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)

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.

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

“…(Ann Thorac Surg 2018;106:1578-89) Ó 2018 by The Society of Thoracic Surgeons T he International Society for Nomenclature of Paediatric and Congenital Heart Disease (ISNPCHD) has defined a ventricular septal defect (VSD) as a congenital cardiac malformation in which there is a hole or pathway between the ventricular chambers [1]. It is known as a VSD in English and German and is literally translated as an interventricular communication in Spanish, French, and Portuguese.…”

A new anatomic approach of the ventricular septal defect in the interruption of the aortic arch

“…We classified the VSDs in four main types according to the Eleventh Iteration of the International Classification of Diseases (ICD‐11, Franklin et al. ; Fig. ).…”

The genetic workup for structural congenital heart disease

“…Medical and surgical advances have improved the overall survival to adulthood to over 90%, greatly increasing the prevalence of adults with CHD (Ntiloudi et al, 2016). CHD encompasses a wide spectrum of heterogenous malformations (Franklin et al, 2017), and Figure 1 shows the prevalence in graph form of the most common types of this spectrum (Liu et al, 2019). deletion size and presence of CHD (Rozas, Benavides, León, & Repetto, 2019).…”