Objective: Congenitally corrected transposition of the great arteries (ccTGA) is a rare anomaly which can lead to reduced survival in adult age. We aimed to determine what potential problems those patients may face and what is their prognosis in longer time perspective.
Methods: Clinical records since 1995 of all patients with ccTGA were retrospectively reviewed and analyzed for clinical status, systemic right ventricular (RV) dysfunction, atrioventricular regurgitation, heartblock, arrhythmias, surgical procedures and mortality.
Results: Data were obtained from 30 patients. The mean length of follow-up was 7 years. Out of 30, 4 patients died (13,3%) at a mean age of 49,5 years. The longest survival was 77 years! The most common anomaly associated with ccTGA was pulmonary stenosis (33,3%) and VSD (30%). Surgery was performed in 13 patients (43.3%). Twelve patients have had permanent pacemakers implanted because of complete heart block which developed either spontaneously or as a consequence of surgery. Endocarditis was observed in one patient. By univariate analysis, atrial fibrillation was a risk factor for increased mortality (p=0,01). Risk factors for developing heart failure included age (p=0,03), atrial fibrillation (p=0,002), tricuspid valve replacement (p=0,0008), RV end-diastolic diameter (p=0,01).
Conclusions: ccTGA is not a benign lesion in adults although long survivals can be observed. Large group of ccTGA patients show tendency to have complete heart block. Atrial fibrillation is associated with heart failure and increased mortality.