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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.

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.

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.

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.

Funding Information: We gratefully acknowledge the contributions of ECDB participating surgeons and centers, whose CHS data have made this study possible. The authors received no financial support for the research, authorship, and/or publication of this article. Publisher Copyright: © The Author(s) 2021. 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. publishersversion published

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.

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.

BACKGROUND: Anomalous aortic origin of coronary arteries (AAOCA) is a rare abnormality, whose optimal management is still undefined. We describe early outcomes in patients treated with different management strategies. METHODS: This is a retrospective clinical multicenter study including patients with AAOCA, undergoing or not surgical treatment. Patients with isolated high coronary take off and associated major congenital heart disease were excluded. Preoperative, intraoperative, anatomical and postoperative data were retrieved from a common database. RESULTS: Among 217 patients, 156 underwent Surgical repair (median age 39 years, IQR: 15-53), while 61 were Medical (median age 15 years, IQR: 8-52), in whom AAOCA was incidentally diagnosed during screening or clinical evaluations. Surgical patients were more often symptomatic when compared to medical ones (87.2% vs 44.3%, p

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.

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.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.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.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.

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.

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.

The World Society for Pediatric and Congenital Heart Surgery (WSPCHS) is the largest professional organization in the world dedicated to pediatric and congenital heart surgery. The purpose of this article is to document the first decade of the history of WSPCHS from its formation in 2006, to summarize the current status of WSPCHS, and to consider the future of WSPCHS. The WSPCHS was incorporated in Canada on April 7, 2011, with a head office in Montreal, Canada. The vision of the WSPCHS is that every child born anywhere in the world with a congenital heart defect should have access to appropriate medical and surgical care. The mission of the WSPCHS is to promote the highest quality comprehensive cardiac care to all patients with congenital heart disease, from the fetus to the adult, regardless of the patient’s economic means, with an emphasis on excellence in teaching, research, and community service.

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.

Background: Mortality through single-ventricle palliation remains high and the effect of the timing of stage 2 palliation (S2P) is not well understood. We investigated current practice patterns in the timing of S2P across two professional societies and compared them to actual practice patterns from two databases of patients who underwent S2P. Methods: A ten-question survey was distributed to the members of the Congenital Heart Surgeons’ Society (CHSS) and the European Congenital Heart Surgeons’ Association (ECHSA). Results were summarized using descriptive statistics. Surgeon-reported preferences were compared to clinical data from the CHSS Critical Left Ventricular Outflow Tract Obstruction (LVOTO) Registry and the Pediatric Heart Network Single Ventricle Reconstruction (SVR) database. Results: Overall, 38% (88 of 232) of surgeons from 74 institutions responded, of which 70% (62 of 88) were CHSS members and 30% (26 of 88) were ECHSA members. Surgeons reported performing S2P at a median of five months after stage 1 (interquartile range [IQR]: 4.5-6), with no difference between CHSS and ECHSA surgeons. Surgeons reported performing nonelective S2P at a median of 4.5 months after stage 1 (IQR: 3.5-5.5), again with no difference by society. No difference existed between the surgeon-reported preferences and patient data in the Critical LVOTO and SVR databases for the timing of elective (5 vs 5.1 vs 5.3 months, P = .19) or nonelective S2P (4.5 vs 4.6 vs 4.2 months, P = .06). Conclusion: There was a remarkable lack of variation in surgeon preferences regarding the timing of S2P. This may represent a natural standardization of practice across congenital heart surgery, which is notable, given the current lack of guidelines regarding the timing of S2P.

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.

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

Atrial fibrillation (AF) is the most common sustained cardiac arrhythmia and its prevalence is similar to 1-2% of the general population, but higher with increasing age and in patients with concomitant heart disease. The Cox-maze III procedure was a groundbreaking development and remains the surgical intervention with the highest cure rate, but due to its technical difficulty alternative techniques have been developed to create the lesions sets. The field is fast moving and there are now multiple energy sources, multiple potential lesion sets and even multiple guidelines addressing the issues surrounding the surgical treatment of AF both for patients undergoing this concomitantly with other cardiac surgical procedures and also as stand-alone procedures either via sternotomy or via videothoracoscopic techniques. The aim of this document is to bring together all major guidelines in this area into one resource for clinicians interested in surgery for AF. Where we felt that guidance was lacking, we also reviewed the evidence and provided summaries in those areas. We conclude that AF surgery is an effective intervention for patients with all types of AF undergoing concomitant cardiac surgery to reduce the incidence of AF, as demonstrated in multiple randomized studies. There is some evidence that this translates into reduced stroke risk, reduced heart failure risk and longer survival. In addition, symptomatic patients with AF may be considered for surgery after failed catheter intervention or even as an alternative to catheter intervention where either catheter ablation is contraindicated or by patient choice.

