Nineteen patients with double outlet right ventricle and discordant atrioventricular connexion were studied in order to analyze their angiographic anatomy. The discordant atrioventricular connexion was parallel in 18 patients and criss-cross in one. A ventricular septal defect, present in all cases, was perimembranous in ten (50%), muscular in seven (37%), and was part of an atrioventricular septal defect in two. The defect was related spatially to the subaortic outflow in six (29%), to the subpulmonary outflow in 11 (52%), was doubly committed in one and non-committed in two. Long axial and four-chamber views of the ventriculograms were most useful for the angiographic delineation of the basic anatomy of this entity. In patients with abnormally located hearts, variations of the axial views were necessary for proper delineation of the anatomy. It is concluded that modified axial views of ventriculograms are useful for diagnosis of double outlet right ventricle and discordant atrioventricular connexions.