A nomenclature is described for congenital heart disease employing sequential chamber localization. It is an eclectic system based in part upon the previous classifications of Van Praagh and Kirklin. It links together the atrial, ventricular, and arterial segments of the heart and then permits tabulation of associated anomalies. The atrial segment of the heart can exist as situs solitus, situs inversus, or situs ambiguus. Atrioventricular connexions can be concordant or discordant. In certain circumstances the terms concordant and discordant cannot be used. These are in the presence of primitive ventricle and in the presence of situs ambiguus. Alternative terms are described for these contingencies. Ventriculo-arterial connexions can be (a) normal; (b) transposition; (c) double outlet ventricle; or (d) single arterial trunk. These are defined as connexions; relations are relegated to secondary position. Associated anomalies are categorized in terms of venous return, atria, atrioventricular junction, ventricles, and great arteries. Controversial topics are discussed with regard to previous definitions.
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