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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)

Lookup NU author(s): Professor Bob Anderson


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© 2017 Cambridge University Press. 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.

Publication metadata

Author(s): Franklin RCG, Beland MJ, Colan SD, Walters HL, Aiello VD, Anderson RH, Bailliard F, Boris JR, Cohen MS, Gaynor JW, Guleserian KJ, Houyel L, Jacobs ML, Juraszek AL, Krogmann ON, Kurosawa H, Lopez L, Maruszewski BJ, St Louis JD, Seslar SP, Srivastava S, Stellin G, Tchervenkov CI, Weinberg PM, Jacobs JP

Publication type: Conference Proceedings (inc. Abstract)

Publication status: Published

Conference Name: Proceedings of the 2017 7th World Congress of Pediatric Cardiology & Cardiac Surger (WCPCCS 2017)

Year of Conference: 2017

Pages: 1872-1938

Print publication date: 01/12/2017

Acceptance date: 02/04/2016

ISSN: 1467-1107

Publisher: Cambridge University Press


DOI: 10.1017/S1047951117002244

Series Title: Cardiology in the Young