Original Article

Nomenclature for congenital and paediatric cardiac disease: Historical perspectives and The International Pediatric and Congenital Cardiac Code

Rodney C.G. Franklin^a1 c1, Jeffrey Phillip Jacobs^a2, Otto N. Krogmann^a3, Marie J. Béland^a4, Vera D. Aiello^a5, Steven D. Colan^a6, Martin J. Elliott^a7, J. William Gaynor^a8, Hiromi Kurosawa^a9, Bohdan Maruszewski^a10, Giovanni Stellin^a11, Christo I. Tchervenkov^a12, Henry L. Walters III^a13, Paul Weinberg^a14 and Robert H. Anderson^a15

^a1 Paediatric Cardiology Directorate, Royal Brompton & Harefield NHS Trust, Harefield, Middlesex, United Kingdom

^a2 The Congenital Heart Institute of Florida (CHIF), Division of Thoracic and Cardiovascular Surgery, All Children’s Hospital and Children’s Hospital of Tampa, University of South Florida College of Medicine, Cardiac Surgical Associates (CSA), Saint Petersburg and Tampa, Florida, United States of America

^a3 Paediatric Cardiology – CHD, Heart Center Duisburg, Duisburg, Germany

^a4 Division of Pediatric Cardiology, The Montreal Children’s Hospital of the McGill University Health Centre, Montréal, Quebec, Canada

^a5 Heart Institute (InCor), Sao Paulo University School of Medicine, Sao Paulo, Brazil

^a6 Department of Cardiology, Children’s Hospital, Boston, Massachusetts, United States of America

^a7 Cardiac Unit, Great Ormond Street Hospital for Children, London, United Kingdom

^a8 Cardiac Surgery, Children’s Hospital of Philadelphia, Philadelphia, Pennsylvania, United States of America

^a9 Cardiovascular Surgery, Heart Institute of Japan, Tokyo Women’s Medical University, Tokyo, Japan

^a10 The Children’s Memorial Health Institute, Department of Cardiothoracic Surgery, Warsaw, Poland

^a11 Pediatric Cardiac Surgery Unit – University of Padova Medical School, Padova, Italy

^a12 Division of Pediatric Cardiovascular Surgery, The Montreal Children’s Hospital of the McGill University Health Centre, Montréal, Quebec, Canada

^a13 Children’s Hospital of Michigan, Wayne State University School of Medicine, Detroit, Michigan

^a14 Division of Pediatric Cardiology, The Children’s Hospital of Philadelphia, Pennsylvania, United States of America

^a15 Cardiac Unit, Institute of Child Health, Great Ormond Street Hospital for Children, London, United Kingdom

Abstract

Clinicians working in the field of congenital and paediatric cardiology have long felt the need for a common diagnostic and therapeutic nomenclature and coding system with which to classify patients of all ages with congenital and acquired cardiac disease. A cohesive and comprehensive system of nomenclature, suitable for setting a global standard for multicentric analysis of outcomes and stratification of risk, has only recently emerged, namely, The International Paediatric and Congenital Cardiac Code. This review, will give an historical perspective on the development of systems of nomenclature in general, and specifically with respect to the diagnosis and treatment of patients with paediatric and congenital cardiac disease. Finally, current and future efforts to merge such systems into the paperless environment of the electronic health or patient record on a global scale are briefly explored.

On October 6, 2000, The International Nomenclature Committee for Pediatric and Congenital Heart Disease was established. In January, 2005, the International Nomenclature Committee was constituted in Canada as The International Society for Nomenclature of Paediatric and Congenital Heart Disease. This International Society now has three working groups. The Nomenclature Working Group developed The International Paediatric and Congenital Cardiac Code and will continue to maintain, expand, update, and preserve this International Code. It will also provide ready access to the International Code for the global paediatric and congenital cardiology and cardiac surgery communities, related disciplines, the healthcare industry, and governmental agencies, both electronically and in published form. The Definitions Working Group will write definitions for the terms in the International Paediatric and Congenital Cardiac Code, building on the previously published definitions from the Nomenclature Working Group. The Archiving Working Group, also known as The Congenital Heart Archiving Research Team, will link images and videos to the International Paediatric and Congenital Cardiac Code. The images and videos will be acquired from cardiac morphologic specimens and imaging modalities such as echocardiography, angiography, computerized axial tomography and magnetic resonance imaging, as well as intraoperative images and videos.

Efforts are ongoing to expand the usage of The International Paediatric and Congenital Cardiac Code to other areas of global healthcare. Collaborative efforts are underway involving the leadership of The International Nomenclature Committee for Pediatric and Congenital Heart Disease and the representatives of the steering group responsible for the creation of the 11th revision of the International Classification of Diseases, administered by the World Health Organisation. Similar collaborative efforts are underway involving the leadership of The International Nomenclature Committee for Pediatric and Congenital Heart Disease and the International Health Terminology Standards Development Organisation, who are the owners of the Systematized Nomenclature of Medicine or “SNOMED”.

The International Paediatric and Congenital Cardiac Code was created by specialists in the field to name and classify paediatric and congenital cardiac disease and its treatment. It is a comprehensive code that can be freely downloaded from the internet (http://www.IPCCC.net) and is already in use worldwide, particularly for international comparisons of outcomes. The goal of this effort is to create strategies for stratification of risk and to improve healthcare for the individual patient. The collaboration with the World Heath Organization, the International Health Terminology Standards Development Organisation, and the healthcare industry, will lead to further enhancement of the International Code, and to its more universal use.

Keywords

• Databases;
• cardiac;
• nomenclature;
• congenital heart disease

Correspondence:

^c1 Correspondence to: Dr Rodney C.G. Franklin, Paediatric Cardiologist, Harefield Site, Royal Brompton and Harefield NHS Trust, Harefield, Middlesex UB9 6JH, UK. Tel/fax: 01895 828659; E-mail: r.franklin@rbht.nhs.uk