Outcome after surgical repair/palliation of congenitally corrected transposition of the great arteries

Background: The surgical concepts for patients with congenitally corrected transposition of the great arteries (CCTGA) address discordant connections and associated lesions. The outcomes after biventricular repair without correction of discordant connections ("classic repair", or with its correction "anatomic repair") and after "univentricular palliation" were investigated. Methods: All patients with CCTGA who underwent "classic repair" (n = 39), "anatomic repair" (n = 6), or "univentricular palliation" (n = 11) between 1978 and 2006 were analyzed. The most frequently associated lesions were ventricular septal defect (n = 48), tricuspid insufficiency (TI) (n = 20) and functionally single ventricle (n = 11). Results: Thirty-day mortality was 4% (2/56). Mean follow-up for early survivors was 7.2 ± 7.1 years. Eight patients died late, two after heart transplantation. Survival was not significantly different between patients who underwent "anatomic" or "classic repair", or "univentricular palliation": 83.3 ± 15.2%, 79.7 ± 6.9%, 90.9 ± 8.7% at 10 years, respectively. In multivariate analysis, the presence of TI emerged as the only risk factor for late death (p = 0.004). Twenty patients required reoperation, mainly for TI (n = 10) and conduit failure (n = 6). Freedom from reoperation was lower after "anatomic repair", but ventricular function was better and atrioventricular valves were more competent than after "classic repair". Conclusions: Biventricular "anatomic" or "classic repair" and "univentricular palliation" yield equivalent survival rates in the mid-term. Biventricular "anatomic repair", when feasible, should be promoted because of its better long-term outcome.