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Lymphatic failure is a broad term that describes the lymphatic circulation's inability to adequately transport fluid and solutes out of the interstitium and into the systemic venous circulation, which can result in dysfunction and dysregulation of immune responses, dietary fat absorption, and fluid balance maintenance. Several investigations have recently elucidated the nexus between lymphatic failure and congenital heart disease, and the associated morbidity and mortality is now well-recognized. However, the precise pathophysiology and pathogenesis of lymphatic failure remains poorly understood and relatively understudied, and there are no targeted therapeutics or interventions to reliably prevent its development and progression. Thus, there is growing enthusiasm towards the development and application of novel percutaneous and surgical lymphatic interventions. Moreover, there is consensus that further investigations are needed to delineate the underlying mechanisms of lymphatic failure, which could help identify novel therapeutic targets and develop innovative procedures to improve the overall quality of life and survival of these patients. With these considerations, this review aims to provide an overview of the lymphatic circulation and its vasculature as it relates to current understandings into the pathophysiology and pathogenesis of lymphatic failure in patients with congenital heart disease, while also summarizing strategies for evaluating and managing lymphatic complications, as well as specific areas of interest for future translational and clinical research efforts., (Copyright © 2024 Elsevier Inc. All rights reserved.)

Introduction: As the adult Fontan population with Fontan associated liver disease continues to increase, more patients are being referred for transplantation, including combined heart and liver transplantation., Methods: We report updated mortality and morbidity outcomes after combined heart and liver transplant in a retrospective cohort series of 40 patients (age 14 to 49 years) with Fontan circulation across two centers from 2006-2022., Results: The 30-day, 1-year, 5-year and 10-year survival rate was 90%, 80%, 73% and 73% respectively. Sixty percent of patients met a composite comorbidity of needing either post-transplant mechanical circulatory support, renal replacement therapy or tracheostomy. Cardiopulmonary bypass time > 283 min (4.7 h) and meeting the composite comorbidity were associated with mortality by Kaplan Meier analysis., Conclusion: Further study to mitigate early mortality and the above comorbidities as well as the high risk of bleeding and vasoplegia in this patient population is warranted., (© 2024 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.)

Background: Surgeons in training for congenital cardiac surgery face considerable challenges owing to procedure complexity, closely scrutinized outcomes, and a steep learning curve. Simulation methods have been initiated in other surgical specialties, but have yet to be established for congenital cardiac surgery trainees. The purpose of this study was to assess high-fidelity simulation as a method to train and improve skills of resident trainees learning critical components of index congenital cardiac surgical procedures., Methods: Using 5 neonatal piglets over a period of 2.5 days, the following procedures were simulated: Norwood procedure, arterial switch operation, neonatal Ross procedure, tetralogy of Fallot repair, systemic to pulmonary artery shunt procedures, transmediastinal coarctation repair, atrial septal defect repair, ventricular septal defect repair, and right ventricular to pulmonary artery conduit. Anastomoses were tested with saline, all procedures were timed and video recorded, and resident trainee techniques and skills were critiqued by the instructor., Results: All aspects of the procedures were simulated with minimal modifications. Anastomoses were tested, and the procedure successfully replicated without the pressures of operative time. Operative techniques involving suture placement in neonatal tissue, depth perception, and patch size estimation were corrected in real time, resulting in observed improvement of surgical skills. Video review allowed for further pedagogic value through examination and documentation of competency., Conclusions: This neonatal porcine simulation model allows surgical trainees in congenital heart surgery to make and correct mistakes in a safe and controlled learning environment without compromising patient safety, thereby fostering surgeon competence and confidence., (Copyright © 2018 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: We have previously shown that the minor alleles of vascular endothelial growth factor A (VEGFA) single-nucleotide polymorphism rs833069 and superoxide dismutase 2 (SOD2) single-nucleotide polymorphism rs2758331 are both associated with improved transplant-free survival after surgery for CHD in infants, but the underlying mechanisms are unknown. We hypothesised that one or both of these minor alleles are associated with better systemic ventricular function, resulting in improved survival., Methods: This study is a follow-up analysis of 422 non-syndromic CHD patients who underwent neonatal cardiac surgery with cardiopulmonary bypass. Echocardiographic reports were reviewed. Systemic ventricular function was subjectively categorised as normal, or as mildly, moderately, or severely depressed. The change in function was calculated as the change from the preoperative study to the last available study. Stepwise linear regression, adjusting for covariates, was performed for the outcome of change in ventricular function. Model comparison was performed using Akaike's information criterion. Only variables that improved the model prediction of change in systemic ventricular function were retained in the final model., Results: Genetic and echocardiographic data were available for 335/422 subjects (79%). Of them, 33 (9.9%) developed worse systemic ventricular function during a mean follow-up period of 13.5 years. After covariate adjustment, the presence of the VEGFA minor allele was associated with preserved ventricular function (p=0.011)., Conclusions: These data support the hypothesis that the mechanism by which the VEGFA single-nucleotide polymorphism rs833069 minor allele improves survival may be the preservation of ventricular function. Further studies are needed to validate this genotype-phenotype association and to determine whether this mechanism is related to increased vascular endothelial growth factor production.

