Congenital coronary artery stenosis coexisting with aortic coarctation in nonsyndromic patients has not previously been reported. This report describes a nonsyndromic aortic coarctation patient who experienced intraoperative cardiac arrest due to a previously undiagnosed critical left main coronary artery stenosis. The patient was successfully resuscitated, underwent patch coronary ostioplasty, and was discharged home. He remains well for four months following repair.

We report a case of hypoplastic left heart syndrome and with subsequent aortopathy and then found to have hereditary haemorrhagic telangiectasia/juvenile polyposis syndrome due to a germline SMAD4 pathologic variant. The patient’s staged palliation was complicated by the development of neoaortic aneurysms, arteriovenous malformations, and gastrointestinal bleeding thought to be secondary to Fontan circulation, but workup revealed a SMAD4 variant consistent with hereditary haemorrhagic telangiectasia/juvenile polyposis syndrome. This case underscores the importance of genetic modifiers in CHD, especially those with Fontan physiology.

Substantial progress has been made in the standardization of nomenclature for paediatric and congenital cardiac care. In 1936, Maude Abbott published her Atlas of Congenital Cardiac Disease, which was the first formal attempt to classify congenital heart disease. The International Paediatric and Congenital Cardiac Code (IPCCC) is now utilized worldwide and has most recently become the paediatric and congenital cardiac component of the Eleventh Revision of the International Classification of Diseases (ICD-11). The most recent publication of the IPCCC was in 2017. This manuscript provides an updated 2021 version of the IPCCC.

The International Society for Nomenclature of Paediatric and Congenital Heart Disease (ISNPCHD), in collaboration with the World Health Organization (WHO), developed the paediatric and congenital cardiac nomenclature that is now within the eleventh version of the International Classification of Diseases (ICD-11). This unification of IPCCC and ICD-11 is the IPCCC ICD-11 Nomenclature and is the first time that the clinical nomenclature for paediatric and congenital cardiac care and the administrative nomenclature for paediatric and congenital cardiac care are harmonized. The resultant congenital cardiac component of ICD-11 was increased from 29 congenital cardiac codes in ICD-9 and 73 congenital cardiac codes in ICD-10 to 318 codes submitted by ISNPCHD through 2018 for incorporation into ICD-11. After these 318 terms were incorporated into ICD-11 in 2018, the WHO ICD-11 team added an additional 49 terms, some of which are acceptable legacy terms from ICD-10, while others provide greater granularity than the ISNPCHD thought was originally acceptable. Thus, the total number of paediatric and congenital cardiac terms in ICD-11 is 367. In this manuscript, we describe and review the terminology, hierarchy, and definitions of the IPCCC ICD-11 Nomenclature. This article, therefore, presents a global system of nomenclature for paediatric and congenital cardiac care that unifies clinical and administrative nomenclature.

The members of ISNPCHD realize that the nomenclature published in this manuscript will continue to evolve. The version of the IPCCC that was published in 2017 has evolved and changed, and it is now replaced by this 2021 version. In the future, ISNPCHD will again publish updated versions of IPCCC, as IPCCC continues to evolve.

Anomalous aortic origin of the coronary arteries is associated with exercise-induced ischaemia, leading some physicians to restrict exercise in patients with this condition. We sought to determine whether exercise restriction was associated with increasing body mass index over time. From 1998 to 2015, 440 patients ⩽30 years old were enrolled into an inception cohort. Exercise-restriction status was documented in 143 patients. Using linear mixed model repeated-measures regression, factors associated with increasing body mass index z-score over time, including exercise restriction and surgical intervention as time-varying covariates, were investigated. The 143 patients attended 558 clinic visits for which exercise-restriction status was recorded. The mean number of clinic visits per patient was 4, and the median duration of follow-up was 1.7 years (interquartile range (IQR) 0.5–4.4). The median age at first clinic visit was 10.3 years (IQR 7.1–13.9), and 71% (101/143) were males. All patients were alive at their most recent follow-up. At the first clinic visit, 54% (78/143) were exercise restricted, and restriction status changed in 34% (48/143) during follow-up. The median baseline body mass index z-score was 0.2 (IQR 0.3–0.9). In repeated-measures analysis, neither time-related exercise restriction nor its interaction with time was associated with increasing body mass index z-score. Surgical intervention and its interaction with time were associated with decreasing body mass index z-score. Although exercise restriction was not associated with increasing body mass index over time, surgical intervention was associated with decreasing body mass index z-score over time in patients with anomalous aortic origin of the coronary arteries.

