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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.

Significant advances have been made in the care of children with cardiac disease. Most advances have been guided by anatomical and functional understanding of the changes from normal imposed by the cardiac malformations. As survival has increased, it has become rare to obtain untreated and unaltered hearts for morphologic examination. This limited availability of new specimens, coupled with the inevitable deterioration of currently available specimens, means that alternative methods of "preservation" become increasingly important. In this review, we propose and outline a format for the development of a Global Virtual Museum of Congenital Cardiac Pathology , based on established principles of anatomical analysis, classification based on an internationally accepted nomenclature (The International Pediatric and Congenital Cardiac Code or IPCCC), and the use of digital methods of storage of images and videos. In this fashion, we hope to preserve, in a virtual sense, the currently available anatomical specimens.

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.

Tremendous progress has been made in the field of pediatric heart disease over the past 30 years. Although survival after heart surgery in children has improved dramatically, complications still occur, and optimization of outcomes for all patients remains a challenge. To improve outcomes, collaborative efforts are required and ultimately depend on the possibility of using a common language when discussing pediatric and congenital heart disease. Such a universal language has been developed and named the International Pediatric and Congenital Cardiac Code (IPCCC). To make the IPCCC more universally understood, efforts are under way to link the IPCCC to pictures and videos. The Archiving Working Group is an organization composed of leaders within the international pediatric cardiac medical community and part of the International Society for Nomenclature of Paediatric and Congenital Heart Disease ( www.ipccc.net ). Its purpose is to illustrate, with representative images of all types and formats, the pertinent aspects of cardiac diseases that affect neonates, infants, children, and adults with congenital heart disease, using the codes and definitions associated with the IPCCC as the organizational backbone. The Archiving Working Group certifies and links images and videos to the appropriate term and definition in the IPCCC. These images and videos are then displayed in an electronic format on the Internet. The purpose of this publication is to report the recent progress made by the Archiving Working Group in establishing an Internet-based, image encyclopedia that is based on the standards of the IPCCC.

BackgroundFlorida is the fourth largest state in the United States of America. In 2004, 218,045 live babies were born in Florida, accounting for approximately 1744 new cases of congenital heart disease. We review the initial experience of The Society of Thoracic Surgeons Congenital Heart Surgery Database with a regional outcomes report, namely the Society of Thoracic Surgeons Florida Regional Report.MethodsEight centres in Florida provide services for congenital cardiac surgery. The Children’s Medical Services of Florida provide a framework for quality improvement collaboration between centres. All congenital cardiac surgical centres in Florida have voluntarily agreed to submit data to the Society of Thoracic Surgeons Database. The Society of Thoracic Surgeons and Duke Clinical Research Institute prepared a Florida Regional Report to allow detailed regional analysis of outcomes for congenital cardiac surgery.ResultsThe report of 2007 from the Society of Thoracic Surgeons Congenital Heart Surgery Database includes details of 61,014 operations performed during the 4 year data harvest window, which extended from 2003 through 2006. Of these operations, 6,385 (10.5%) were performed in Florida. Discharge mortality in the data from Florida overall, and from each Florida site, with 95% confidence intervals, is not different from cumulative data from the entire Society of Thoracic Surgeons Database, both for all patients and for patients stratified by complexity.ConclusionsA regional consortium of congenital heart surgery centres in Florida under the framework of the Children’s Medical Services has allowed for inter-institutional collaboration with the goal of quality improvement. This experience demonstrates, first, that the database maintained by the Society of Thoracic Surgeons can provide the framework for regional analysis of outcomes, and second, that voluntary regional collaborative efforts permit the pooling of data for such analysis.

