Congenitally Corrected Transposition of the Great Arteries plus Dextrocardia Operated with an Unusual Operative Technique

All articles of this category (opens in new window) Introduction Congenitally corrected transposition of the great arteries (CCTGA) described by Schiebler and colleagues [ 1 ] is a rare form of congenital heart disease for which significant tricuspid insufficiency (TI) has been reported in 20 % to 50 % of patients [ 2 ]. Dysfunction, mostly regurgitation of the morphologically tricuspid systemic atrioventricular valve, is one of the strongest risk factors for clinical congestive heart failure (CHF) and right ventricular dysfunction. The main underlying disorder in CCTGA is atrioventricular and ventriculoarterial discordance. The morphologic right ventricle functions as the systemic ventricle, and the morphologic left ventricle functions as the pulmonary ventricle. The atrioventricular valve connected to the systemic ventricle is morphologically tricuspid, and the valve connected to the pulmonary ventricle is morphologically mitral. Although in some patients the morphologic right ventricle retains its normal function even into late adulthood, some dysfunction of the systemic ventricle occurs progressively with age [ 3 ]. Various surgical approaches have been proposed to treat this anomaly. We report a patient with CCTGA who underwent a left (tricuspid) atrioventricular (AV) valve replacement through a left atriotomy anterior to the left pulmonary veins with an excellent postoperative outcome.