In recent issues of the Journal of the Society for Cardiovascular Angiography and Interventions and the Journal of the American College of Cardiology: Cardiovascular Interventions, Holzer and colleagues presented an Expert Consensus Document titled: “PICS / AEPC / APPCS / CSANZ / SCAI / SOLACI: Expert consensus statement on cardiac catheterization for pediatric patients and adults with congenital heart disease.” This Expert Consensus Document is a massively important contribution to the community of paediatric and congenital cardiac care. This document was developed as an Expert Consensus Document by the Pediatric and Congenital Interventional Cardiovascular Society, the Association for European Paediatric and Congenital Cardiology, the Asia-Pacific Pediatric Cardiac Society, the Cardiac Society of Australia and New Zealand, the Society for Cardiovascular Angiography and Interventions, and the Latin American Society of Interventional Cardiology, as well as the Congenital Cardiac Anesthesia Society and the American Association of Physicists in Medicine.

As perfectly stated in the Preamble of this Expert Consensus Document, “This expert consensus document is intended to inform practitioners, payors, hospital administrators and other parties as to the opinion of the aforementioned societies about best practices for cardiac catheterisation and transcatheter management of paediatric and adult patients with congenital heart disease, with added accommodations for resource-limited environments.” And, the fact that the authorship of this Expert Consensus Document includes global representation is notable, commendable, and important.

This Expert Consensus Document has the potential to fill an important gap for this patient population. National guideline documents for specific aspects of interventions in patients with paediatric heart disease, including training guidelines, do exist. However, this current Expert Consensus Document authored by Holzer and colleagues provides truly globally applicable standards on cardiac catheterisation for both paediatric patients and adults with congenital heart disease (CHD).

Our current Editorial provides different regional perspectives from senior physicians dedicated to paediatric and congenital cardiac care who are practicing in Europe, the Asia-Pacific region, Latin America, Australia/New Zealand, and North America. Establishing worldwide standards for cardiac catheterisation laboratories for children and adults with CHD is a significant stride towards improving the quality and consistency of care. These standards should not only reflect the current state of medical knowledge but should also be adaptable to future advancements, ultimately fostering better outcomes and enhancing the lives of individuals affected by CHD worldwide.

Ensuring that these standards are accessible and adaptable across different healthcare settings globally is a critical step. Given the variability in resources and infrastructure globally, the need exists for flexibility and tailoring to implement recommendations.

The potential impact of the Expert Consensus Document and its recommendations is likely significant, but heterogeneity of healthcare systems will pose continuing challenges on healthcare professionals. Indeed, this heterogeneity of healthcare systems will challenge healthcare professionals to finally close the gap between acceptable and ideal in the catheterisation of patients with paediatric and/or congenital heart disease.

Surgical repair of channels between the ventricles is enhanced when the surgeon knows precisely where to place a patch, or baffle, so as to restore septal integrity. The paediatric cardiologist should provide the necessary information. Communication will be enhanced if the same words are used to account for the structures in question. Currently, however, the same term, namely “ventricular septal defect,” is used to account for markedly different areas within the heart. Closure of perimembranous defects found in hearts with concordant or discordant ventriculo-arterial connections restores the integrity of the ventricular septum, at the same time separating the systemic and pulmonary blood streams. When both arterial trunks arise from the right ventricle, in contrast, the surgeon when placing a baffle so as to separate the blood streams, does not close the channel most frequently described as the “ventricular septal defect.” In this review, we show that the perimembranous lesions as found in hearts with concordant or discordant ventriculo-arterial connections are the right ventricular entrances to the areas subtended beneath the hinges of the leaflets of the aortic or pulmonary valves. When both arterial trunks arise from the right ventricle, and the channel between the ventricles is directly subaortic, then the channel termed the “ventricular septal defect” is the left ventricular entrance to the comparable space subtended beneath the aortic root. We argue that recognition of these fundamental anatomical differences enhances the appreciation of the underlying morphology of the various lesions that reflect transfer, during cardiac development, of the aortic root from the morphologically right to the morphologically left ventricle.

