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.

Online learning has become an increasingly expected and popular component for education of the modern-day adult learner, including the medical provider. In light of the recent coronavirus pandemic, there has never been more urgency to establish opportunities for supplemental online learning. Heart University aims to be “the go-to online resource” for e-learning in CHD and paediatric-acquired heart disease. It is a carefully curated open access library of paedagogical material for all providers of care to children and adults with CHD or children with acquired heart disease, whether a trainee or a practising provider. In this manuscript, we review the aims, development, current offerings and standing, and future goals of Heart University.

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.

Interventional cardiology for paediatric and congenital cardiac disease is a relatively young and rapidly evolving field. As the profession begins to establish multi-institutional databases, a universal system of nomenclature is necessary for the field of interventional cardiology for paediatric and congenital cardiac disease. The purpose of this paper is to present the results of the efforts of The International Society for Nomenclature of Paediatric and Congenital Heart Disease to establish a system of nomenclature for cardiovascular catheterisation for congenital and paediatric cardiac disease, focusing both on procedural nomenclature and on the nomenclature of complications associated with interventional cardiology. This system of nomenclature for cardiovascular catheterisation for congenital and paediatric cardiac disease is a component of The International Paediatric and Congenital Cardiac Code. This manuscript is the first part of a two-part series. Part 1 will cover the procedural nomenclature associated with interventional cardiology as treatment for paediatric and congenital cardiac disease. This procedural nomenclature of The International Paediatric and Congenital Cardiac Code will be used in the IMPACT Registry™ (IMproving Pediatric and Adult Congenital Treatment) of the National Cardiovascular Data Registry® of The American College of Cardiology. Part 2 will cover the nomenclature of complications associated with interventional cardiology as treatment for paediatric and congenital cardiac disease.

Interventional cardiology for paediatric and congenital cardiac disease is a relatively young and rapidly evolving field. As the profession begins to establish multi-institutional databases, a universal system of nomenclature is necessary for the field of interventional cardiology for paediatric and congenital cardiac disease. The purpose of this paper is to present the results of the efforts of The International Society for Nomenclature of Paediatric and Congenital Heart Disease to establish a system of nomenclature for cardiovascular catheterisation for congenital and paediatric cardiac disease, focusing both on procedural nomenclature and the nomenclature of complications associated with interventional cardiology. This system of nomenclature for cardiovascular catheterisation for congenital and paediatric cardiac disease is a component of The International Paediatric and Congenital Cardiac Code. This manuscript is the second part of the two-part series. Part 1 covered the procedural nomenclature associated with interventional cardiology as treatment for paediatric and congenital cardiac disease. Part 2 will cover the nomenclature of complications associated with interventional cardiology as treatment for paediatric and congenital cardiac disease.

On 6 October, 2000, A Meeting of representatives from the Association for European Paediatric Cardiology, the Society of Thoracic Surgeons, and the European Association for Cardiothoracic Surgery, took place in Frankfurt, Germany to discuss the publications earlier that year of two separate systems of nomenclature for paediatric and congenital heart disease: the European Paediatric Cardiac Code and the International Congenital Heart Surgery Nomenclature and Database Project. It was agreed at this meeting that the Short Lists of both systems should be mapped to each other in a first attempt to gravitate toward a single system for describing cardiac defects and procedures related to the heart. The need for this mapping, the historical background of the two parallel nomenclature systems and the later ratification of the mapping process by the first International Summit on Nomenclature for Congenital Heart Disease on 27 May, 2001, in Toronto, Canada, are discussed in the current issue of Cardiology in the Young.

Congenital heart defects are the most common cause of death in infants and young children in the developed world. As the mortality in this population has declined to less than 5%, more attention is being focused now on reducing post-procedural morbidities that may seriously impact the patient and their families. Because of multiple reasons, paediatric cardiac surgery and anaesthesia is a perfect model for studying human errors and their impact on patient safety. Congenital cardiac disease is a common lesion causing much morbidity, pain, and loss of life. Over 44,000 surgical procedures are performed yearly to repair congenital cardiac problems in the United States alone. The reduction or elimination of iatrogenic adverse outcomes, given the current mortality rates of 4.2%–4.5%, might lead to as many as 500 children achieving better outcomes or shorter hospitalizations.

Efforts to quantify the frequency of complications related to anaesthesia in patients undergoing congenital cardiac surgery have been difficult to date because of the low occurrence of this surgery compared to other surgeries on children and the relatively rare incidence of complications related to anaesthesia in this population. Anaesthesiologists play a crucial role in the reduction, recognition, and timely treatment of medical errors that impact this morbidity. Paediatric cardiac surgery encompasses many complex procedures that are highly dependent upon a sophisticated organizational structure, effective communication, coordinated efforts of multiple individuals working as a team, and high levels of cognitive and technical performance. Human factor error analysis in this patient population has shown how frequently both minor and major errors occur. The goal of this paper is to outline the frequency and sources of these errors and to suggest treatment strategies which may minimize their occurrence.

