Nomenclature and classification of congenital heart disease.

Abstract

SUMMARY At present there is no universally accepted nomenclature for congenital cardiac malformations. Much of the controversy results from failure to distinguish the structural connections of the heart from the morphology and spatial relations of its components. The confusion is compounded by an abundance of individual definitions, many of them speculative.The present article proposes a totally descriptive nomenclature. It describes in turn the connections of the cardiac segments, their morphology, their relations, and additional anomalies in any segment. Each step in the segmental approach is discrete. The overall effect is to force a succinct and comprehensive description of any cardiac malformation, no matter how complex.To be of value, a nomenclature must allow all congenital heart defects to be described unambiguously, accurately, and succinctly. It must assist the clinical cardiologist to diagnose and describe all these anomalies during life. It must be compatible with the morphological findings so that it is as valid for examinations at operation or necropsy as it is during diagnostic investigations. A classification and nomenclature should not be difficult to leam or to remember. Clarity, not erudition, is the touchstone of success.It is our contention that classifications at present in use do not satisfy these criteria. They lack flexibility and are frequently derived from morphogenetic concepts which are more or less speculative. In the less flexible systems, where a Procrustean approach is necessary, attempts to make them comprehensive result in sub-classifications with alphabetical and numerical designations (Edwards and Burchell, 1949) which have no descriptive value. In those based in part upon deductions from

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