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Lookup NU author(s): Professor Bob Anderson
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Background: Classification 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). Methods: We 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). Results: The 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). Conclusion: Our 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.
Author(s): Houyel L, Khoshnood B, Anderson RH, Lelong N, Thieulin AC, Goffinet F, Bonnet D, EPICARD Study Group
Publication type: Article
Publication status: Published
Journal: Orphanet Journal of Rare Diseases
Year: 2011
Volume: 6
Issue: 0
Pages: ARTN 64
Print publication date: 03/10/2011
ISSN (print): 1750-1172
ISSN (electronic):
Publisher: BioMed Central Ltd.
URL: http://dx.doi.org/10.1186/1750-1172-6-64
DOI: 10.1186/1750-1172-6-64
PubMed id: 21968022
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