Aim of the study: To investigate the long-term outcome of surgical treatment for congenitally corrected transposition of the great arteries (CCTGA), in patients with biventricular repair with the right ventricle as systemic ventricle.Methods: A total of 32 patients with CCTGA were operated between January 1972 and October 2008. These operations comprised 18 patients with a repair with a normal left ventricular outflow tract, 11 patients with a Rastelli repair of the left ventricle to the pulmonary artery and 3 patients with a cardiac transplantation.Results: Excluding the cardiac transplantation patients, mean age at operation was 16 years (sd 15 years, range 1 week - 49 years). Median follow-up was 12 years (sd 10 years, range 7 days - 32 years). Survival obtained from Kaplan-Meier analysis at 20 years after surgery was 63% (CI 53-73%). For the non-Rastelli group these data at 20 years were 62% (CI 48-76%) and for the Rastelli group 67% (CI 51-83%). Freedom of reoperation at 20 years was 32% (CI 19-45%) in the overall group. In the non-Rastelli group the data at 20 years were 47% (CI 11-83%) and for the Rastelli group 21% (CI 0-54%) after almost 19 years.Conclusions: Long term follow up confirms that surgery in CCTGA with the right ventricle as systemic ventricle has a suboptimal survival and limited freedom of reoperation. Death occurred mostly as a result of cardiac failure.

Adolescent, Adult, Atrioventricular Block/etiology/therapy, Cardiac Surgical Procedures/adverse effects/methods, Child, Child, Preschool, Follow-Up Studies, Heart Ventricles/*surgery, Humans, Infant, Infant, Newborn, Kaplan-Meier Estimate, Middle Aged, Pacemaker, Artificial, Reoperation, Survival Rate, Tricuspid Valve Insufficiency, Ventricular Dysfunction, Right/etiology, Young Adult,
Journal of Cardiothoracic Surgery
Erasmus MC: University Medical Center Rotterdam

Bogers, A.J.J.C, Head, S.J, de Jong, P.L, Witsenburg, M, & Kappetein, A.P. (2010). Long term follow up after surgery in congenitally corrected transposition of the great arteries with a right ventricle in the systemic circulation. Journal of Cardiothoracic Surgery, 5(1). doi:10.1186/1749-8090-5-74