Search

Galactosaemia is an autosomal recessive metabolic disorder that occurs due to galactose-1-phosphate uridyl transferase enzyme deficiency that leads to hepatic, ophthalmic, neural and renal derangements. Association of cyanotic congenital heart disease with galactosaemia is a rare occurence and a matter of great concern for the anaesthesiologist during open heart surgery. In this case report, the perioperative management of a newborn with galactosaemia operated for correction of transposition of great arteries (TGA) is discussed.