Anatomically corrected malposition of the great arteries. Report of 2 cases, one with congenital asplenia; frequent association with juxtaposition of atrial appendages

Abstract

Anatomically corrected malpositions are uncommon cardiac anomalies in which the pulmonary artery originates above the morphological right ventricle, and the aorta above the morphological left ventricle. However, because of interposition of abnormal subaortic conal myocardium, there is absence of aortic valve mitral valve fibrous continuity. The relation between the pulmonary artery and aorta is also abnormal because of the abnormal conal morphology. Two cases of anatomically corrected malposition of the great arteries are presented, one of which is the first reported in a patient with the congenital asplenia syndrome; in the other, there was left juxtaposition of the atrial appendages. Review of all known cases of anatomically corrected malposition reveals a significant association with juxtaposition of the atrial appendages and hence with hypoplasia of the right ventricle, subpulmonary and subaortic obstruction. Possible surgical implications of this association are discussed.