Surgical advancements in paediatric cardiovascular surgery have led to improved survival rates for those patients with the most complex CHDs leading to greater numbers of patients who are living well into adulthood. Despite this new era of long-term survival, our current reporting systems continue to focus largely on using short-term postoperative outcomes as the criteria to both rate and rank hospitals. Using such limited criteria to rate and rank hospitals may mislead the intended audiences: patients and families. The goal of this article is to describe the creation of a local benchmarking report which aims to retrospectively review long-term outcomes from our single centre. This report is updated annually and published on our cardiac surgery webpage in an effort to be as transparent as possible for our patient and family communities.

In the 1980s, heart transplantation was the first successful treatment for infants born with hypoplastic left heart syndrome. Infants who have required heart transplantation benefit from immunologic “advantages,” including long-term survival free from cardiac allograft vasculopathy. Currently ∼ 90% of children undergoing a heart transplant are reaching their first-year anniversary and the clinical practices of paediatric heart transplantation have dramatically improved. These successes are largely attributed to research sponsored by the Pediatric Heart Transplant Study Group, the International Society of Heart and Lung Transplantation and, more recently, the Non-profits Enduring Hearts and Additional Ventures. Despite these successes, the field is challenged to increase progress to achieve long-term survival into adulthood. The wait-list mortality, especially among infants, is unacceptably high often leading to palliative measures that can increase post-transplant mortality. Cardiac allograft vasculopathy remains a major cause for progressive graft loss of function and sudden death. The relative tolerance seen in immature recipients has not been translated to modifying older recipients’ post-transplant outcomes. The modifiable cause(s) for the increased risks of transplantation in children of different ethnicities and races require definition. Addressing these challenges faces the reality that for-profit research favours funding adult recipients, with ∼ 10-fold greater numbers, and their more modest longevity goals. Advocacy for funding “incentives” such as the Orphan Drug rules in the United States and upholding principles of equity and inclusion are critical to addressing the challenges of paediatric heart transplant recipients worldwide.

Pulmonary valve replacement and right ventricular outflow tract reconstruction with valved conduits have been the shortcomings of paediatric cardiac surgeons in the treatment of CHD. In recent decades, encouraging achievements have been made in right ventricular outflow tract technology. Since Klinner reported the first right ventricle-to-pulmonary artery connection using unvalved conduits made of autologous pericardium in 1964, various right ventricle-to-pulmonary artery conduits have gradually been used in the treatment of various complex CHD. Compared with other materials, valved homograft conduit (VHC) is more consistent with physiological characteristics, better haemodynamics, easy suture and good haemostasis, anti-calcification, anti-infection, and without the need for lifelong anticoagulation, which makes VHC the best material for reconstruction of right ventricular outflow tract. However, due to the shortage of donor sources, other alternative conduits such as polytetrafuoroethylene valved conduits have been developed, and the results are not inferior to VHC in clinical application. The emerging tissue engineering technology is expected to utilise recipient-derived endothelial cells for implantation onto the decellularized VHC or degradable synthetic materials in order to construct a recipient-specific tissue-engineered valved conduit. This advancement holds great potential as an ideal biological transplant material and valve replacement for CHD. It will completely solve the problems of immune rejection and the growth of the conduit that cannot adapt to the physical growth of children. This review provides a comprehensive review of the clinical indications for right ventricle-to-pulmonary artery conduits application, optimal timing for surgery, current practices in utilising various types of external conduits, and considerations for re-replacement.

The population of people living with a Fontan circulation are highly heterogenous, including both children and adults, who have complex health issues and comorbidities associated with their unique physiology throughout life. Research focused on nutritional considerations and interventions in the Fontan population is extremely limited beyond childhood. This review article discusses the current literature examining nutritional considerations in the setting of Fontan physiology and provides an overview of the available evidence to support nutritional management strategies and future research directions. Protein-losing enteropathy, growth deficits, bone mineral loss, and malabsorption are well-recognised nutritional concerns within this population, but increased adiposity, altered glucose metabolism, and skeletal muscle deficiency are also more recently identified issues. Emergencing evidence suggets that abnormal body composition is associated with poor circulatory function and health outcomes. Many nutrition-related issues, including the impact of congenital heart disease on nutritional status, factors contributing to altered body composition and comorbidities, as well as the role of the microbiome and metabolomics, remain poodly understood.