OBJECTIVE: The objective of this European multicenter study was to report surgical outcomes of Fontan takedown, Fontan conversion and heart transplantation (HTX) for failing Fontan patients in terms of all-cause mortality and (re-)HTX.METHODS: A retrospective international study was conducted by the European Congenital Heart Surgeons Association among 22 member centres. Outcome of surgery to address failing Fontan was collected in 225 patients among which were patients with Fontan takedown (n=38; 17%), Fontan conversion (n=137; 61%) or HTX (n=50; 22%).RESULTS: The most prevalent indication for failing Fontan surgery was arrhythmia (43.6%), but indications differed across the surgical groups (pCONCLUSIONS: Takedown surgery for failing Fontan is mostly performed in the early postoperative phase, with a high risk of mortality. There is no difference in survival after Fontan conversion or HTX.

OBJECTIVES: Management strategy of patients with tetralogy of Fallot (TOF)—including timing, as well as surgical technique—remains a controversial topic. We sought to analyse both early and late results of our consistent policy of non-neonatal transatrial/transpulmonary (TA/TP) repair of TOF over almost 14 years, in order to assess preservation or possible deterioration of right ventricular (RV) function. METHODS: All 245 consecutive patients with TOF, referred to our group for repair between September 1997 and December 2010, have been prospectively followed up. Their clinical and echocardiographic data were retrospectively analysed. All underwent complete TA/TP repair at a median age of 1.6 years (range 0.2 to 55.6 years). RESULTS: Follow-up (median 8.5 years, range 0.5 to 14.6 years) was 100% completed. There was no operative death. There were three early re-operations: one for residual right ventricular outflow tract obstruction (RVOTO), one for intractable arrhythmias and one for remote second ventricular septal defect (VSD). There were three non-cardiac-related late deaths. Actuarial survival at 14 years was 98.8% (242/245). Twenty five patients required late re-operation, including 23 patients (9.4%, 23/245) who had pulmonary valve replacement (PVR). All other patients remained asymptomatic at follow-up. Mean residual right ventricular outflow tract (RVOT) pressure gradients have remained stable (6.8 ± 6.6 mmHg, 95% CI 5.9–7.6, early postoperatively, versus 7.6 ± 7.5 mmHg, 95% CI 6.6–8.6 at followup; P= 0.015). The mean qualitative grade of pulmonary valve insufficiency (PVI) increased (from 1.4 ± 0.9, 95% CI 1.3–1.5 at discharge to 2.1 ± 1.2, 95% CI 2–2.3 at follow-up; P < 0.001). Similarly, mean qualitative grade of tricuspid valve insufficiency (TVI) also increased (from 1 ± 0.7, 95% CI 0.9–1 at discharge to 1.5 ± 0.8, 95% CI 1.4–1.6 at follow-up; P < 0.001). However, RV function has remained normal in most patients. No significant arrhythmias have been noted. CONCLUSIONS: Our management strategy of non-neonatal TA/TP repair for all patients with TOF is associated with minimal early and late mortality and morbidity, relatively low re-operation rate, preserved RV function and excellent clinical outcomes at follow-up over almost 14 years. Since many patients demonstrated progressive increase in PVI and TVI, a much longer follow-up is necessary to determine the ultimate rates of late re-operation for pulmonary valve replacement (PVR).

Tetralogy of Fallot (TOF) is the most common cyanotic congenital heart disease beyond 1 week of age. “Classic” TOF usually presents for surgical repair during the first 3–9 months of life. Surgical repair techniques vary among surgeons and institutions. The optimal timing and approach to complete (vs. staged) surgical repair remain a topic of continued debate in the modern surgical era. A pulmonary valve sparing or reconstruction surgical approach has become popularized over the past decade or so. The purpose of this chapter will be to review the morphologic and physiologic features of “classic” TOF with an emphasis on contemporary approaches to surgical treatment.

