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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)*

  • Rodney C. G. Franklin (a1), Marie J. Béland (a2), Steven D. Colan (a3), Henry L. Walters (a4), Vera D. Aiello (a5), Robert H. Anderson (a6), Frédérique Bailliard (a7), Jeffrey R. Boris (a8), Meryl S. Cohen (a8), J. William Gaynor (a8), Kristine J. Guleserian (a9), Lucile Houyel (a10), Marshall L. Jacobs (a11) (a12), Amy L. Juraszek (a13), Otto N. Krogmann (a14), Hiromi Kurosawa (a15), Leo Lopez (a9), Bohdan J. Maruszewski (a16), James D. St. Louis (a17), Stephen P. Seslar (a18), Shubhika Srivastava (a19), Giovanni Stellin (a20), Christo I. Tchervenkov (a21), Paul M. Weinberg (a8) and Jeffrey P. Jacobs (a11) (a12)...

Abstract

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.

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Copyright

Corresponding author

Correspondence to: Dr R. C. G. Franklin, Paediatric Cardiologist, Royal Brompton & Harefield NHS Trust, London SW3 6NP, United Kingdom. Tel: +44 20 7351 8613; Fax: +44 1895 826589; E-mail: r.franklin@rbht.nhs.uk

Footnotes

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*

Presented at The Fifth International Summit on Nomenclature for Pediatric and Congenital Heart Disease at the 2017 Seventh World Congress of Pediatric Cardiology & Cardiac Surgery (WCPCCS 2017), Barcelona, Spain, July 16–21, 2017. Presented Sunday, July 16, 2017.

Footnotes

References

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