A 9-year-old boy who was born with bicuspid aortic stenosis underwent two unsuccessful aortic valvuloplasty interventions, and by 2 years of age he developed restrictive cardiomyopathy caused by left ventricular endocardial fibroelastosis and diastolic dysfunction. The attending cardiologist referred the patient to a high-volume, high-profile congenital cardiac surgical programme 1000 miles away that has a team with considerable experience with left ventricular endocardial fibroelastosis resection and a reputation of achieving good results. Owing to problems with insurance coverage, the parents sought other options for the care of their child in their home state. Dr George Miller is a well-respected local congenital and paediatric cardiac surgeon with considerable experience with the Ross operation as well as with right ventricular endocardial fibroelastosis resection. When talking with Dr Miller, he implied that there is little difference between right ventricular endocardial fibroelastosis and left ventricular endocardial fibroelastosis resection, and stated that he would perform the operation with low mortality based on his overall experience. Dr Miller stated that the local institution could provide an equivalent surgical procedure with comparable outcomes, without the patient and family having to travel out of state. A fundamental dilemma that often arises in clinical surgical practice concerns the conduct of assessing and performing new procedures, especially in rare cases, for which the collective global experience is scant. Although Dr Miller has performed right ventricular endocardial fibroelastosis resection, this procedure differs from left ventricular endocardial fibroelastosis resection, and he cannot be sure that he will indeed be able to perform the procedure better than the high-volume surgeon. This ethical situation is best understood in terms of the principles of respect for patient autonomy, beneficence, non-maleficence, and justice. The tension between the imperatives of beneficence and the obligation to respect the autonomy of the patient by acting only with the patient’s best interest in mind is discussed.

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.

Respect for patient autonomy is an important and indispensable principle in the ethical practice of clinical medicine. Legal tenets recognise the centrality of this principle and the inherent right of patients of sound mind – properly informed – to make their own personal medical decisions. In the course of everyday medical practice, however, challenging cases may result in ethical dilemmas for the patient, the physician, and society. Resolution of these dilemmas requires a thorough understanding of the underlying principles that allow the clinician to make informed decisions and to offer considered therapeutic options to the patient. We argue in this paper that there is also need for a transition of moral competency from understanding principles to attaining virtue in the classic Aristotelian tradition. Achieving moral virtue is based on a lifetime of learning, practising, and watching how others, who have achieved virtue, act and perform their duties. We further claim that learning moral virtue in medical practice is best realised by incorporating the lessons learnt during daily rounds where frank discussions and considered resolutions can occur under the leadership of senior practitioners who have achieved a semblance of moral excellence.

Anomalous aortic origins of the coronary arteries comprise approximately one-third of all coronary artery anomalies and are characterised by coronary arteries with anomalies of aortic origin involving abnormal courses, stenoses, and compression that can lead to myocardial ischaemia and sudden death. Operative techniques to treat these anomalies have not been standardised yet. Moreover, the management of potential complications has not been addressed. Common and rare forms of anomalous aortic origins of the coronary arteries are reviewed and understood standard techniques for an uncomplicated unroofing procedure are illustrated. Also noted are techniques that can be applied to unexpected anatomical findings and unwanted complications that could prove to be life-threatening. Several technical recommendations are offered.

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.

Despite tremendous advances in surgical treatment of tetralogy of Fallot, augmenting the small right ventricular outflow tract remains a challenge. Transannular patch augmentation revolutionised surgical management, but did so at the expense of rendering patients with pulmonary insufficiency and the resulting problems associated therewith. Recent surgical efforts have focused on pulmonary valve preservation at initial correction and pulmonary valve restoration after transannular patching, with favourable results. In this manuscript, we review methods of pulmonary valve preservation and restoration.

