Methods: Between August 2009 and September 2014, data of a total of 81 patients (48 males, 33 females; mean age 4 years; range, 15 days to 50 years) with congenitally corrected transposition of the great arteries were retrospectively analyzed. The patients were divided into two groups: congenitally corrected transposition of the great artery patients with (group 1; n=66) and without (group 2; n=15) associated cardiac lesions. Age and gender of the patients, surgical history, atrioventricular block, arrhythmia, and systemic atrioventricular valve regurgitation were noted.

Results: The median follow-up was 15 (range 1 to 52) months. Sixteen patients had clinical signs of congestive heart failure in group 1, while none of the patients had in group 2. Surgery was performed in 31 patients in group 1 and none in group 2. Twelve patients were directed to biventricular correction (nine physiologic and three anatomic corrections). The Glenn shunt was performed in six patients and Fontan circulation in one. Of these patients with the Glenn shunt, one planned to underwent physiologic correction and four to the hemi-Mustard-Rastelli surgery. During follow-up, seven patients died and four patients developed systemic ventricular dysfunction. Cardiac resynchronization therapy was performed in two patients. Previous heart surgery, association of pulmonary atresia or stenosis, the presence of a large ventricular septal defect, tricuspid regurgitation, and atrioventricular block were found to be statistically significant risk factors for clinical congestive heart failure (p<0.05). Moderate to severe tricuspid regurgitation, arrhythmias, and the history of an open heart surgery were found to be significant risk factors for the right and left ventricular dysfunction (p<0.05).

Conclusion: Timing of clinical presentation and survival of the patients with congenitally corrected transposition of the great arteries mainly depend on the presence of associated cardiac defects. The development of tricuspid regurgitation, atrioventricular block and systemic ventricular dysfunction, shortly after physiologic repair highlights the consideration of anatomic correction methods in children, such as the double-switch at first hand.