The natural and "unnatural" history of congenitally corrected transposition.

The data available on 111 patients with congenitally corrected transposition and 2 adequate ventricles managed over the 20-year period to 1988 were reviewed retrospectively. The ages of survivors ranged from 1 to 58 years (median 20) and all but 10 had additional anatomic abnormalities. Tricuspid valve abnormalities were more prevalent in patients symptomatic with heart failure (26 of 43 patients) than those whose main problem was cyanosis (11 of 52 patients); all dysplastic or Ebstein valves were at least moderately incompetent. Intracardiac repair of the lesion was attempted in 51 patients with 11 early deaths; in multivariate models, the risk factors for early death or a bad early outcome or poor result 6 months later related to poor preoperative symptomatic status (especially from heart failure), impaired right ventricular function, heart block and younger age at surgery. Patients with more than mild preoperative tricuspid regurgitation whose valves were not replaced did very poorly. Thus, patients symptomatic from heart failure should probably be repaired early in the natural history of the disease, before the systemic right ventricles dilate. By contrast, the course of patients who were predominantly cyanosed was more stable in early childhood and their surgical outcome was less compromised by poor preoperative symptomatic status; their intracardiac repair can probably be delayed until symptoms become unacceptable.