Objectives: In patients with congenitally corrected transposition of the great arteries( cc-TGA), the morphological right ventricle (RV) sustains the systemic circulation and is frequently associated with progressive tricuspid regurgitation and declining RV function.
Methods: Thirty-four patients with cc-TGA and systemic RV were enrolled in this retrospective study to examine long-term outcome.
Results: Age was averaged 38.5 years. Associated cardiac anomaly included ventricular septal defect (VSD) and pulmonary stenosis in 18, VSD in 4. Twelve had no associated cardiac defect. The physiologic repair was performed in 22 patients at the mean age of 7.8 years old. Reoperation was frequent including tricuspid valve surgery in 11 and left ventricle( LV)-pulmonary artery( PA) conduit replacement in 10. Five patients needed pacemaker implantation due to heart block. Aortic insufficiency was a relatively common finding and aortic valve replacement was performed in 4. All but 2 patients were better than New York Heart Association (NYHA) class Ⅱ. One needed implantable ventricular assist device implantation and the other underwent heart transplantation.
Conclusions: Long-term outcome for patients with cc-TGA was satisfactory. Frequent reoperations including tricuspid valve surgery and LV-PA conduit replacement were required. Further careful follow-up will be needed to detect the progression of RV dysfunction and tricuspid and aortic insufficiency.