Nomenclature for Pediatric and Congenital Cardiac Care: Unification of Clinical and Administrative Nomenclature - The 2021 International Paediatric and Congenital Cardiac Code (IPCCC) and the Eleventh Revision of the International Classification of Diseases (ICD-11).

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.

Nomenclature for congenital and paediatric cardiac disease: the International Paediatric and Congenital Cardiac Code (IPCCC) and the Eleventh Iteration of the International Classification of Diseases (ICD-11).

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.

The problems that exist when considering the anatomic variability between the channels that permit interventricular shunting.

Although steps are being taken to produce a universally acceptable coding system for categorisation of the congenitally malformed hearts, obstacles remain in the search for consensus. One of the groups of lesions continuing to produce the greatest problems is those that permit interventricular shunting. The difficulties relate partly to the words used to describe the group itself, as those using Germanic languages describe the holes as ventricular septal defects, whereas those using Romance languages consider them to represent interventricular communications. The two terms, however, are not necessarily synonymous. Further disagreements relate to whether the lesions placed within the group should be sub-categorised on the basis of their geographical location within the ventricular mass, as opposed to the anatomic nature of their borders. In reality, attention to both the features is necessary if we are to recognise the full extent of phenotypic variability. In this review, we first review the evolution and theories of analysis naming the channels that permit interventricular shunting. We then demonstrate that embryologic techniques provide evidence that the changing morphology of the developing murine heart parallels the anatomy of the different lesions encountered in the congenitally malformed human heart. We suggest that, with attention paid to the temporal development of the normal murine heart, combined with a strict definition of the plane of separation between the right and left ventricular cavities, it will be feasible to produce a categorisation that is acceptable to all.

Nomenclature for Pediatric and Congenital Cardiac Care: Unification of Clinical and Administrative Nomenclature - The 2021 International Paediatric and Congenital Cardiac Code (IPCCC) and the Eleventh Revision of the International Classification of Diseases (ICD-11).

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.

Introduction to cardiac imaging in infants and children: techniques, potential, and role in the imaging work-up of various cardiac malformations and other pediatric heart conditions.

The increasing prevalence of congenital heart disease (CHD) can be attributed to major improvements in diagnosis and treatment. Although echocardiography is the most commonly used imaging modality for diagnosis and follow-up of subjects with CHD, the evolution of cardiovascular magnetic resonance (MR) imaging and increasingly computed tomography (CT) does offer new ways to visualize the heart and the great vessels. The development of cardiovascular MR techniques allows for a comprehensive assessment of cardiac anatomy and function. This provides information about the long-term sequlae of the underlying complex anatomy, hemodynamic assessment of residual post-operative lesions and complications of surgery. As much of the functional data in CHD patients is usually acquired with invasive X-ray angiography, non-invasive alternatives such as cardiovascular MR (and CT) are desirable. This review evaluates the role of MR imaging in the management of subjects with CHD, particularly detailing recent developments in imaging techniques as they relate to the various CHD diagnoses we commonly encounter in our practice.

Improving Quality of Congenital Heart Disease Research in Canada: Standardizing Nomenclature Across Canada.

In an effort to improve the quality of interinstitutional and nation-wide research into congenital heart disease (CHD) in Canada, the authors propose the national implementation of a single nomenclature list for CHD as a first step towards achieving a common disease classification system in all Canadian institutions that deal with congenital heart malformations. The authors offer a brief overview of the history and state of nomenclature for CHD in Canada and recommend the national use of the CHD diagnostic list that was recently finalized by the International Society for Nomenclature of Paediatric and Congenital Heart Disease. This list was submitted to the World Health Organization for incorporation into the 11th iteration of the International Classification of Diseases and was recently translated into French by members of the International Society for Nomenclature of Paediatric and Congenital Heart Disease. The bilingual list of the 11th iteration of the International Classification of Diseases CHD terms is published online in this issue of the Canadian Journal of Cardiology. The national standardization of the nomenclature pertaining to CHD using the bilingual list of terms published herein will optimize national efforts to establish longitudinal CHD cohorts, capitalizing on Canada's health care infrastructure and solidifying Canadian leadership in CHD research.

