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A heart is described with double inlet left ventricle, rudimentary right ventricle and ventriculo-arterio discordance. The lesion was complicated by a malformation of the left atrioventricular valve. The valve was divided into two distinct orifices. The major orifice connected to the morphologically left ventricle along with the right atrioventricular valve. The minor orifice opened predominantly into the rudimentary right ventricle but in part to the dominant left ventricle. The problems such a lesion produces in terms of nomenclature are discussed with regard to sequential segmental analysis.
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