Persistent patency of the arterial duct represents one of the most common lesions in the field of congenital cardiac disease. The strategies for management continue to evolve. In this review, we focus on management beyond the neonatal period. We review the temporal evolution of strategies for management, illustrate the currently available the techniques for permanent closure of the patent arterial duct, review the expected outcomes after closure, discuss the current controversy over the appropriate treatment of the so-called “silent” duct, and provide recommendations for the current state of management of patients with persistent patency of the arterial duct outside of the neonatal period.At the Congenital Heart Institute of Florida, we now recommend closure of all patent arterial ducts, regardless of their size. Before selecting and performing the type of procedure, we explain the natural history of the persistently patent arterial duct to the parents or legal guardian of the child. Particular emphasis is placed on the risks of endocarditis, including the recognition that many cases of endocarditis may not be preventable.The devastating effects of endocarditis, coupled with the perception of more anecdotal reports of endocarditis with the silent duct, as well as the low risk of interventions, has led us to recommend closure of the patent arterial duct in these situations. We now recommend intervention, after informed consent, for all patients with a patent arterial duct regardless of size, including those in which the patent duct is “silent”. We recognize, however, that this remains a controversial topic, especially given the new recommendations for endocarditis prophylaxis from American Heart Association.Since 2003, our strategy for closure of the patent arterial duct has changed subsequent to the availability of the Amplatzer occluder. This new device has allowed significantly larger patent arterial ducts to be closed with interventional catheterization procedures that in the past would have been closed at surgery. During the interval between 2002 and 2006 inclusive, the overall surgical volume at our Institute has been stable. Over this period, the number of patients undergoing surgical ligation of the patent arterial duct has decreased, with this decline in volume most notable for the subgroup of patients weighing more than five kilograms. This decrease has been especially notable in thoracoscopic procedures and is attributable to the increased ability to close larger ducts using the Amplatzer occluder.For infants with symptomatic pulmonary overcirculation weighing less than 5 kilograms, our preference is for the surgical approach. For patients who have ductal calcification, significant pleural scarring, or “window-like” arterial ducts, video-assisted ligation is not an option and open surgical techniques are used. When video-assisted ligation is possible, the approach is based on family and surgeon preference. When open thoracotomy is selected, we usually use a muscle-sparing left posterolateral thoracotomy.For patients weighing more than 5 kilograms, we currently recommend percutaneous closure for all patent arterial ducts as the first intervention, reserving surgical treatment for those cases that are not amenable to the percutaneous approach. For symptomatic infants weighing greater than 5 kilogram with large ducts, we prefer to use the Amplatzer occluder. In rare instances, the size of the required ductal occluder is so large that either encroachment into the aorta or pulmonary arteries is noted, and the device is removed. The child is then referred for surgical closure. We can now often predict via echocardiography that a duct is too large for transcatheter closure, even with the Amplatzer occluder, and refer these patients directly to surgery.For patients with an asymptomatic patent arterial duct, we prefer to wait until the weight is from 10 to 12 kilograms, or they are closer to 2 years of age. If the patent arterial duct is greater than 2.0 to 2.5 millimetres in diameter, our preference is to use the Amplatzer occluder. For smaller ducts, we typically use stainless steel coils. Using this strategy, we close all patent arterial ducts, regardless of their size.

After repair of tetralogy of Fallot, many patients present in need of reoperative surgical reconstruction of the right ventricular outflow tract. The predominant physiologic lesion is pulmonary insufficiency, but there may also be varying degrees of obstruction of the right ventricular outflow tract. In the past, it has been felt that patients tolerate pulmonary insufficiency reasonably well. In some patients, however, the long-term effects of pulmonary insufficiency and subsequent right ventricular dilation and dysfunction are associated with poor exercise tolerance and increased incidence of arrhythmias and sudden death.1,2 Numerous studies support replacement of the pulmonary valve as treatment for pulmonary insufficiency in order to improve performance, optimize hemodynamics, and better control arrhythmias.3–10 The indications for reconstruction of the right ventricular outflow tract in this setting, nonetheless, as well as the operative strategy, continue to evolve. There are multiple surgical options for replacement of the pulmonary valve for these patients, including aortic and pulmonary homografts, stented and stentless porcine valves, porcine valved conduits, bovine jugular venous conduits, and even mechanical valves and mechanical valved conduits.11–32 It was a less than ideal experience with these currently available options that stimulated our interest into employing alternative materials and techniques. Favorable experimental and clinical experience with valves made of a polytetrafluoroethylene monoleaflet33–36 encouraged us to consider a new method of reconstruction with this material, using a bifoliate polytetrafluoroethylene valve. In this work, we review our indications for replacement of the pulmonary valve after repair of tetralogy of Fallot, the surgical options available, and our experience reconstructing the right ventricular outflow tract with a new surgically created bifoliate polytetrafluoroethylene valve.