Classification of Ventricular Septal Defects for the Eleventh Iteration of the International Classification of Diseases-Striving for Consensus: A Report From the International Society for Nomenclature of Paediatric and Congenital Heart Disease.

The definition and classification of ventricular septal defects have been fraught with controversy. The International Society for Nomenclature of Paediatric and Congenital Heart Disease is a group of international specialists in pediatric cardiology, cardiac surgery, cardiac morphology, and cardiac pathology that has met annually for the past 9 years in an effort to unify by consensus the divergent approaches to describe ventricular septal defects. These efforts have culminated in acceptance of the classification system by the World Health Organization into the 11th Iteration of the International Classification of Diseases. The scheme to categorize a ventricular septal defect uses both its location and the structures along its borders, thereby bridging the two most popular and disparate classification approaches and providing a common language for describing each phenotype. Although the first-order terms are based on the geographic categories of central perimembranous, inlet, trabecular muscular, and outlet defects, inlet and outlet defects are further characterized by descriptors that incorporate the borders of the defect, namely the perimembranous, muscular, and juxta-arterial types. The Society recognizes that it is equally valid to classify these defects by geography or borders, so the emphasis in this system is on the second-order terms that incorporate both geography and borders to describe each phenotype. The unified terminology should help the medical community describe with better precision all types of ventricular septal defects.

Worsening bradycardia following antithymocyte globulin treatment of severe aplastic anemia.

Immunosuppressive regimens, which include antithymocyte globulin (ATG), are widely used for the treatment of severe aplastic anemia (SAA). However, bradycardia has been reported only as a rare side effect of ATG therapy in the manufacturer's product information and, in rare cases, in the adult literature. We present an adolescent with SAA and preexisting bradycardia who underwent immunosuppression therapy with ATG, methylprednisolone, and tacrolimus and developed profound sinus bradycardia with successive doses of ATG.

Tetralogy of Fallot.

Tetralogy of Fallot is a congenital cardiac malformation that consists of an interventricular communication, also known as a ventricular septal defect, obstruction of the right ventricular outflow tract, override of the ventricular septum by the aortic root, and right ventricular hypertrophy. This combination of lesions occurs in 3 of every 10,000 live births, and accounts for 7-10% of all congenital cardiac malformations. Patients nowadays usually present as neonates, with cyanosis of varying intensity based on the degree of obstruction to flow of blood to the lungs. The aetiology is multifactorial, but reported associations include untreated maternal diabetes, phenylketonuria, and intake of retinoic acid. Associated chromosomal anomalies can include trisomies 21, 18, and 13, but recent experience points to the much more frequent association of microdeletions of chromosome 22. The risk of recurrence in families is 3%. Useful diagnostic tests are the chest radiograph, electrocardiogram, and echocardiogram. The echocardiogram establishes the definitive diagnosis, and usually provides sufficient information for planning of treatment, which is surgical. Approximately half of patients are now diagnosed antenatally. Differential diagnosis includes primary pulmonary causes of cyanosis, along with other cyanotic heart lesions, such as critical pulmonary stenosis and transposed arterial trunks. Neonates who present with ductal-dependent flow to the lungs will receive prostaglandins to maintain ductal patency until surgical intervention is performed. Initial intervention may be palliative, such as surgical creation of a systemic-to-pulmonary arterial shunt, but the trend in centres of excellence is increasingly towards neonatal complete repair. Centres that undertake neonatal palliation will perform the complete repair at the age of 4 to 6 months. Follow-up in patients born 30 years ago shows a rate of survival greater than 85%. Chronic issues that now face such adults include pulmonary regurgitation, recurrence of pulmonary stenosis, and ventricular arrhythmias. As the strategies for surgical and medical management have progressed, the morbidity and mortality of those born with tetralogy of Fallot in the current era is expected to be significantly improved.

Complete atrioventricular canal: comparison of modified single-patch technique with two-patch technique.

