Congenitally Corrected Transposition of the Great Arteries: Current Treatment Options

Congenitally corrected transposition of the great arteries is a relatively rare cardiac malformation characterized by atrioventricular and ventriculoarterial discordance. This double discordance results in a physiologically corrected circulation with the morphologic right ventricle (RV) serving as the systemic pump. Associated anomalies are present in approximately 98% of cases and include most commonly ventricular septal defect, pulmonary stenosis, and anomalies of the systemic atrioventricular valve (SAVV). Conduction abnormalities are common as well, predisposing these patients to the development of complete heart block. The management of these patients is primarily determined by the presence and severity of the associated anomalies. Conventional repair, which leaves the morphologic RV as the systemic ventricle, has resulted in high incidence of tricuspid regurgitation (TR) and progressive dysfunction of the RV. In the majority of patients, congestive heart failure secondary to RV dysfunction occurs by the fifth or sixth decade. The cause and effect relationship between TR and RV dysfunction remains to be determined. The advent of the Double Switch operation, which restores the morphologic left ventricle (LV) as the systemic ventricle, has yielded favorable outcomes at early follow-up. This procedure should be applied in young patients to achieve optimal results. Retraining the LV by pulmonary artery banding is associated with high morbidity and mortality when attempted after infancy or early childhood. Long-term follow-up is needed to evaluate the potential superiority of this procedure and the incidence of rhythm, baffle, and conduit complications for this management option.