Objective: This study aims to analyse the collective experience of participating European Congenital Heart Surgeons Association centres in the surgical management of complications resulting from trans-catheter closure of atrial septal defects (ASDs). Methods: The records of all (n = 56) patients, aged 3-70 years (median 18 years), who underwent surgery for complications of trans-catheter ASD closure in 19 participating institutions over a 10-year period (1997-2007) were retrospectively reviewed. Risk factors for surgical complications were sought. Surgical outcomes were compared with those reported for primary surgical ASD closure in the European Association of Cardio-thoracic Surgery Congenital Database. Results: A wide range of ASD sizes (5-34 mm) and devices of various types and sizes (range 12-60 mm) were involved, including 13 devices less than 20 mm. Complications leading to surgery included embolisation (n = 29), thrombosis/thrombo-embolism/cerebral ischaemia or stroke (n = 12), significant residual shunt (n = 12), aortic or atrial perforation or erosion (n = 9), haemopericardium with tamponade (n = 5), aortic or mitral valve injury (n = 2) and endocarditis (n = 1). Surgery (39 early emergent and 17 late operations) involved device removal, repair of damaged structures and ASD closure. Late operations were needed 12 days to 8 years (median 3 years) after device implantation. There were three hospital deaths (mortality 5.4%). During the same time period, mortality for all 4453 surgical ASD closures reported in the European Association of Cardio-Thoracic Surgery Congenital Database was 0.36% ( p = 0.001). Conclusions: Trans-catheter device closure of ASDs, even in cases when small devices are used, can lead to significant complications requiring surgical intervention. Once a complication leading to surgery occurs, mortality is significantly greater than that of primary surgical ASD closure. Major complications can occur late after device placement. Therefore, lifelong follow-up of patients in whom ASDs have been closed by devices is mandatory. (C) 2010 European Association for Cardio-Thoracic Surgery. Published by Elsevier B.V. All rights reserved.

Objective: The valved bovine conduit “ Contegra” for RVOTreconstruction became availablefor clin- ical use within a 100% source data monitored and echo core lab controlled prospective European Multicentre Study, carried out from 1999 to 2006. We present the results of this study. Methods: A total of 165 Contegras were im- planted in 8 centres. The mean patient age was 3.9 years (2 days – 18 years, median 2.0). Total fol- low-up was 687 patient years. Diagnoses in- cluded: tetralogy of Fallot (64 patients, 39%), truncus arteriosus (50, 30%), double outlet right ventricle (16, 10%), aortic valve disease/Ross pro- cedure (11, 7%), pulmonary valve atresia (10, 6%), transposition of the great arteries (10, 6%), 4 oth- er malformations (2%). Previous procedures were: 82 patients (50%) – none; 37 (22%) – valved conduit implantation; 14 (8%) aortopulmonary shunt; 6 (4%) catheter intervention. Follow-up appointments which included standardised echo- cardiography investigations were scheduled at 1, 3, 6, and 12 months, then annually. We evaluated freedom from death, explantation, intervention, stenosis, insufficiency, and degeneration. Results were stratified by age, diagnosis group and con- duit size. Results: The 5-year freedom-from rates were: ex- plantation – 90% (for patients aged 1 to 10 years) and 68% (for younger patients); endocarditis – over 92%; catheter intervention – 74% (patients with congenital malformations); stenosis – 75% and more (any group); insufficiency – 50% (12 and 14 mm diameter conduits); any event – 13% (patients under 1 year), 58% (1 to 10 years), 82% (> 10 years). Trace or mild insufficiency was a fre- quent, but not progressive finding. Mild calcifica- tion was detected in only 8 examinations. Conclusions: The performance of the Contegra conduit compares well with that of homografts when used to reconstruct paediatric right ventric- ular outflow tracts. " heart valve surgery l " right ventricular outflow tract l" bovine valved conduit l" longterm followup l" homograft

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.