Congenital heart disease is the most common birth defect in the United States, with many of the affected infants requiring surgical and/or interventional procedures within their first year of life. The parental impacts of a child’s diagnosis, subsequent hospitalization, and transition to home after discharge are numerous and burdensome, and many experience symptoms of traumatic stress along this trajectory. The purpose of this scoping review was to summarize current available literature related to the traumatic stress experienced by parents of children with heart disease to better understand the prevalence, related factors, and consequences. The Joanna Briggs Institute Scoping Review Framework was implemented to identify 31 relevant peer-reviewed articles published between 2000 and early 2024, including 25 quantitative studies, 3 qualitative studies, and 3 systematic reviews or meta-analyses. This scoping review provides an overview of parent traumatic stress for clinicians caring for children with heart disease at every stage of their clinical course.

Hypertrophic cardiomyopathy is the second most common cardiomyopathy affecting children and adolescents and is the main cause of sudden death of young athletes. The natural prognosis of children with severe hypertrophic obstructive cardiomyopathy is not optimistic, and it is not uncommon for children with hypertrophic obstructive cardiomyopathy who do not respond to medication. Surgical treatment is often the only solution. Conventional surgical methods in the past include classic or modified extended Morrow operation, classic or modified Konno operation, and Ross-Konno operation. In recent years, with the development of minimally invasive surgery, various minimally invasive surgical methods have emerged endlessly. Because the incision of minimally invasive cardiac surgery is significantly smaller than that of traditional surgery, it causes less trauma, recovers quickly after surgery, and has the advantage of no difference in surgical effect compared with traditional median sternotomy. Tally endoscopic transmitral myectomy, RTM, minimally right thoracotomy, and other surgical methods have achieved encouraging results in adults and some older children with hypertrophic obstructive cardiomyopathy. The appearance of transapical beating-heart septectomy has brought the treatment of hypertrophic obstructive cardiomyopathy from the era of cardiopulmonary bypass and cardiac arrest to a new era of minimally invasive beating-heart surgery. In the past, there were few articles about the treatment of children with hypertrophic obstructive cardiomyopathy. This article reviewed the new progress and prognosis of surgical treatment of children with hypertrophic obstructive cardiomyopathy at home and abroad.

Anomalous left coronary artery from the pulmonary artery is a rare CHD. It is the most common type of anomalous coronary origin. It may cause myocardial ischaemia or infarction, mitral regurgitation, congestive heart failure, and early death in infancy if left untreated. Surgery is the only treatment for anomalous left coronary artery from the pulmonary artery. In recent years, with advancements in surgical techniques and the widespread utilisation of extracorporeal cardiac assist devices such as extracorporeal membrane oxygenation, the treatment outcomes for anomalous left coronary artery from the pulmonary artery have demonstrated significant improvements. However, the surgical indications and methods of anomalous left coronary artery from the pulmonary artery, especially the surgical methods of anomalous left coronary artery from the pulmonary artery with intramural coronary artery, and whether to treat mitral regurgitation at the same time are still controversial. The long-term complications and prognosis remain discouraging simultaneously, with significant variations in outcomes across different centres. The present review specifically addresses these aforementioned concerns. Based on the literature published at home and abroad, we found that no matter what type of anomalous left coronary artery from the pulmonary artery patients, even asymptomatic patients, regardless of the collateral circulation between the left and right coronary arteries, should immediately undergo surgical treatment to promote the recovery of left ventricular function. Based on different coronary artery anatomical morphology and preoperative cardiac function, the long-term follow-up results of individualised surgical treatment of anomalous left coronary artery from the pulmonary artery children show good prognosis, and most children have significant improvement in cardiac function. Patients with moderate to severe mitral regurgitation should undergo mitral valve operation at the same time as anomalous left coronary artery from the pulmonary artery repair. Mitral valvuloplasty can quickly improve mitral regurgitation and promote the early recovery of cardiac function after operation, and does not increase the risk of operation. Mechanical circulatory support is a safe and effective means of early postoperative transition for children with severe anomalous left coronary artery from the pulmonary artery. Anomalous left coronary artery from the pulmonary artery with intramural coronary artery is a rare anomaly. According to different anatomical types, different surgical methods can be used for anatomical correction, and satisfactory early and mid-term results can be obtained.