Anatomic repair for congenitally corrected transposition of the great arteries: a single-institution 19-year experience.

OBJECTIVE(S): Anatomic repair for congenitally corrected transposition of the great arteries (ccTGA) has been shown to improve patient survival. We sought to examine long-term outcomes in patients after anatomic repair with focus on results in high-risk patients, the fate of the neo-aortic valve, and occurrence of morphologically left ventricular dysfunction.

METHODS: We conducted a retrospective, single-institution study of patients undergoing anatomic repair for ccTGA. A total of 113 patients from 1991 to March 2011 were included. Double-switch (DS) repair was performed in 68 patients, with Rastelli-Senning (RS)-type repair in 45. Pulmonary artery banding for retraining was performed in 23 cases. Patients were followed up for survival status, morbidity, and reinterventions. A subgroup of 17 high-risk patients in severe heart failure, ventilated, and on inotropes before repair, were included.

RESULTS: Median age at repair was 3.2 years (range, 25 days to 40 years) and weight was 14.3 kg (3.2-61.4). There were 5 (of 68; 7.4%) early deaths in the DS group and 0 (of 45) in the RS group. Actuarial survivals in the DS group were 87.6%, 83.9%, 83.9% at 1, 5, and 10 years versus 91.6%, 91.6%, 77.3% in the RS group (log-rank: P = .98). Freedom from death, transplantation, or heart failure was significantly better in the RS group at 10 years (P = .03). There was no difference in reintervention at 10 years (DS, 50.3%; RS, 49.1%; P = .44). In the DS group, the Lecompte maneuver was associated with late reinterventions on the pulmonary arteries. Overall survival in the high-risk group was 70.6%. During follow-up, 14.2% patients had poor function of the morphologically left ventricle, all in the DS group, but this was not related to preoperative status or previous banding. The majority of patients after DS had mild aortic incompetence, which appeared well tolerated. Annuloplasty of the aortic root at time of DS reduced the risk of late aortic valve replacement.

CONCLUSIONS: There is significant morbidity after anatomic repair of ccTGA, which is higher in the DS than the RS group. Nevertheless, the majority of patients are free of heart failure at 10 years, including high-risk patients in severe heart failure before repair. Aortic annuloplasty may reduce risk of late aortic insufficiency.