Medical schools teach their students about anatomy, histology, cell biology, genetics, embryology, physiology, biochemistry, pathophysiology, pharmacology, and other subdivisions of the medical knowledge and know-how. Despite acquiring this complex science and having the opportunity to interact with patients in the clinical setting, very rarely do medical students gather a global understanding of what impact a disease represents on a personal, societal and global level. Congenital heart disease (CHD) is a condition that is present in approximately 1 in 100 births, has a remarkable heterogeneity and complexity and has a significant impact on the child’s survival and quality of life if left untreated. Even when the initial congenital defect is successfully repaired, patients with CHD require a lifelong follow-up and the possibility of subsequent reinterventions. Physicians are involved from the beginning to the end with the diagnosis, counselling, every step of the treatment and the lifelong follow-up. The treatment of CHD requires an enormous effort, resources and the participation of a huge team made up of a multitude of highly trained medical specialists, such as cardiac surgeons, cardiologists, cardiac anaesthetists, cardiac intensivists and many others, who often have dedicated their entire professional lives to this exciting field of medicine. Far from claiming to be a thorough and exhaustive expose on CHD, this article rather aims to present to medical students from everywhere in the world an overview of the nature of this important disease and of its global impact. We will provide a definition of CHD, highlight a few examples, give a summary of its epidemiology, and finally summarize the history of the study and treatment modalities. Our focus will also be on the inequalities in the treatment of CHD between the western world and developing nations. Finally, the challenges that must be addressed to truly improve care around the world will be presented.

The optimal anticoagulation following Fontan operation and its modifications remain controversial and it is even less well defined as regards patients with inherited thrombophilia. We present a case of a child with bidirectional Glenn anastomosis for double inlet left ventricle that suffered a stroke despite aspirin prophylaxis; the patient was combined homozygous for prothrombin G20210A mutation and for methylenetetrahydrofolate reductase C677T mutation as well. The family history was positive for fetal loss and premature cardiovascular disease. Large-scale studies are needed to evaluate whether carriers of thrombophilia mutations need more intense thromboprophylaxis.

Catheter Ablation of Ligament of Marshall Tissue. Introduction: The ligament of Marshall is a left atrial neuromuscular bundle with sympathetic innervation that may be a source of atrial e brillation (AF)-inducing automatic activity. Methods and Results: Twenty-four patients with paroxysmal AF (including 18 with adrenergic AF) and 25 with other arrhythmias underwent catheter mapping. In cases of adrenergic AF, radiofrequency ablation was attempted when Marshall potentials were recorded. Patients were followed for 2 months before and 11.2 6 4.2 months after the procedure. Catheterization of the distal superoposterior coronary sinus was feasible in 14 patients with AF (10 with adrenergic AF) and 12 patients without AF. A discrete Marshall potential was recorded in 12 patients with AF versus 3 patients without AF (P 5 0.004). In 10 patients with adrenergic AF, this potential followed the atrial electrogram during sinus rhythm by 26 6 5 msec on left atrial recordings and 24 6 4 msec on coronary sinus recordings, and preceded it during atrial ectopy by 29 6 5 msec and 26 6 5 msec, respectively. It was abolished by epicardial (n 5 1), endocardial (n 5 4), or combined epicardial and endocardial ablation (n 5 5). Seven patients with ablation showed signie cant reductions in adrenergic AF, whereas no signie cant change was seen in 8 adrenergic AF patients not undergoing ablation (P 5 0.004). No improvement was seen in 3 of 4 patients with only endocardial ablation, whereas all 6 patients with epicardial ablation improved (P 5 0.033). Conclusion: Recording of Marshall potential is feasible in patients with paroxysmal AF. Combined epicardial and endocardial catheter ablation of ligament of Marshall tissue may reduce the paroxysms of

We report a case of congenital atresia of the superior vena cava (SVC) with stenotic anastomoses between systemic and pulmonary veins, resulting in cyanosis and symptoms consistent with SVC syndrome in an infant.

Background . Despite improved surgical results for truncus arteriosus, overall mortality rates, remain higher than those reported for other complex congenital heart diseases, especially with truncal valve regurgitation or an interrupted aortic arch. Methods . Seventeen patients had complete repair of the truncus arteriosus at the Cleveland Clinic Foundation between August 1993 and June 1997. The age at operation ranged from 2 days to 4.5 years. Associated abnormalities included interrupted aortic arch in 3 patients and abnormal coronary artery anatomy in 3. Four patients had more than moderate truncal valve insufficiency requiring concomitant truncal valve repair. Results . There were no early deaths and only one late death at a mean follow-up of 24 months. The death occurred 3 months postoperatively and resulted from refractory pulmonary vascular obstructive disease in a patient who was referred at 1 year of age. Reoperation was required in 4 patients. Conclusions . Even in the presence of associated anomalies complete repair was performed with a low mortality rate. Truncal valve repair can be performed safely in the neonate with good results.

