Congenitally Corrected Transposition of the Great Arteries: A Clinical and Surgical Study

Summary

From 1967 to 1979, 40 patients with the diagnosis of congenitally corrected transposition of the great arteries (C-TGA) have been followed. Associated cardiac defects were present in all but one patient, most frequently ventricular septal defect (80%), and pulmonary Stenosis (70%). Left sided atrioventricular valve dysfunction developed in 25%, third degree atrio-ventricular block (at least intermittently) in 33% of the patients. Twenty-eight patients were operated: palliative procedures were done in 6, corrective operations in 22 patients. Ten of the 40 patients have died during a mean observation period of 4 years: 4 early postoperatively, 3 late postoperatively and 3 non-operated patients. Sudden unexplained cardiac arrest has been the most frequent cause of death (2 late postoperative and 2 non-operated patients). The incidence of residual ventricular septal defects and residual pulmonary stenosis after corrective surgery has been relatively high owing to the complex anatomy in these patients. Also reconstruction of the atrio-ventricular valves has been difficult because of severe deformities, particularly on the left side. It is concluded that in C-TGA the pacemaker should be implanted early, at the first sign of AV-conduction disturbance. Since the relief of the pulmonary stenosis is difficult, the operation should be deferred until an adequate-sized conduit can be implanted.

Key words

Congenitally corrected transposition of the great arteries - Atrio-ventricular and ventriculo-arterial discordance