Congenitally Corrected Transposition of the Great Arteries and Concomitant Coronary Artery and Valvular Disease in the Adult Patient

Congenitally corrected transposition of the great arteries (ccTGA) accounts for less that 1% of cardiac anomalies, and is defined as ventriculoarterial and atrioventricular (AV) discordance. The double discordant connection allows for survival with the right ventricle performing as the systemic ventricle, and the left ventricle as the pulmonary ventricle. We report a case of ccTGA in a 35-year-old male with situs inversus totalis status post repair of a ventricular septal defect (VSD) with a residual VSD, severe systemic AV valve regurgitation, and coronary artery disease who presented with chest pain. He subsequently underwent tricuspid valve replacement and VSD repair, followed by percutaneous coronary revascularization. This case highlights many important issues of adults with congenital cardiac disease, as well as the specific surgical management of anomalies associated with ccTGA. We review the literature and discuss the management of these complicated patients.