Anatomic correction for complete transposition and double-outlet right ventricle

Between February, 1981, and December, 1984, 30 patients underwent anatomic correction of transposition of the great arteries with intact ventricular septum (n = 8), transposition with ventricular septal defect (n = 15), and double-outlet right ventricle with subpulmonary ventricular septal defect, the Taussig-Bing anomaly (n = 7). At operation, ages ranged from 18 hours to 6 years (mean 11.3 months) and weights ranged from 2.6 to 16.4 kg (mean 6.1 kg). The group with transposition and intact ventricular septum on average was younger (mean 1.2 months) and smaller (mean 3.5 kg) than the other two groups. Associated congenital heart defects were seen in 12 patients, including five with coarctation, three with multiple ventricular septal defects, two with right ventricular hypoplasia, two with juxtaposed atrial appendages, and one each with interrupted aortic arch, Wolff-Parkinson-White syndrome, and left ventricular outflow tract obstruction. All 10 patients who had undergone prior palliative operations had pulmonary artery banding. In addition, four of these patients had coarctation repairs, four had atrial septectomy, and one had systemic/pulmonary shunting. All recognized patterns of coronary anatomy were encountered. The aorta and pulmonary artery were side by side in 14 patients and anteroposterior in 16 patients. The Lecompte maneuver to establish right ventricular-pulmonary arterial continuity was successfully used in 12 of 13 patients with anteroposterior great vessels but in none of those with side-by-side arteries. Seven patients had subvalvular right ventricular outflow tract obstruction, recognized either at operation (five) or postoperatively (two). This was responsible for death in three patients. The 30 day hospital mortalities were as follows: one death (12.5%) in the group with transposition and intact ventricular septum, six deaths (40%) in the group with transposition plus ventricular septal defect, and one death (14.3%) among patients with double-outlet right ventricle and subpulmonary ventricular septal defect; the overall mortality was eight deaths (26.7%). There have been no late deaths (mean follow-up 17.2 months). Ninety-five percent of the survivors are in New York Heart Association Functional Class I. Postoperative catheterization in 13 patients has shown normal left ventricular function, no coronary stenosis, and no aortic incompetence. Sixty-nine percent of these patients had clinically unsuspected gradients across the right ventricular outflow tract, which may be prevented by avoiding the Lecompte maneuver or the use of conduits.