Background In an effort to analyze our experience and develop treatment guidelines, we reviewed all our patients with patent ductus arteriosus (PDA) treated with video-assisted thoracoscopic surgery (VATS) or interventional cardiology coil occlusion. Methods One hundred patients underwent 102 cardiac catheterizations. Forty-five children underwent VATS. The entire cohort of patients is 141 because 4 patients underwent both catheterization and VATS. Results Successful PDA coil occlusion occurred in 91 patients (91 of 100; 91%); 8 had unsuccessful attempts at coil occlusion and 1 was referred for surgical ligation after catheterization without any attempt at coil placement. Thirty-nine children had successful VATS PDA closure. Six children required conversion to thoracotomy because of inadequate exposure during VATS. Hospital stay for children more than 45 days of age was as follows: VATS median stay, 1 day, mean, 1.4 days; thoracotomy median stay, 4 days, mean, 4.6 days. One patient treated with PDA coil occlusion developed a recurrent PDA and required reembolization. Three children underwent initial catheterization without successful coil placement with subsequent successful VATS. All VATS patients left the operating theater with echocardiography documenting no residual PDA. Two children who underwent successful VATS with no residual PDA at hospital discharge were found on outpatient follow-up to have developed tiny recurrent PDAs and both were successfully coil occluded; 1 of these 2 children is 1 of the 3 children initially evaluated by catheterization and then referred for VATS. Conclusions Video-assisted thoracoscopic surgery and coil occlusion represent complementary techniques for PDA treatment. A rationale for selection of the appropriate treatment modality can be based upon the size and age of the patient and the size and morphology of the PDA.

Survival for children with cardiac disease has dramatically increased in the past four decades with the advent of improvements in diagnosis and treatment. In order to further decrease morbidity and mortality, optimization of outcomes must be vigorously pursued, and this optimization requires a common language when discussing and comparing results of the available diagnostic and therapeutic options. This common language exists and is named The International Pediatric and Congenital Cardiac Code (IPCCC). In order to make the IPCCC more universally understood, a ‘virtual visual encyclopedia’ has been created that links and illustrates the terms and definitions of the IPCCC with images of all types. The Archiving Working Group (AWG) of the International Society for Nomenclature of Paediatric and Congenital Heart Disease (ISNPCHD) is an organization composed of members of the international pediatric cardiac medical and surgical community that collaborate to illustrate, with representative images of all types and formats, the pertinent aspects of cardiac diseases that affect all pediatric patients, using the codes and definitions of the IPCCC as the organizational structure. This chapter describes the process of linking illustrations and nomenclature in the effort to better understand congenital and acquired cardiac disease and improve outcomes.

It is timely, in the 125th anniversary of the initial description by Fallot of the hearts most frequently seen in patients presenting with “la maladie bleu”, that we revisit his descriptions, and discuss his findings in the light of ongoing controversies. Fallot described three hearts in his initial publication, and pointed to the same tetralogy of morphological features that we recognise today, namely, an interventricular communication, biventricular connection of the aorta, subpulmonary stenosis, and right ventricular hypertrophy. In one of the hearts, he noted that the aorta arose exclusively from the right ventricle. In other words, one of his initial cases exhibited double-outlet right ventricle. When we now compare findings in hearts with the features of the tetralogy, we can observe significant variations in the nature of the borders of the plane of deficient ventricular septation when viewed from the aspect of the right ventricle. We also find that this plane, usually described as the ventricular septal defect, is not the same as the geometric plane separating the cavities of the right and left ventricles. This means that the latter plane, the interventricular communication, is not necessarily the same as the ventricular septal defect. We are now able to provide further insights into these features by examining hearts prepared from developing mice. Additional molecular investigations will be required, however, to uncover the mechanisms responsible for producing the morphological changes underscoring tetralogy of Fallot.

Objective: A variety of stents are available to aid in the management of complex tracheal, carinal and bronchial stenoses. We reviewed our multi-institutional experience with airway stents in children. Methods: Thirty-three children (age, 13 days-18 years) from four institutions have had a total of 40 stents placed to aid in the management of complex airway stenoses. Three stent types were utilized: 29 silastic stents, five expandable metal stents and six customized carinal stents (four patients had two stents and one patient had four stents). Thirty children had tracheal stents, six children had bronchial stents, and two infants had carinal stents (three children had stenting of more than one area and two had stenting of all three locations). Twenty-eight patients (age, 5 months-18 years; mean, 8.06 years; SEM, 1.13 years) had stents placed after a variety of airway reconstructive procedures. Four underwent stenting in a non-operative setting and one as preoperative stabilization. Results: Twenty-seven patients survived. One patient died early due to bleeding. Five patients died late: two due to bleeding, one from mediastinitis, and two patients with functional airways died late from unrelated problems. Complications are related to scent type and location. Carinal stents can migrate; several techniques are available to help manage this problem. Wire stents are essentially non-removable requiring periodic dilation. Silastic stents stimulate granulation tissue formation requiring periodic bronchoscopic removal. Conclusion: Tracheal stenting can aid in the management of pediatric airway problems. Complications are common, but can be managed with appropriate intervention. 2000 Elsevier Science B.V. All rights reserved.