A detailed hierarchal nomenclature of arrhythmias is offered with definition of its applications to diagnosis and complications. The conceptual and organizational approach to discussion of arrhythmias employs the following sequence: location – mechanism – aetiology – duration. The classification of arrhythmias is heuristically divided into an anatomical hierarchy: atrial, junctional, ventricular, or atrioventricular. Mechanisms are most simplistically classified as either reentrant, such as macro-reentrant atrial tachycardia, previously described as atrial flutter, or focal, such as automatic or micro-reentrant tachycardia, for example, junctional ectopic tachycardia. The aetiology of arrhythmias can be either iatrogenic, such as postsurgical, or non-iatrogenic, such as genetic or congenital, and in many cases is multi-factorial. Assigning an aetiology to an arrhythmia is distinct from understanding the mechanism of the arrhythmia, yet assignment of a possible aetiology of an arrhythmia may have important therapeutic implications in certain clinical settings. For example, postoperative atrial arrhythmias in patients after cardiac transplantation may be harbingers of rejection or consequent to remediable imbalances of electrolytes. The duration, frequency of, and time to occurrence of arrhythmia are temporal measures that further refine arrhythmia definition, and may offer insight into ascription of aetiology. Finally, arrhythmias do not occur in a void, but interact with other organ systems. Arrhythmias not only can result from perturbations of other organ systems, such as renal failure, but can produce dysfunction in other organ systems due to haemodynamic compromise or embolic phenomena.

When designed in 2000, the Aristotle Complexity Score was entirely based on subjective probability. This approach, based on the opinion of experts, was considered a good solution due to the limited amount of data available. In 2008, the next generation of the complexity score will be based on observed data available from over 100,000 congenital cardiac operations currently gathered in the congenital cardiac surgery databases of the Society of Thoracic Surgeons and the European Association for Cardio-Thoracic Surgery.

A mortality score is created based on 70,000 surgeries harvested in the congenital databases of The Society of Thoracic Surgeons and The European Association for Cardio-Thoracic Surgery. It is derived from 118 congenital cardiovascular operations, representing 91% of the operations and including 97% of the patients. This Mortality Index of the new Aristotle Complexity Score could further be stratified into 5 levels with minimal within-group variation and maximal between-group variation, and may contribute to the planned unification of the Aristotle Complexity Score with the Risk Adjustment for Congenital Heart Surgery system.

Similarly, a score quantifying morbidity risk is created. Due to the progress of congenital cardiac surgery, the mortality is today reduced to an average of 4%. No instrument currently exists to measure the quality of care delivered to the survivors representing 96% of the patients. An objective assessment of morbidity was needed. The Morbidity Index, based on 50,000 operations gathered in the congenital databases of The Society of Thoracic Surgeons and The European Association for Cardio-Thoracic Surgery, is derived from 117 congenital cardiovascular operations representing 90% of the operations and including 95% of the patients. This morbidity indicator is calculated on an algorithm based on length of stay in the hospital and time on the ventilator.

The mortality and morbidity indicators will be part of the next generation of the complexity score, which will be named the Aristotle Average Complexity Score. It will be based on the sum of mortality, morbidity, and subjective technical difficulty. The introduction of objective data in assessment of mortality and morbidity in congenital cardiac surgery is a significant step forward, which should allow a better evaluation of the complexity of the operations performed by a given centre or surgeon.

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.

A complication is an event or occurrence associated with a disease or a healthcare intervention, which constitutes 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 an 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 Multi-Societal 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 patients who have received transplantation of the heart, heart and lung(s) or lung(s). These specific definitions and terms will be used to track morbidity associated with transplantation in a common language across many separate databases.

The complications of transplantation are known risks of congenital cardiac surgery. The purpose of this effort is to propose consensus definitions for post-transplant complications following cardiac surgery so that collection of such data can be standardized. Clinicians caring for patients who have had transplantation of thoracic organs will be able to use this list for databases, quality improvement initiatives, reporting of complications, and comparing treatment strategies.

The International Consortium for Evidence-Based Perfusion (www.bestpracticeperfusion.org) is a collaborative partnership of societies of perfusionists, professional medical societies, and interested clinicians, whose aim is to promote the continuous improvement of the delivery of care and outcomes for patients undergoing extracorporeal circulation. Despite the many advances made throughout the history of cardiopulmonary bypass, significant variation in practice and potential for complication remains. To help address this issue, the International Consortium for Evidence-Based Perfusion has joined the Multi-Societal Database Committee for Pediatric and Congenital Heart Disease to develop a list of complications in congenital cardiac surgery related to extracorporeal circulation conducted via cardiopulmonary bypass, extracorporeal membrane oxygenation, or mechanical circulatory support devices, which include ventricular assist devices and intra-aortic balloon pumps. Understanding and defining the complications that may occur related to extracorporeal circulation in congenital patients is requisite for assessing and subsequently improving the care provided to the patients we serve. The aim of this manuscript is to identify and define the myriad of complications directly related to the extracorporeal circulation of congenital patients.

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 a collection of loosely related topics that include the following groups of complications: 1) Complications of the Integument, 2) Complications of the Vascular System, 3) Complications of the Vascular-Line(s), 4) Complications of Wounds. 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.

As surgical survival in children with congenital cardiac disease has improved in recent years, focus has necessarily shifted to reducing the morbidity of congenital cardiac malformations and their treatment. A comprehensive list of complications is presented. This list is a component of a systems-based compendium of complications that will standardize terminology and thereby allow the study and quantification of morbidity in patients with congenital cardiac malformations. Clinicians caring for patients with congenital cardiac disease will be able to use this list for databases, initiatives to improve quality, reporting of complications, and comparing strategies of treatment.