• Luis E De León, Carlos M Mery, Raymond A Verm, Daniel Trujillo-Díaz, Ankita Patro, Francisco A Guzmán-Pruneda, Iki Adachi, Jeffrey S Heinle, Lauren C Kane, McKenzieE DeanEDDivision of Congenital Heart Surgery, Texas Children's Hospital, Houston, TX, and Michael E DeBakey Department of Surgery, Baylor College of Medicine, Houston, TX., and Charles D Fraser.
• Division of Congenital Heart Surgery, Texas Children's Hospital, Houston, TX, and Michael E DeBakey Department of Surgery, Baylor College of Medicine, Houston, TX. Electronic address: lxdeleon@texaschildrens.org.
• J. Am. Coll. Surg. 2017 Apr 1; 224 (4): 707715707-715.

BackgroundOptimal management of patients with congenitally corrected transposition of the great arteries (ccTGA) is unclear. The goal of this study was to compare the outcomes in patients with ccTGA undergoing different management strategies.Study DesignPatients with ccTGA believed suitable for biventricular circulation, treated between 1995 and 2016, were included. The cohort was divided into 4 groups: systemic right ventricle (RV) (patients without surgical intervention or with a classic repair), anatomic repair, Fontan palliation, and patients receiving only a pulmonary artery band (PAB) or a shunt. Transplant-free survival from presentation was calculated for each group.ResultsThe cohort included 97 patients: 45 (46%) systemic RV, 26 (27%) anatomic repair, 9 (9%) Fontan, and 17 (18%) PAB/shunt. Median age at presentation was 2 months (range 0 days to 69 years) and median follow-up was 10 years (1 month to 28 years). At initial presentation, 10 (11%) patients had any RV dysfunction (8 mild, 2 severe), and 16 (18%) patients had moderate or severe tricuspid regurgitation (TR). During the study, 10 (10%) patients died, and 3 (3%) patients underwent transplantation. At last follow-up, 11 (11%) patients were in New York Heart Association class III/IV, 5 (5%) had moderate or severe systemic ventricle dysfunction, and 16 (16%) had moderate or severe systemic atrioventricular valve regurgitation. Transplant-free survivals at 10 years were 93%, 86%, 100%, and 79% for systemic RV, anatomic repair, Fontan palliation, and PAB/shunt, respectively (p = 0.33). On multivariate analysis, only systemic RV dysfunction was associated with a higher risk for death or transplant (p = 0.001).ConclusionsTransplant-free survival in ccTGA appears to be similar between patients with a systemic RV, anatomic repair, and Fontan procedure. Systemic RV dysfunction is a risk factor for death and transplant.Copyright © 2017 American College of Surgeons. Published by Elsevier Inc. All rights reserved.

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