The treatment of rare and expensive medical conditions is one of the defining qualities of paediatric cardiology and congenital heart surgery. Increasing concerns over healthcare resource allocation are challenging the merits of treating more expensive forms of congenital heart disease, and this trend will almost certainly continue. In this manuscript, the problems of resource allocation for rare and expensive medical conditions are described from philosophical and economic perspectives. The argument is made that current economic models are limited in the ability to assess the value of treating expensive and rare forms of congenital heart disease. Further, multi-disciplinary approaches are necessary to best determine the merits of treating a patient population such as those with significant congenital heart disease that sometimes requires enormous healthcare resources.

The tricuspid valve is being increasingly recognised as an important safeguard to the heart with congenital heart disease. Both structural anomalies of the valve and functional burdens from other malformations of the right heart can lead to major haemodynamic consequences both upstream and downstream. The indications to surgically intervene on the tricuspid valve are evolving and vary depending on the malformation. The extant surgical techniques and their applications to corresponding frequent congenital anomalies of the tricuspid valve are reviewed.

Right-sided aortic arch with an aberrant left subclavian artery is a rare aortic arch anomaly. Although usually asymptomatic, aneurysm formation, dissection, and rupture can occur due to the aberrant vasculature and can be life-threatening. Hybrid, endovascular techniques have been implemented in instances of elective repair of aneurysmal diverticula of Kommerell in similar anatomical settings, but little has been written regarding urgent cases of rupture. We report a case of ruptured right-sided aortic arch with an aberrant left subclavian artery arising from a diverticulum of Kommerell successfully treated with hybrid aortic debranching and thoracic endovascular aortic stenting., (© The Author(s) 2014.)

Background: Informed consent refers to the process by which physicians and patients engage in a dialogue to explain and comprehend the nature, alternatives, and risks of a procedure or course of therapy. The goal of this study is to better "inform the process of informed consent" by offering empirically derived procedural complication lists that provide objective contemporary data that surgeons may share with patients and families., Methods: The Society of Thoracic Surgeons Congenital Heart Surgery Database was queried for complications for 12 congenital heart operations (2010 to 2011) performed across all Society of Thoracic Surgeons-European Association of Cardio-Thoracic Surgery Congenital Heart Surgery (STAT) risk categories., Results: The 12 index procedures reviewed for rates of complications were repair of atrial septal defect (ASD), ventricular septal defect (VSD), atrioventricular septal defect (AVSD), tetralogy of Fallot (TOF), coarctation, and truncus arteriosus, as well as arterial switch operation (ASO), ASO-VSD, BiGlenn, Fontan, Norwood procedure, and systemic to pulmonary artery (S-P) shunt. Arrhythmia was the most frequent complication for VSD (5.8%), TOF (8.9%), and AVSD (14.7%) repairs. There was a high rate of sternum left open (planned, unplanned) for ASO (26%, 7%), ASO-VSD (29%, 10%), truncus repair (41%, 11%), and Norwood (63%, 7%). The most frequent complications for other procedures include ASD (unplanned readmission, 1.9%), BiGlenn (chylothorax, 7%), Fontan (pleural effusion, 16%), S-P shunt (reintubation, 10.6%), and coarctation (reintubation, 5.2%)., Conclusions: The informed consent process for congenital heart surgery may be served by accurate contemporary data on occurrence of complications. While a threshold rate of occurrence of individual complications may guide the physician, rare but important debilitating complications should also be discussed irrespective of frequency. We propose to better inform the process of informed consent by providing objective complications data., (Copyright © 2014 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

