Management of the Adult Patient With Congenitally Corrected Transposition: Challenges and Uncertainties

Congenitally corrected transposition (ccTGA) is a rare form of congenital heart disease characterized by atrioventricular and ventriculoarterial discordance. Patients with ccTGA usually have associated congenital cardiovascular conditions; less than 1% have no associated lesions. Generally, ccTGA is identified during infancy or childhood with features of heart failure or cyanosis when there are associated lesions such as ventricular septal defect and/or pulmonic stenosis. Presentation later in life generally occurs when there are either mild or no associated lesions. Presentation during adulthood may be prompted by symptoms or signs of cardiovascular disease or due to abnormal findings on cardiac testing. Management of patients with ccTGA depends on presentation, symptoms, and associated defects. In this review, we will focus on the management of adult patients with ccTGA.