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)

Abstract

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.

Accuracy and impact of prenatal diagnosis of common arterial trunk

“…Diagnosis was confirmed postnatally by either fetopsy or postnatal echocardiography. CAT patients were classified according to the dominance of either the systemic or the pulmonary components of the CAT, as described below 24 ; the corresponding revised Van Praagh classification proposed by the Society of Thoracic Surgeons – Congenital Heart Surgery Database Committee 20–23,25,26 is mentioned in parentheses:CAT with aortic dominance and both pulmonary arteries arising from the trunk (Type A1–A2 of the modified Van Praagh classification); CAT with aortic dominance and one pulmonary artery absent from the trunk (Type A3 of the modified Van Praagh classification); one pulmonary artery originates from the CAT, the other is supplied by the arterial duct or collateral arteries; CAT with pulmonary dominance, and interruption of the aortic arch (usually Type B of Celoria and Patton 26 between the left carotid artery and the left subclavian artery) or coarctation of the aorta (Type A4 of the modified Van Praagh classification). …”

Cardiac surgery and congenital heart disease: reflections on a modern revolution

“…Advances in imaging were underpinned by the development of the sequential segmental approach to cardiac morphology, resulting from collaboration between Robert Anderson, Fergus Macartney, Elliot Shinebourne and Michael Tynan in London 36. This made complex congenital heart disease easier to understand and communicate and led ultimately to the development of an international, unified nomenclature, vital for successful registries 37. Rodney Franklin at the Royal Brompton Hospital provided important leadership in this painstaking project.…”

Divided left atrium with totally anomalous drainage of normally connected pulmonary veins

“…More importantly, the authors have identified a phenotypic entity that, again at least to our eyes, lacks either a current definition or code within the newly produced 11 th Revision of the International Classification of Diseases (ICD-11). [2][3][4] A potential code does exist in the current version of The International Paediatric and Congenital Cardiac Code (IPCCC) [https://ipccc.net/], but that code is not entirely accurate.…”

“…When described in our suggested fashion, the arrangement can be listed as a "daughter" code of divided left atrium, or "cor triatriatum sinister," in the newly produced 11 th Revision of the International Classification of Diseases (ICD-11). [2][3][4] (In the typical heart with divided left atrium, an obliquely orientated fibromuscular partition divides the morphologically left atrium into a compartment connected to the pulmonary veins, and a second component in communication with the atrial appendage and the mitral valvar vestibule. The two components of the divided left atrium are therefore named the pulmonary venous component and the vestibular component.)…”