Abstract
BackgroundClassification of the overall spectrum of congenital heart defects (CHD) has always been challenging, in part because of the diversity of the cardiac phenotypes, but also because of the oft-complex associations. The purpose of our study was to establish a comprehensive and easy-to-use classification of CHD for clinical and epidemiological studies based on the long list of the International Paediatric and Congenital Cardiac Code (IPCCC).MethodsWe coded each individual malformation using six-digit codes from the long list of IPCCC. We then regrouped all lesions into 10 categories and 23 subcategories according to a multi-dimensional approach encompassing anatomic, diagnostic and therapeutic criteria. This anatomic and clinical classification of congenital heart disease (ACC-CHD) was then applied to data acquired from a population-based cohort of patients with CHD in France, made up of 2867 cases (82% live births, 1.8% stillbirths and 16.2% pregnancy terminations).ResultsThe majority of cases (79.5%) could be identified with a single IPCCC code. The category "Heterotaxy, including isomerism and mirror-imagery" was the only one that typically required more than one code for identification of cases. The two largest categories were "ventricular septal defects" (52%) and "anomalies of the outflow tracts and arterial valves" (20% of cases).ConclusionOur proposed classification is not new, but rather a regrouping of the known spectrum of CHD into a manageable number of categories based on anatomic and clinical criteria. The classification is designed to use the code numbers of the long list of IPCCC but can accommodate ICD-10 codes. Its exhaustiveness, simplicity, and anatomic basis make it useful for clinical and epidemiologic studies, including those aimed at assessment of risk factors and outcomes.
Highlights
Classification of the overall spectrum of congenital heart defects (CHD) has always been challenging, in part because of the diversity of the cardiac phenotypes, and because of the oft-complex associations
Because of the diversity of the cardiac phenotypes, classification of the overall spectrum of congenital cardiac defects has always been challenging, with the challenge exacerbated by the oft-complex association of intracardiac and extracardiac defects
Our suggested re-classification separates the entire spectrum of CHD into a manageable number of categories that use the code numbers of the International Paediatric and Congenital Cardiac Code (IPCCC) long list, and are based on anatomic criteria, as well as those used for clinical and surgical management
Summary
Classification of the overall spectrum of congenital heart defects (CHD) has always been challenging, in part because of the diversity of the cardiac phenotypes, and because of the oft-complex associations. ICD-10 was created by the World Health Organization to “permit the systematic analysis, the interpretation and the comparison of the mortality and morbidity data harvested in different countries or regions at different periods of time” [1] It is based on the frequency of the various groups of diseases. The group of congenital anomalies, and CHD, is poorly detailed, and includes many doublets and inaccuracies This classification, despite its wide use by non-specialists, is increasingly considered inadequate by paediatric cardiologists and cardiac surgeons for describing the manifold congenital cardiac malformations [3,4,5]
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