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.

On 15 October, 2020, Dr. Paul M. Weinberg, a true giant in the field of paediatric cardiology, succumbed to a prolonged illness. Dr. Weinberg had a 43-year career and was described as a pillar of The Children’s Hospital of Philadelphia and the spirit of the Division of Cardiology, a cherished and beloved teacher, and an outstanding clinician. His impact on the field and on the careers of his students will be remembered for generations to come.

In 2019, Dr. Weinberg wrote for Jefferson Medical School’s 50th year reunion memory book: “In the true spirit of Hippocrates, I seek to mentor the next generation as I was mentored by the last, without expectation of reward. I am forever indebted to these educators for all the knowledge they imparted to me and for the wisdom that I acquired under their tutelage.” These words are a true reflection of his unassuming dedication to teaching the next generation of paediatric cardiologists. His legacy will continue to live on through these trainees and impact the field for generations to come.

An internationally approved and globally used classification scheme for the diagnosis of CHD has long been sought. The International Paediatric and Congenital Cardiac Code (IPCCC), which was produced and has been maintained by the International Society for Nomenclature of Paediatric and Congenital Heart Disease (the International Nomenclature Society), is used widely, but has spawned many “short list” versions that differ in content depending on the user. Thus, efforts to have a uniform identification of patients with CHD using a single up-to-date and coordinated nomenclature system continue to be thwarted, even if a common nomenclature has been used as a basis for composing various “short lists”. In an attempt to solve this problem, the International Nomenclature Society has linked its efforts with those of the World Health Organization to obtain a globally accepted nomenclature tree for CHD within the 11th iteration of the International Classification of Diseases (ICD-11). The International Nomenclature Society has submitted a hierarchical nomenclature tree for CHD to the World Health Organization that is expected to serve increasingly as the “short list” for all communities interested in coding for congenital cardiology. This article reviews the history of the International Classification of Diseases and of the IPCCC, and outlines the process used in developing the ICD-11 congenital cardiac disease diagnostic list and the definitions for each term on the list. An overview of the content of the congenital heart anomaly section of the Foundation Component of ICD-11, published herein in its entirety, is also included. Future plans for the International Nomenclature Society include linking again with the World Health Organization to tackle procedural nomenclature as it relates to cardiac malformations. By doing so, the Society will continue its role in standardising nomenclature for CHD across the globe, thereby promoting research and better outcomes for fetuses, children, and adults with congenital heart anomalies.

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.

Borderline left ventricle refers to a spectrum of left ventricular underdevelopment, typically associated with other cardiac anomalies. The left ventricle may be mildly hypoplastic, as is sometimes seen accompanying aortic coarctation, or it can be severely hypoplastic, as is seen in hypoplastic left heart syndrome. For patients with a borderline left ventricle that is at either extreme, the treatment decision is relatively straightforward. Those with the most severe form of left ventricle hypoplasia will require single ventricle palliation or cardiac transplantation, whereas those with the mildest form may not need any intervention. It is the management strategy of children that fall within the grey zone of the spectrum, which continues to be controversial and remains variable within and among different institutions. Cardiac diseases with associated left ventricle hypoplasia include critical aortic stenosis, mitral stenosis, coarctation of the aorta, arch hypoplasia, cor triatriatum, unbalanced common atrioventricular canal, Shone’s complex, total anomalous pulmonary venous return, and complex conotruncal abnormalities. In this review, we will discuss the assessment and management of infants with borderline left ventricle with critical aortic stenosis or arch obstruction and associated mitral anomalies.

The long-term outcome of patients with congenitally malformed hearts involving abnormal right ventricular morphology and haemodynamics is variable. In most instances, the patients are at risk for right ventricular failure, in part due to morphological differences between the right and left ventricles and their response to chronic volume and pressure overload. In patients after repair of tetralogy of Fallot, and after balloon valvotomy for valvar pulmonary stenosis, pulmonary regurgitation is the most significant risk factor for right ventricular dysfunction. In patients with a dominant right ventricle after Fontan palliation, and in those with systemic right ventricles in association with surgically or congenitally corrected transposition, the right ventricle is not morphologically capable of dealing with chronic exposure to the high afterload of the systemic circulation. In patients with Ebstein’s malformation of the tricuspid valve, the degree of atrialisation of the right ventricle determines how well the right ventricle will function as the pump for the pulmonary vascular bed.