Operations for left ventricular outflow tract abnormalities are centred on hemodynamic conditions that relate to subvalvar stenosis, valvar stenosis/regurgitation, aortic annular hypoplasia, and supravalvar aortic stenosis. Operative interventions over the years have evolved because the intervening outcomes proved to be unsatisfactory. The resection for subvalvar aortic stenosis has progressed from a fibrous “membrane” resection to a more extensive fibromuscular resection. Operative solutions for valvar aortic stenosis and regurgitation have resulted in operative interventions that depend on simple commissurotomy, leaflet extensions, prosthetic mechanical valve replacement, biologic valve replacement, including the pulmonary autograft, and operations to treat aortic annular stenosis. Although there are enthusiastic proponents for all of these strategies, the fact remains that none have proven to be curative; patients can expect to undergo further procedures during their lifetimes. The short- and mid-term solutions to these left ventricular outflow tract abnormalities have improved based on operations that have been attended by increasing operative complexity. The purpose of this review is to chronicle the operative steps of the Ross operation, the Konno–Rastan operation, the modified Konno operation, the Ross–Konno operation, and the modified Ross–Konno operation.

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.

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.

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.

In utero diagnosis of complex progressive cardiac disease such as hypoplastic left heart syndrome presents a novel opportunity for antepartum, intrapartum, and neonatal management. The clinical possibilities and potential for differing outcomes challenge the mother–foetus dyad with regard to informed consent. Previous studies reveal that rates of termination of pregnancy for foetuses with hypoplastic left heart syndrome vary widely in the United States and Europe, leading us to surmise that informed consent may be practised differently. The purpose of this paper is to review the ethical considerations and physician responsibilities of informed consent as they relate to prenatal and postnatal patients with hypoplastic left heart syndrome. Special consideration is paid to the informed consent process as practised by the obstetrician, perinatologist, paediatric cardiologist, and paediatric cardiac surgeon as it relates to termination of pregnancy, comfort care, and surgical palliation. We will argue that informed consent as it relates to hypoplastic left heart syndrome is far from standardised and that there exists a state of bioethical equipoise concerning the extent and limits of its application in the current clinical setting.

A complication is an event or occurrence that is associated with a disease or a healthcare intervention, is a departure from the desired course of events, and may cause, or be associated with, suboptimal outcome. A complication does not necessarily represent a breech in the standard of care that constitutes medical negligence or medical malpractice. An operative or procedural complication is any complication, regardless of cause, occurring (1) within 30 days after surgery or intervention in or out of the hospital, or (2) after 30 days during the same hospitalization subsequent to the operation or intervention. Operative and procedural complications include both intraoperative/intraprocedural complications and postoperative/postprocedural complications in this time interval.

The MultiSocietal Database Committee for Pediatric and Congenital Heart Disease has set forth a comprehensive list of complications associated with the treatment of patients with congenital cardiac disease, related to cardiac, pulmonary, renal, haematological, infectious, neurological, gastrointestinal, and endocrinal systems, as well as those related to the management of anaesthesia and perfusion, and the transplantation of thoracic organs. The objective of this manuscript is to examine the definitions of operative morbidity as they relate specifically to the cardiac system. These specific definitions and terms will be used to track morbidity associated with surgical and transcatheter interventions and other forms of therapy in a common language across many separate databases.

The MultiSocietal Database Committee for Pediatric and Congenital Heart Disease has prepared and defined a near-exhaustive list of cardiac complications, including intraoperative complications and cardiopulmonary bypass-related complications. These cardiac complications are presented in the following subgroups:

1) Cardiac (general)

2) Cardiac – Metabolic

3) Cardiac – Residual and Recurrent cardiac lesions

4) Arrhythmia

5) Cardiopulmonary bypass and Mechanical circulatory support, and

6) Operative/Procedural.

Within each subgroup, complications are presented in alphabetical order. Clinicians caring for patients with congenital cardiac disease will be able to use this list for databases, quality improvement initiatives, reporting of complications, and comparing strategies for treatment.