Atrioventricular (AV) discordance with ventriculoarterial (VA) concordance is a rare form of congenital heart disease that consists of 5 different anatomic types. The salient therapeutic consideration uniting these 5 different anatomic entities is that anatomic correction can be achieved by an atrial switch procedure with closure of any associated septal defect. Three patients who had AV discordance with VA concordance are presented, with emphasis upon 2-dimensional echocardiographic diagnosis and surgical management. One of these patients had ventricular inversion with inverted normally related great arteries in situs solitus (i.e., [S,L,I]), thus representing a form of congenital heart disease that until now has not been documented anatomically. Although these are complex defects, multiple-plane imaging with 2-dimensional echocardiography was found to be extremely reliable in ascertaining the anatomy when a comprehensive, segmental analysis is applied. This approach includes determination of visceroatrial situs, ventricular loop and great arterial position and alignment. A surgical approach to closure of the ventricular septal defect through the left-sided infundibulum in isolated ventricular inversion afforded excellent exposure of the defect and avoided AV conduction block.