ABSTRACT
This rare condition (0.5% of all congenital heart disease) is characterized by both atrioventricular (AV) and ventriculoarterial (VA) discordance. Thus, physiologically the circulation is “corrected” in the sense that the systemic venous blood is directed to the lungs (via a morphological left ventricle) and the pulmonary venous return is directed to the aorta. However, the systemic ventricle is a morphological right ventricle (mRV), guarded by a tricuspid valve. The condition was first described by Rokitansky in 1875 and is typified by laevo-or L-transposition in which the transposed aorta sits anterior and to the left of the pulmonary artery.