Junctional ectopic tachycardia is usually a limited but potentially life-threatening postoperative arrhythmia. We describe a case of malignant postoperative junctional ectopic tachycardia in a 13-month-old child who had undergone transatrial and transpulmonary repair of tetralogy of Fallot resistant to all conventional therapeutic measures and complicated by recurrent ventricular tachycardia. The arrhythmia was finally treated by open surgical cryoablation of the atrioventricular node and the implantation of a permanent pacemaker. The patient made an uneventful recovery followed by good long-term outcome.

Objective: To retrospectively examine a 4 year policy of restoring the morphologically left ventricle to the systemic circuit in patients presenting after 3 months of age with ventriculo-arterial discordance with or without associated atrio-ventricular discordance. This policy was stimulated by thc known tendency of the morphologically right ventricle to develop dysfunction sooner or later when left in the systemic circuit. Such a policy dictates a more complex surgical approach and, at this point. it remains controversial whether or not the increased surgical complexity is warranted. Methods: From July 1, 1993 to March 31, 1997, a total of 29 patients were entered into a protocol for placement of the morphologically left ventricle into the systemic circuit. Three groups of patients were identified. Group I: congenitally corrected transposition in 14 patients - were treated with either a Senning plus arterial switch operation or Senning plus Rastelli procedure. Group II ; failed atrial switch procedure in 12 patients of which nine proceeded to arterial switch operation with Senning or Mustard takedown and atrial reseptation. Group III; D-transposition of the great vessels presenting more than I year after birth in three patients who underwent arterial switch operation alone. A deconditioned morphologically left ventricle required reconditioning by means of preparatory pulmonary artery banding in 17 of 29 patients. In the patients requiring pulmonary artery banding, an average of 2.1 pulmonary artery bandings was required to prepare the morphologically left ventricle for a systemic pressure workload. Results: In those patients with a deconditioned morphologically left ventricle requiring preparatory pulmonary artery banding, the mean ratio between the left ventricular and right ventricular systolic pressure increased from 0.48 to 0.95. The left ventricular mass increased from 46.6 to 81.8 g/m 2 in five patients subjected to serial MRI measurement. Three patients failed the preparatory pulmonary artery banding and did not proceed to anatomical correction. Two subsequently died at a later time. In the patients proceeding to complete anatomical correction: group I - there were no early or late deaths. Two patients required pacemaker implantation post-operatively. Group II - there were two in-hospital deaths, one early due to intrapulmonary hemorrhage and one late. secondary to postoperative left ventricular failure with a stormy post-operative course requiring successful ECMO placement and weaning. These patients were 18 and 25 years old, respectively. One patient proceeded to cardiac transplantation 3 months after surgery due to ongoing morphologically left and right ventricular dysfunction. Group III - all patients continue to do well. Conclusions: Late anatomic correction of ventriculo-arterial discordance with or without atrio-ventricular discordance can be performed at a relatively low risk. Reconditioning of the morphologically left ventricle can be achieved by sequential pulmonary banding but is not without risk. Failure to achieve adequate reconditioning of the morphologically left ventricle by pulmonary artery banding in the older patient probably increases the risk of non-survival and may be offset by timely transplantation. Longer follow-up and an assessment of the functional status of these patients is required to assess whether or not this complex surgical approach is indeed warranted.

Anomalous origin of the left coronary from the pulmonary artery (ALCAPA) is a rare congenital malformation, which may result in myocardial infarction, congestive heart failure, and sudden death if left untreated. Despite frequently advanced pathologic changes, there seems to be significant potential for the recovery of myocardial function in individuals with left ventricular dysfunction after the establishment of physiologic coronary circulation, particularly in the pediatric population. Reports of ALCAPA repair in adulthood are scarce and little information exists regarding the response of the left ventricle to revascularization in this age group. In this report, repair of ALCAPA in a significantly symptomatic adult patient with ventricular dysfunction is described, leading to an early recovery of left ventricular function.

