Anaesthetic considerations in a rare presentation of transposition of great arteries in adulthood with an unusual association of severe valvular aortic stenosis

Transposition of the Great Arteries (TGA) with Intact Ventricular Septum (IVS) is incompatible with life due to systemic circulation of deoxygenated blood. It usually presents in the neonatal period with cyanosis and an arterial switch operation is done within 3 weeks of life. We present a case of TGA with IVS and severe valvular Aortic Stenosis (AS) presenting for the first time in adulthood. The occurrence of valvular AS with dextro-TGA is a rarity and the anaesthetic considerations in such a case have not been reported. The patient survived till adulthood due to the presence of a large atrial septal defect. Worsening of AS leading to heart failure led to the delayed presentation. Understanding the circulatory pathway and pathophysiology of congenital heart disease before and after surgical correction is essential in managing heart failure peri-operatively. Discussing the anaesthetic considerations in such a case is paramount due to multiple unusual circulatory abnormalities.