BACKGROUND: The purpose of this study was to compare the modified single-patch technique to the two-patch technique for infants with complete atrioventricular canal (CAVC) defects. METHODS: Between January 2000 and June 2006, 55 infants underwent CAVC repair. Twenty-six patients had a modified single-patch technique; 29 patients had a two-patch technique. Trisomy 21 was present in 23 of 26 and 26 of 29 patients (p = not significant [ns]). Mean age was 4.4 +/- 1.3 months (single-patch) versus 5.5 +/- 1.9 months (two-patch, p < 0.02). Mean weight was 4.74 +/- 0.92 versus 5.28 +/- 1.67 kilograms (p = ns). RESULTS: There was one death in the modified single-patch group (postoperative day 130, liver failure) and no deaths in the two-patch group. Cross-clamp times and cardiopulmonary bypass times were shorter in the modified single-patch group (97.3 +/- 19.9 vs 123.3 +/- 28.2 minutes, p < 0.0003; 128 +/- 25 vs 157 +/- 37, p < 0.03). Rastelli classification was type A (18 vs 14), B (1 vs 0), and C (7 vs 15). Mean size of the ventricular septal defect as assessed by transesophageal echocardiogram was 9 +/- 2 mm, (single-patch) versus 10 +/- 3 mm (two-patch) (p = ns). Median postoperative length of stay did not differ (10 vs 8 days). There was no difference in the degree of postoperative left or right AV valve insufficiency as assessed by serial echocardiography. One patient (4%) required reoperation for mitral insufficiency in the modified single-patch versus three patients in the two-patch group (10%, p = ns). There were no patients with third degree atrioventricular block or that required reoperation for residual VSD in the modified single-patch group. There was one patient with third-degree AV block that required a pacemaker and one patient who had reoperation for a residual ventricular septal defect in the two-patch group (p = ns). No patient in either group required reoperation for left ventricular outflow tract obstruction. CONCLUSIONS: The modified single-patch technique produced results comparable with the two-patch technique in younger patients with similarly sized ventricular septal defects. Furthermore, the modified single-patch technique was performed with significantly shorter cross-clamp and cardiopulmonary bypass times.

Evolving surgical strategy for sinus venosus atrial septal defect: effect on sinus node function and late venous obstruction.

BACKGROUND: Our surgical strategy for repair of sinus venosus atrial septal defect has evolved chiefly to avoid sinus node dysfunction. We reviewed our experience with the single-patch, two-patch, and Warden repairs. METHODS: We identified 54 patients with repair of sinus venosus atrial septal defect from 1990 to 2006. Mean age was 9.5 +/- 12.6 years; median age was 4.2 years. Partial anomalous pulmonary venous connection was found in 52 patients (96%); drainage was to the right atrium in 8, right atrial-superior vena cava (SVC) junction in 17, and directly to the SVC in 27. Techniques were single-patch repair (24), two-patch repair (25), and Warden repair (5). Autologous pericardium was used in all patients. Echocardiogram and electrocardiogram follow-up were available for 48 patients (89%). RESULTS: There were no early or late deaths and no reoperations. No patient had pulmonary vein stenosis. Five patients had SVC stenosis: 2 mild after two-patch repair; 1 moderate and 1 mild after single-patch repair; and 1 severe stenosis after Warden procedure (p = 0.3). The incidence of rhythm change from sinus to low atrial or junctional rhythm was 35% and was significantly greater among patients with two-patch repair (12 of 22, 55%) compared with single-patch repair (5 of 21, 24%), or the Warden repair (0 of 5, p = 0.02). CONCLUSIONS: Repair of sinus venosus atrial septal defect with autologous pericardium is associated with a low incidence of late SVC or pulmonary vein stenosis with all techniques. Use of the two-patch technique, however, is associated with a significantly greater incidence of sinus node dysfunction. For the patients with partial anomalous pulmonary venous connection entering the SVC, the Warden procedure avoids interfering with the sinus node and should be used preferentially. The single-patch technique remains the procedure of choice for sinus venosus atrial septal defect with partial anomalous pulmonary venous connection entering the right atrium or right atrium-SVC junction.