Repair of scimitar syndrome presenting in infancy involves either tunneling or reimplantation of the anomalous vein to the left atrium and may be fraught with serious complications such as thrombosis and secondary pulmonary infarction necessitating pneumonectomy. The authors present the case of a severely symptomatic infant with scimitar syndrome, managed initially with closure of an atrial septal defect in the hope of avoiding a repair with considerable risk of scimitar vein thrombosis and pulmonary infarction. Despite initial clinical improvement, subsequent rapid development of spontaneous massive emphysematous degeneration of the right lung necessitated emergency pneumonectomy, which was accomplished via the median sternotomy approach.

Objectives. Postcardiotomy acute severe heart failure cannot be managed by medical treatment alone and most often requires some form of mechanical support. In this study we evaluate the efficacy of postoperative extracorporeal membrane oxygenation (ECMO) support following surgery for congenital heart disease (CHD) in infants and children. Methods. Over a 6-year period from October 1997 to October 2003, 10 patients aged 5 days to 28.5 months (median 3 months) who underwent surgical procedures for CHD received postoperative mechanical support for failing cardiac function despite optimal medical therapy. In 3 patients ECMO was instituted in the operating room (OR) and in 7 patients this was introduced in the intensive care unit (ICU) 2 to 48 (median 20) hours postoperatively. Results. Four patients (40%) were successfully weaned, while support was withdrawn in the remaining 6 due to irreversible vital organ damage. Following successful weaning, one of the survivors died 8 hours later from barotrauma and intrapulmonary hemorrhage, and another died 4 months later from persistent heart failure. The other two patients remain well in NYHA class II. Conclusions. Despite the adverse effects of ECMO, the methodology provided the necessary support and allowed the failing heart to recover in a number of patients where inotropic support alone proved inadequate.

OBJECTIVES: Right ventricular (RV) dysfunction and pulmonary insufficiency (PI) after tetralogy of Fallot (TOF) repair may contribute to early and late morbidity and mortality. RV dysfunction may be related to the ventriculotomy employed in the transventricular repair technique, particularly when it is combined with a transannular patch (TAP). Transatrial/transpulmonary (TA/TP) repair without ventriculotomy has been advocated as a method potentially diminishing such adverse events. However, the prevalence and early as well as the late results of these different surgical approaches in Europe have not been studied. To ascertain ‘the current prevalence and associated early mortality’ of various surgical approaches for repair of TOF, relevant data in the European Association for Cardio-Thoracic Surgery Congenital Database were analysed. METHODS: The study population was all types of reparative operations (n= 6654) for patients with primary diagnosis of TOF reported between 1999 and 2011. RESULTS: Overall hospital mortality (HM) was 2.58% (172 of 6654). Repair via ventriculotomy with TAP was the most prevalent technique (n = 3827, 57.5%), with HM 3.11%. Repair via ventriculotomy with non-TAP was performed in 1309 patients (19.7%, HM = 1.53%). Repair without ventriculotomy was performed in 1214 patients (18.2%, HM = 1.48%). Logistic regression analysis showed statistically significant association between HM risk and the type of surgery. In particular, ventriculotomy with TAP is associated with increased mortality risk significantly compared with ventriculotomy with non-TAP (crude odds ratio [OR] 2.13 (95% confidence interval [CI]: 1.33– 3.63). Similar results were obtained by analysing for operative mortality risk (30-day mortality, OM). Operations that have been performed before 2005 have resulted in increased surgical risk compared with those performed after 2005 (ORs for OM 1.45, 95% CI: 1.03–2.013). CONCLUSIONS: Overall HM for TOF repair is low. TOF repair by means of ventriculotomy with TAP is the most prevalent approach and is associated with higher mortality. Repair with ventriculotomy but no TAP and repair without ventriculotomy are both less prevalent and with lower mortality. Surgical risk appears to be decreasing over time.

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.

