Background: Right ventricular (RV) impairment is a well recognized late sequelae of atrial inversion operation. Although conservative interventions as well as anatomic correction have been advocated late after Mustard or Senning procedures, heart transplantation remains the main treatment of patients with end-stage failure of the systemic RV. Aim of this study is to evaluate operative and postoperative results in patients undergoing heart transplantation for end-stage failure of the systemic RV following Mustard/Senning operations. Methods: Retrospective review of patients referred to the Regional Cardiothoracic Centre for cardiac transplantation was performed. Results: Since January 1987, 62 out of 567 patients underwent heart transplantation for congenital heart disease. Nine of these had a previous atrial inversion operation performed (6 Mustard, 3 Senning) for TGA. Mean age at time of atrial inversion operation was 16.9 ± 10.1 months. Time interval between atrial inversion and heart transplant was 22.0 ± 11.6 years. Mean age at heart transplant was 23.6 ± 10.8 years. Three patients were on inotropic support before the operation, while one was on ECMO support. Indication for heart transplantation was end-stage RV faiure (mean NYHA class 3.6 ± 0.5) with severe impaired function and severe tricuspid valve regurgitation in all patients. Results are shown in Table 1. At a mean follow up time of 21.5 ± 10.09 months, 71.5% of hospital survivors are alive. Conclusions: Heart transplantation for end-stage RV failure following atrial inversion operation is a high-risk procedure; however it remains the main treatment for this group of patients. The presence of a failing RV and previous surgery increases the operative mortality and exposes patients to late complications.