The arterial switch operation is the extant surgical correction after a long series of palliations attempted and/or successfully achieved for the treatment of discordant ventriculoarterial connections. As early as 1954, pioneers such as Mustard, Bailey, Kay, and Idriss led the way with at first disheartening failures, temporarily leading to abandoning the procedure. The first successful atrial baffle procedure in 1958 established itself as the procedure of choice for treating discordant ventriculoarterial connections, but tenacity, courage, and vision to pursue anatomic correction finally led to the first successful arterial switch in 1975 by Jatene. After a decade to perfect surgical technique and timing indications for the various anatomic subtypes, the new era of the neonatal arterial switch since the late 1980s set the very high standards that we all know and expect today. Despite excellent early and long-term survival, important residual lesions are increasingly being recognised. Expected anatomic residuals include supravalvar pulmonary stenosis, neoaortic valve insufficiency, and coronary ostial stenosis. Reinterventions and rare, but challenging surgical reoperations address these residual findings with satisfactory outcomes. Quality of life into young adulthood is satisfactory, but functional problems include reduced exercise capacity, diffuse coronary insufficiency, and neurodevelopmental shortcomings, of which the true incidence and potential clinical implications are still unknown. The arterial switch is a spectacular anatomic correction for a once lethal condition and currently the best surgical solution for patients with discordant ventriculoarterial connections. It is, however, far from a true cure; closer and ongoing follow-up for future care will continue to be required.

Significant challenges face developing countries as a result of the maldistribution of access to healthcare throughout the world, specifically access to paediatric cardiac care. Sustainable paediatric cardiac programmes must be established in developing countries to provide care to all children with congenital heart disease. Education and research are essential components to sustainable paediatric cardiac programmes in developing countries to define local problems and the incidence of disease, and to generate solutions thereto related. Research can contribute to developing local expertise, improving technology, providing opportunities for local talent, generating financial resources, enhancing the dignity of people, and the facilitating resolution of health problems throughout the world. Clinical trials conducted in developing countries should meet the same ethical standards as trials based in developed countries.

Significant advances have been made in extracorporeal life support, which has resulted in the increased use of post-cardiotomy extracorporeal membrane oxygenation. Retrospective studies have contributed to the ongoing evolution of selection criteria for post-cardiotomy extracorporeal membrane oxygenation. Current indications include failure to wean from cardiopulmonary bypass, haemodynamic collapse, pulmonary hypertension, post-repair of hypoplastic left heart syndrome, or need for bridge to transplantation. Short- and mid-term results are improving. Ethical concerns still attend the process, however. Moral risks related to post-cardiotomy extracorporeal membrane oxygenation may be encountered before, during, and after the open heart procedure. At each stage of the decision-making process, moral risks are encountered by many factors that may result in decisions that may be contrary to the best interests of the patient, parents, or use of shared societal resources. These moral risks centre around the selection process, informed consent, decision making in the operating room, and post-operative maintenance of extracorporeal membrane oxygenation. Consideration of such risks is affected by questions of haemodynamic stability, haematologic compromise, neurologic status, and family concerns. We conclude that thorough understanding of the relevant scientific literature, heightened awareness of moral risks, and incorporation of ethical tenets in clinical deliberation will guide the clinician to do the right thing.

Although recent advances have helped identify cases where foetal cardiac surgery might reverse the development of certain lesions, the indications and measurement of success in these procedures have yet to be established. Thus, both patients and physicians have a "burden of knowledge", whereby a diagnosis is made without a clear course of action. The profound issues raised by foetal intervention, specifically the question of how concepts such as "patient" and "success" can be used, complicate this burden further and test the limits of language and logic. Similar issues raised in postmodern philosophy are discussed and can be incorporated into foetal cardiac surgery dialogues to produce a multi-disciplinary approach that will elucidate, not obfuscate, these issues in the future.