Anatomically Corrected Malposition of the Great Arteries (ACMGA) is a rare congenital heart disease in which the great arteries are abnormally related to each other and to the ventricles, but arise nonetheless above the anatomically correct ventricles. There are 4 types of ACMGA: Type 1 (S,D,L)-situs solitus, d-loop ventricles, left and anterior aorta; Type 2 (S,L,D)-situs solitus, l-loop ventricles, right and anterior aorta; Type 3 (I,L,D)-situs inversus, l-loop ventricles, right and anterior aorta; and Type 4 (I,D,L)-situs inversus, d-loop ventricles, left and anterior aorta. The existence of I,L,D type has been theorized but not demonstrated in a patient till date. We report a case of ACMGA type (I,L,D) in a 9 year old child. To our knowledge this is the first case of ACMGA (type I,L,D) reported in literature so far.
