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.

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.

Tetralogy Surgery - Back To Baltimore 70 Years Later: Melbourne Heritage and Group Tribute to Juan Comas.

Surgery for Tetralogy of Fallot progressed rapidly from the palliative arterio-pulmonary Blalock-Taussig shunt, introduced in Baltimore 70 years ago, to the "classic" complete transventricular repair technique, with which excellent early results were achieved soon thereafter. However, as duration of follow-up increased, so did the awareness of development of troubling late complications, including severe pulmonary insufficiency, right ventricular dilatation and dysfunction, and tricuspid valve insufficiency, all contributing to increasing incidence of late reoperations, as well as to arrhythmias and sudden death. This realization fueled the initial introduction of the transatrial-transpulmonary repair technique by Kawashima, as well as the subsequent firm establishment of this technique within the framework of an integrated surgical approach by Roger Mee in Melbourne. In turn, Mee's numerous trainees and associates led the dissemination of this approach and provided the impetus for the current wide adoption of a variety of right ventricular and pulmonary valve preservation techniques. In addition to the outstanding surgical results reported by individual centers adopting this surgical strategy, encouraging multi-institutional data are emerging regarding the benefits of these approaches for more favorable early and, most importantly, late outcome. One student and strong proponent of the Melbourne approach was our late colleague and friend Juan Comas, to whose memory this article can serve as tribute.

International quality improvement initiatives.

Across the globe, the implementation of quality improvement science and collaborative learning has positively affected the care and outcomes for children born with CHD. These efforts have advanced the collective expertise and performance of inter-professional healthcare teams. In this review, we highlight selected quality improvement initiatives and strategies impacting the field of cardiovascular care and describe implications for future practice and research. The continued leveraging of technology, commitment to data transparency, focus on team-based practice, and recognition of cultural norms and preferences ensure the success of sustainable models of global collaboration.

Surgery for primary cardiac tumors in children: early and late results in a multicenter European Congenital Heart Surgeons Association study.

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 (P=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.

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.

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.

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.

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.

Anterior aortic reimplantation of anomalous left coronary artery from the pulmonary artery (ALCAPA) originating from the nonfacing sinus in an adult.

We report successful repair of a rare type of anomalous origin left main coronary artery from the nonfacing pulmonary artery sinus in an adult patient presenting with cardiac arrest as first symptom. Intraoperative findings and surgical technique are discussed.

Remarkably Still Repairable Large Aortopulmonary Window in an Adult Patient.

Aortopulmonary window (APW) is an abnormal congenital connection between the main pulmonary artery (MPA) and the ascending aorta, with intact aortic and pulmonary valves, leading to heart failure or, if not repaired early, to pulmonary vascular obstructive disease. We report the rare case of an asymptomatic adult with an unrestrictive APW, whose pulmonary arterial hypertension was remarkably still reversible, permitting successful repair.

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.

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.

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.

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.

Etiology and management of chylothorax following pediatric heart surgery.

BACKGROUND: Chylothorax after congenital heart surgery (CHD) is a potentially challenging complication. The purpose of this study was to review our experience with the management of chylothorax following congenital heart surgery. METHODS: Between September 1997 and August 2006, of 1341 pediatric patients undergoing correction of congenital heart disease in our institution, 18 (1.3%) developed chylothorax postoperatively. Surgical procedures included tetralogy of Fallot repair in 10 patients, ventricular septal defect closure (one), atrial septal defect with pulmonary stenosis repair (one), Fontan procedure (three), coarctation of the aorta repair (one), aortopulmonary shunt (one), and ligation of patent ductus arteriosus in one patient. All patients followed a therapeutic protocol including complete drainage of chyle collection and controlled nutrition. Somatostatin was used adjunctively in six (33.3%) patients. Surgical intervention was reserved for persistent lymph leak despite maximal therapy. Following resolution of chylothorax, a medium-chain triglyceride diet was implemented for six weeks. RESULTS: There were no deaths. Fifteen patients (83.3%) responded to conservative therapy. Lymph leak ranged from 2.5 to 14.7 mL/kg per day for 8 to 42 days. Three patients with persistent drainage required thoracotomy with pleurodesis to achieve resolution, in two of which previously attempted chemical pleurodesis with doxycycline proved ineffective. Duration of lymph leak in this subgroup ranged from 15 to 47 days with 5.1 to 7.4 mL/kg per day output. CONCLUSIONS: Postoperative chylothorax is an infrequent complication of surgery for congenital heart disease and can occur even after median sternotomy in the absence of pathologically elevated venous pressure or Fontan circulation. Although hospitalization can be prolonged, conservative therapy is effective in most cases, while surgical pleurodesis proved successful in the refractory cases.

Surgery for anomalous aortic origin of coronary arteries: a multicentre study from the European Congenital Heart Surgeons Association†.

OBJECTIVES: We sought to describe early and late outcomes in a large surgical series of patients with anomalous aortic origin of coronary arteries. METHODS: We performed a retrospective multicentre study including surgical patients with anomalous aortic origin of coronary arteries since 1991. Patients with isolated high coronary takeoff and associated major congenital heart disease were excluded. RESULTS: We collected 156 surgical patients (median age 39.5 years, interquartile range 15-53) affected by anomalous right (67.9%), anomalous left (22.4%) and other anatomical abnormalities (9.6%). An interarterial course occurred in 86.5%, an intramural course in 62.8% and symptoms in 85.9%. The operations included coronary unroofing (56.4%), reimplantation (19.2%), coronary bypass graft (15.4%) and other (9.0%). Two patients with preoperative cardiac failure died postoperatively (1.3%). All survivors were discharged home in good clinical condition. At a median follow-up of 2 years (interquartile range 1-5, 88.5% complete), there were 3 deaths (2.2%), 9 reinterventions in 8 patients (5 interventional, 3 surgical); 91.2% are in New York Heart Association functional class ≤ II, but symptoms persisted in 14.2%; 48.1% of them returned to sport activity. On Kaplan-Meier analysis, event-free survival at follow-up was 74.6%. Morbidity was not significantly different among age classes, anatomical variants and types of surgical procedures. Furthermore, return to sport activity was significantly higher in younger patients who participated in sports preoperatively. CONCLUSIONS: Surgical repair of anomalous aortic origin of coronary arteries is effective and has few complications. Unroofing and coronary reimplantation are safe and are the most common procedures. The occurrence of late adverse events is not negligible, and long-term surveillance is mandatory. Most young athletes can return to an unrestrained lifestyle.