In the past, coronary arterial anomalies have been difficult to diagnose by non-invasive methods. Identification of coronary arterial origins is now a routine part of the standard paediatric echocardiogram. Anomalous origin of a coronary artery from the pulmonary trunk is an extremely important diagnosis to make. Many echocardiographic features are not directly related to the visualisation of the coronary arterial origin. Left ventricular dilation and abnormal ventricular performance are common, along with mitral regurgitation and evidence of collateralisation of the flow from the coronary artery that has an aortic origin. In some cases, the anomalous coronary artery can be seen to arise directly from the pulmonary trunk. Congenital atresia of the main stem of the left coronary artery has a similar echocardiographic presentation, except that its aortic origin is not determined. Anomalous aortic origin of the coronary artery has important implications, as the first presenting symptom can be sudden death. With meticulous attention to the origins of the coronary arteries, echocardiographic diagnosis can also be achieved. In contrast to the anomalous origin of a coronary artery from the pulmonary trunk, ventricular performance is usually normal. Whenever there is doubt as to the definition of the origin of the coronary arteries and, indeed, when there is serious clinical concern that a coronary artery has an anomalous origin, other testing, such as cine-computed tomography, magnetic resonance imaging, or cardiac catheterisation may be indicated for confirmation or to provide greater anatomic detail.

Professionals working in the arena of health care face a variety of challenges as their careers evolve and develop. In this review, we analyze the role of mentorship, learning curves, and balance in overcoming challenges that all such professionals are likely to encounter. These challenges can exist both in professional and personal life.

As any professional involved in health care matures, complex professional skills must be mastered, and new professional skills must be acquired. These skills are both technical and judgmental. In most circumstances, these skills must be learned. In 2007, despite the continued need for obtaining new knowledge and learning new skills, the professional and public tolerance for a “learning curve” is much less than in previous decades. Mentorship is the key to success in these endeavours. The success of mentorship is two-sided, with responsibilities for both the mentor and the mentee. The benefits of this relationship must be bidirectional. It is the responsibility of both the student and the mentor to assure this bidirectional exchange of benefit. This relationship requires time, patience, dedication, and to some degree selflessness. This mentorship will ultimately be the best tool for mastering complex professional skills and maturing through various learning curves. Professional mentorship also requires that mentors identify and explicitly teach their mentees the relational skills and abilities inherent in learning the management of the triad of self, relationships with others, and professional responsibilities.

Up to two decades ago, a learning curve was tolerated, and even expected, while professionals involved in healthcare developed the techniques that allowed for the treatment of previously untreatable diseases. Outcomes have now improved to the point that this type of learning curve is no longer acceptable to the public. Still, professionals must learn to perform and develop independence and confidence. The responsibility to meet this challenge without a painful learning curve belongs to both the younger professionals, who must progress through the learning curve, and the more mature professionals who must create an appropriate environment for learning.

In addition to mentorship, the detailed tracking of outcomes is an essential tool for mastering any learning curve. It is crucial to utilize a detailed database to track outcomes, to learn, and to protect both yourself and your patients. It is our professional responsibility to engage in self-evaluation, in part employing voluntary sharing of data. For cardiac surgical subspecialties, the databases now existing for The European Association for CardioThoracic Surgery and The Society of Thoracic Surgeons represent the ideal tool for monitoring outcomes. Evolving initiatives in the fields of paediatric cardiology, paediatric critical care, and paediatric cardiac anaesthesia will play similar roles.

A variety of professional and personal challenges must be met by all those working in health care. The acquisition of learned skills, and the use of special tools, will facilitate the process of conquering these challenges. Choosing appropriate role models and mentors can help progression through any learning curve in a controlled and protected fashion. Professional and personal satisfaction are both necessities. Finding the satisfactory balance between work and home life is difficult, but possible with the right tools, organization skills, and support system at work and at home. The concepts of mentorship, learning curves and balance cannot be underappreciated.

