharge ranged from trivial to mild in almost all patients. Early mortality was 7.14%, with 3 patients with AoLVT succumbing to cardiac failure. There were 2 early reoperations and 1 late death. AoVT is a rare malformation. AoLVT usually necessitates surgery in early life. AoRVT is rarer, diagnosed and repaired later in life. Surgical repair by patch closure, with concomitant aortic valve repair as needed, is associated with good results.

Performing modified fontan before six years of age leads to superior long-term exercise capacity.

We investigated the influence of age at Fontan completion on the exercise capacity of patients who underwent a Fontan operation. Our study demonstrated that age at the time of the Fontan operation significantly affects the peak oxygen consumption at mid-term follow up and that exercise capacity is superior in patients who have undergone Fontan completion at an earlier age. These findings provide support for recommendations to perform Fontan completion procedures relatively early.

Late management of the aortic root after repair of tetralogy of Fallot: A European multicentre study.

OBJECTIVES: We sought to determine the indications, type, and outcomes of reoperations on the aortic root after repair of tetralogy of Fallot (TOF). METHODS: Eleven centers belonging to the European Congenital Heart Surgeons Association contributed to the data collection process. We included 36 patients who underwent surgical procedures on the aortic root, including surgery on the aortic valve and ascending aorta, between January 1975 and December 2017. Original diagnoses included TOF-pulmonary stenosis (n = 18) and TOF-pulmonary atresia (n = 18). The main indications for reoperation were aortic insufficiency (n = 19, 53%), aortic insufficiency and dilatation of the ascending aorta (n = 10, 28%), aortic root dilatation (n = 4, 11%), and ascending aorta dilatation (n = 3, 8%). RESULTS: The median age at reoperation was 30.4 years (interquartile range 20.3-45.3 years), and mechanical aortic valve replacement was the most common procedure performed. Five patients died early after reoperation (14%), and larger ascending aorta diameters were associated with early mortality (P = .04). The median age at the last follow-up was 41.4 years (interquartile range 24.5-51.6 years). Late death occurred in five patients (5/31, 16%). Most survivors (15/26, 58%) were asymptomatic at the last clinical examination (New York Heart Association, NYHA class I). The remaining patients were NYHA class II (n = 7) and III (n = 3). The most common symptoms were fatigue (n = 5), dyspnea (n = 4), and exercise intolerance (n = 3). CONCLUSIONS: Reoperations on the aortic root are infrequent but may become necessary late after TOF repair. The main indications for reoperation are aortic insufficiency, either isolated or associated with a dilatation of the ascending aorta. The surgical risk at reoperation was high and the presence of ascending aorta dilation is related to higher mortality.