We analyzed our experience with 496 patients who underwent primary cardiac transplantation since the introduction of cyclosporine immunosuppression (Dec. 16, 1980, to Jan. 7, 1993). There were 388 male and 108 female patients. Mean recipient age was 40 ± 16 years (range 0.1 to 70 years, median 44 years). Recipient diagnoses included coronary disease in 188, idiopathic cardiomyopathy in 196, viral cardiomyopathy in 35, and congenital heart disease in 28 patients. Donor age was 25 ± 10 years (range 1 to 53 years, median 24 years). Graft ischemic time was 148 ± 57 minutes (range 38 to 495 minutes, median 149 minutes). Operative mortality (hospital death) rate was 7.9% ± 1.3% (70% confidence intervals). Multivariate logistic regression analysis revealed that (higher) pulmonary vascular resistance and gender (female) were the only independent predictors of hospital death ( p p p

The Contegra® bioprosthetic valved conduit, a glutaraldehyde-preserved valve-containing bovine jugular vein graft (Contegra, Medtronic Inc., Minneapolis MN, USA) introduced for clinical trials in 1998, is used for reconstruction of the right ventricular outflow tract (RVOT), mainly in children. This study evaluates our surgical experience with the Contegra® graft, emphasizing the assessment of conduit durability at mid-term follow up.The intermediate results of RVOT reconstruction utilizing the Contegra conduit were retrospectively analyzed in a series of 34 consecutive patients (25 male, 9 female), with a mean age of 10.9 ± 11.2 years (range 0.2-46 years). Included were 14 patients with tetralogy of Fallot (TOF) with pulmonary atresia, 11 with reoperation of previously corrected TOF, 5 with truncus arteriosus, 2 with TOF with absent pulmonary valve, 1 reoperation of previously repaired double outlet right ventricle with pulmonary atresia, and 1 undergoing a Ross procedure. Contegra conduit sizes varied in diameter between 12 and 22 mm (mean 18.3 ± 3.2 mm).There were no hospital deaths. There was one early conduit replacement as a result of recurrent thrombosis. Four patients developed early thrombus formation in a valve cusp with complete resolution following anticoagulation therapy. At mean follow up of 85 months (range 6-136 months) and median follow up of 95 months, one patient required Contegra graft explantation in another institution (indications unknown). Freedom from reoperation for Contegra grafts was 94% at 11.4 years. Mean transpulmonary pressure gradients remained low (9.6 ± 5.3 mmHg postoperative, 19.6 ± 10.6 mmHg at follow up). Although there was a clear trend towards worsening of conduit valve insufficiency, this was neither statistically significant nor considered clinically so.In our experience of 34 consecutive operations, the Contegra® valved conduit for RVOT reconstruction seems to be a reliable alternative to homograft conduits, with promising mid-term freedom from structural deterioration and reoperation.

Background— Scimitar syndrome is a rare congenital heart disease. To evaluate the surgical results, we embarked on the European Congenital Heart Surgeons Association (ECHSA) multicentric study. Methods and Results— From January 1997 to December 2007, we collected data on 68 patients who underwent surgery for scimitar syndrome. Primary outcomes included hospital mortality and the efficacy of repair at follow-up. Median age at surgery was 1.4 years (interquartile range, 0.46 to 7.92 years). Forty-four patients (64%) presented with symptoms. Surgical repair included intraatrial baffle in 38 patients (56%; group 1) and reimplantation of the scimitar vein onto the left atrium in 21 patients (31%; group 2). Eight patients underwent right pneumectomy, and 1 had a right lower lobe lobectomy (group 3). Four patients died in hospital (5.9%; 1 patient in group 1, 2.6%; 3 patients in group 3, 33%). Median follow-up time was 4.5 years. There were 2 late deaths (3.1%) resulting from severe pulmonary arterial hypertension. Freedom from scimitar drainage stenosis at 13 years was 83.8% in group 1 and 85.8% in group 2. Four patients in group 1 were reoperated, and 3 patients (2 in group 1 [6%] and 1 in group 2 [4.8%]) required balloon dilation/stenting for scimitar drainage stenosis. Conclusions— The surgical treatment of this rare syndrome is safe and effective. The majority of patients were asymptomatic at the follow-up control. There were a relatively high incidence of residual scimitar drainage stenosis that is similar between the 2 reported corrective surgical techniques used.