How best to analyse and describe the features of the situation commonly known as “visceral heterotaxy” remains controversial. Much of the disagreement devolves on how to deal with the concept of isomerism. In the opinion of some, the concept of bilateral right-sidedness and bilateral left-sidedness, while useful in helping to remember which abnormalities are likely to occur in asplenia or polysplenia, should not be granted the status of a specific “situs”, since there are numerous examples of exceptions to these patterns. On the other hand, those who favour the concept of isomerism point out that, when describing only the heart, and taking the structure of the atrial appendages as the starting point for analysis, basing this on the extent of the pectinate muscles relative to the atrioventricular junctions, then the only possible arrangements for the appendages are the usual one, its mirror-image, and the two situations in which appendages of comparable morphology are found on both sides of the heart, these being the arrangements of right or left isomerism. It is certainly the case that the arrangement of the organs is not always in harmony with the arrangement of the atrial appendages, but those circumstances, in which there is disharmony, can readily be described by paying specific attention to each series of organs. On this basis, in this review, we describe the approach to heterotaxy, and isomerism of the atrial appendages, in terms of the genetic background, the diagnosis, and outcomes after cardiac surgery. Attention is given to the various diagnostic modalities, including fetal and postnatal echocardiography, recent tomographic and magnetic resonance imaging techniques, and the time-honoured approach using angiography.

In contrast to older patients, children and young adults rarely have isolated disease of the systemic atrioventricular valve. Stenosis and/or regurgitation of the systemic atrioventricular valve, however, frequently coexist with complex congenital cardiac disease. In addition, most patients undergoing surgery on the systemic atrioventricular valve have had previous intracardiac repairs.

Surgical intervention for hearts with transposition, defined as concordant atrioventricular and discordant ventriculo-arterial connections, has been one of the landmark achievements in the field of paediatric cardiac surgery. In the early 1950s, pioneer surgeons attempted to palliate patients with transposed arterial trunks with an early form of the arterial switch operation. As a result of initially dismal outcomes secondary to difficulties with coronary arterial transfer, the unprepared nature of the morphologically left ventricle, and primitive methods for cardiopulmonary bypass, the arterial switch was abandoned in favour of several procedures achieving correction at atrial and venous levels, culminating in the Mustard and Senning operations.1,2 These innovative procedures produced the earliest surviving children with transposition. Although the atrial switch procedures achieved widespread acceptance and success during the mid-1960s through the mid-1980s, the search for an operation to return the great arteries to their normal anatomic positions continued. This pursuit was stimulated primarily by the accumulating observations in mid-to-late term follow up studies of: an increasing frequency of important arrhythmic complications, including sinus nodal dysfunction, atrial arrhythmias, and sudden, unexplained death, by the development of late right ventricular dysfunction and significant tricuspid regurgitation in a ventricle potentially unsuited for a lifetime of systemic function by a small but important prevalence of obstruction of the systemic and/or pulmonary venous pathways, and by dissatisfaction with the operative mortality in the subgroup of infants complicated by additional presence of a large ventricular septal defect.3–6 As we have already discussed, a number of novel procedures to achieve anatomic correction had been described as early as 1954, but clinical success was not accomplished until 1975, when Jatene and co-workers7 astounded the world of paediatric cardiology with their initial description.

Objectives: The purpose of our study was to assess the prevalence and progression, during childhood and adolescence, of dilation of the neo-aortic root, and neo-aortic valvar regurgitation, and to identify risk factors for such dilation and regurgitation, after the arterial switch operation. Methods: We included all patients who had undergone an arterial switch operation at The Children's Hospital of Philadelphia, and had been followed for a minimum of 4 years, and had at least 2 postoperative echocardiograms. Neo-aortic valvar regurgitation was quantitatively assessed, and measurements were made of the neo-aortic root at the level of the basal attachment of the leaflets, mid-sinusal level, and the sinutubular junction. Results: We found 82 patients who satisfied the criterions for inclusion, of whom 52 patients had transposition with an intact ventricular septum, and 30 had either an associated ventricular septal defect or double outlet right ventricle. The median follow-up time was 8.8 years (4.1 to 16.4 years). The neo-aortic valve had been replaced in 1 patient. Of the patients, 3 had moderate, 66 had trivial to mild, and 12 had no neo-aortic valvar regurgitation at their most recent follow-up. The regurgitation had progressed by at least 1 grade in 38 of the 82 patients (46.4%). Neo-aortic dilation was noted at the basal attachment of the leaflets, and at mid-sinusal level, which was out of proportion to somatic growth. Conclusions: At mid-term follow-up, significant neo-aortic valve regurgitation is present in 3.7%, and trivial to mild regurgitation in 81.4% of patients. The regurgitation progressed in almost half of the patients over time. We also noted progressive dilation of the neo-aortic root out of proportion to somatic growth.