Outcome of 121 patients with congenitally corrected transposition of the great arteries

Congenitally corrected transposition of the great arteries (ccTGA) is a rare disorder with reduced survival that is influenced by the presence of associated anomalies, tricuspid regurgitation (TR), and right ventricular (RV) function. The double switch procedure has been proposed as an aggressive surgical approach in selected patients. We sought to review our experience with conventional repair to determine if a change in surgical strategy was warranted. Clinical records of 121 patients with ccTGA and two adequate-sized ventricles were retrospectively reviewed. Median length of follow-up was 9.3 years; 5-, 10-, and 20-year survival rates were 92%, 91%, and 75%, respectively. Surgery was performed in 86 patients, including conventional biventricular repair in 47 patients. Risk factors for mortality by univariate analysis included age at biventricular repair (p = 0.04), complete atrioventricular (AV) canal defect (p = 0.02), dextrocardia (p = 0.05), moderate or severe TR (p = 0.05), and poor RV function (p = 0.001). By multivariate analysis, complete AV canal defect (p = 0.006) and poor RV function (p = 0.002) remained significant as risk factors for mortality. Risk factors for the development of significant TR included conventional biventricular repair (p = 0.03) and complete AV block (p = 0.04). Risk factors for progressive RV dysfunction included conventional biventricular repair (p = 0.02), complete AV block (p = 0.001), and moderate or severe TR (p < 0.001). This is the largest nonselected cohort of patients with ccTGA followed at a single center. Our results confirm that significant TR and poor RV function are risk factors for poor outcome and provide convincing evidence that patients undergoing conventional biventricular repair are at higher risk for deterioration of tricuspid valve and right ventricular function compared to palliated or unoperated patients. We support a move toward an alternative surgical approach (double switch procedure) in carefully selected patients.