The Fontan operation (modified from its original version) affords excellent palliation for many patients with various forms of anatomic or functional single ventricle. The purpose of this study was to evaluate the outcome of our experience with the Fontan procedure in Greece.Fifty-eight consecutive patients with single ventricle physiology had a modified Fontan operation between 1997 and 2009. Their records were reviewed retrospectively. Follow up, which included clinical evaluation and echocardiographic functional assessment, was complete.Median age at operation was 5.7 years (range 3 years to 29.4 years); 46.5% had multistage palliation (stage I and II); 79% had prior bidirectional cavopulmonary shunt (stage II) and 8.6% single stage Fontan. Fifty-four patients had an extracardiac conduit total cavopulmonary connection (EC-TCPC) and 4 an intra-atrial lateral tunnel (LT-TCPC). Fenestration was performed in 26 (44.8%) patients. Operative mortality was 0%. One patient required a short period of extracorporeal membrane oxygenator support. The most frequent complication was prolonged pleural effusion. Median duration of pleural effusion was 17 days (range 6-47 days). Median duration of follow up was 5.44 years (range 0.36 to 11.5 years). There were 2 late deaths (overall mortality 3.4%). One patient died from progressive deterioration of ventricular function within 2 years of operation and the other from fulminant endocarditis. Ten subjects have undergone device closure of a persistent fenestration. All 56 surviving patients are in excellent clinical condition (NYHA class I or II).We have performed the Fontan procedure over a period of 13 years in Greece with excellent mid- and long-term results. Longer follow up will be necessary to assess the possible incidence of late severe complications, some of which may necessitate heart transplantation.

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. (J Card Surg 2010;25:214-217)

We evaluated subepicardial and subendocardial two-dimensional finite deformations in the left ventricular (LV) anterior, lateral, and posterior regions in the closed-chest, conscious dog heart. Eight dogs underwent placement of 22 radiopaque markers in the LV myocardium. Sets of three markers were implanted in the anterior, lateral, and posterior subepicardium and subendocardium at the mid-ventricular level; reference markers were placed at apical and basal sites. Eight hours later, biplane videofluoroscopy was performed. Finite deformations for each subepicardial and subendocardial region were analyzed during three consecutive beats at end expiration. Circumferential shortening occurred in all layers and regions; similarly, longitudinal shortening occurred in all layers except that of the posterior endocardium. Values of principal strain were -0.19 +/- 0.08 (SD) and -0.10 +/- 0.03 for the anterior subendocardium and subepicardium, -0.20 +/- 0.07 and -0.10 +/- 0.02 for the lateral subendocardium and subepicardium, and -0.13 +/- 0.02 and -0.10 +/- 0.03 for the posterior subendocardium and subepicardium respectively (P less than 0.05 subendocardium vs. subepicardium). Second principal strain tended to be near zero or positive (from -0.01 +/- 0.05 to 0.04 +/- 0.05) in all regions. The end-systolic direction of principal strain was -29 +/- 32 degrees and -34 +/- 29 degrees in the anterior subepicardium and subendocardium, -47 +/- 10 degrees and -30 +/- 37 degrees in the lateral subepicardium and subendocardium, and -4 +/- 29 degrees and +7 +/- 23 degrees in the posterior subepicardium and subendocardium. Anterior and lateral directions of principal strain were similar in the subepicardial and subendocardial layers and oriented along the epicardial fiber axis, but the posterior direction tended to be circumferentially oriented.(ABSTRACT TRUNCATED AT 250 WORDS)

The diagnosis and treatment for paediatric and congenital cardiac disease has undergone remarkable progress over the last 60 years. Unfortunately, this progress has been largely limited to the developed world. Yet every year approximately 90% of the more than 1,000,000 children who are born with congenital cardiac disease across the world receive either suboptimal care or are totally denied care.While in the developed world the focus has changed from an effort to decrease post-operative mortality to now improving quality of life and decreasing morbidity, which is the focus of this Supplement, the rest of the world still needs to develop basic access to congenital cardiac care. The World Society for Pediatric and Congenital Heart Surgery [http://www.wspchs.org/] was established in 2006. The Vision of the World Society is that every child born anywhere in the world with a congenital heart defect should have access to appropriate medical and surgical care. The Mission of the World Society is to promote the highest quality comprehensive care to all patients with pediatric and/or congenital heart disease, from the fetus to the adult, regardless of the patient’s economic means, with emphasis on excellence in education, research and community service.We present in this article an overview of the epidemiology of congenital cardiac disease, the current and future challenges to improve care in the developed and developing world, the impact of the globalization of cardiac surgery, and the role that the World Society should play. The World Society for Pediatric and Congenital Heart Surgery is in a unique position to influence and truly improve the global care of children and adults with congenital cardiac disease throughout the world [http://www.wspchs.org/].