This article combines material from three complementary overviews presented in the Symposium on Pulmonary Venous Anomalies during the Joint Meeting of the World Society for Pediatric and Congenital Heart Surgery and Sociedad Latina de Cardiologia y Cirugia Cardiovascular Pediátrica in Lima, Peru. We discuss the embryologic basis for nomenclature, the hierarchical diagnostic categories, and the important anatomic and morphologic characteristics of anomalous pulmonary venous connections. The anatomic descriptions help to guide an understandable and sensible approach to the diagnosis and surgical management of these various disorders.

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.

It was in 1971, Fontan and Baudet1published their experience using a surgical procedure to restore a physiologic circulation in three patients with tricuspid atresia. Of the three patients, two survived, thus ushering a new era in the treatment of children with complex forms of congenital cardiac disease. This operation itself, however, was the culmination of a series of experimental and clinical observations that underlined the clinical possibility totally to bypass the right side of the heart.

Background As the population of children with repaired congenital heart disease ages, an increasing number of patients will benefit from pulmonary valve insertion. We report our initial experience in 41 consecutive patients treated with placement of a surgically created polytetrafluoroethylene bicuspid pulmonary valve. Methods A bicuspid pulmonary valve with orifice size greater than 24 mm is created with polytetrafluoroethylene and sutured into the right ventricular outflow tract. To obviate the need for reoperation in growing children, this technique is limited to older children and adults. Polytetrafluoroethylene bicuspid pulmonary valves were placed in 41 patients (age: range, 5.0 to 64.7 years, median=15.7 years; weight: range, 14.2 to 99.0 kilograms, median, 52.0 kg). All patients had pulmonary insufficiency, pulmonary stenosis, or both, after previous intervention for tetralogy of Fallot (27), pulmonary stenosis (11), pulmonary atresia with intact ventricular septum (2), or double outlet right ventricle (1). Results All patients left the operating theater with transesophageal echocardiography documenting no pulmonic stenosis and zero to trace pulmonic insufficiency. Median hospital length of stay was 5 days (range, 3 to 15 days; mean, 5.8 days). Follow-up including echocardiography ranged from 0.2 to 3.1 year (median follow-up, 1.5 years) and revealed significant improvement in New York Heart Association Classification, pulmonary insufficiency, and right ventricular end diastolic dimension. Conclusions Polytetrafluoroethylene bicuspid pulmonary valve reconstruction of the right ventricular outflow tract is a safe, effective, and durable technique for the short term. Appropriate oversizing minimizes outflow tract obstruction while maximizing competence. Long-term follow-up is necessary to determine the true value of this technique.

Sir .—We read with interest the article by Thoele and colleagues 1 relating their experience in the prereferral diagnosis of coarctation of the aorta (CoA). We agree with them that in the asymptomatic child, the most important reason for a lack of diagnosis is an incomplete physical examination. Several recommendations were made. Palpation of the peripheral pulses and measurement of blood pressures were the most important of these suggestions. However, in our opinion, misleading emphasis was placed on palpation and blood pressure measurement in the right arm, almost to the exclusion of palpation and measurement on both upper extremities. Although Thoele and colleagues are correct in emphasizing that a left subclavian artery arising near or distal to the site of coarctation is not uncommon, an aberrant right subclavian artery can and does occur in a similar fashion. We recently encountered a child first referred at 5½ years of age (the

Pulsus alternans is characterized by a regular rhythm with alternation of strong and weak contractions. It is a phenomenon generally ascribed to severe myocardial dysfunction and occurs most commonly in patients with heart failure secondary to increased resistance to left ventricular ejection. It has also been described in coronary artery disease and in idiopathic dilated cardiomyopathy. Although pulsus alternans has more often been described in advanced myocardial disease, it has also been described in acute, transient ischemia.1 Right ventricular (RV) alternans has been described previously, but usually in patients with congestive heart failure or severe RV dysfunction, or both.2,3 The present report describes RV pulsus alternans that occurred acutely during balloon pulmonary valvotomy.