Double-switch operation for congenitally corrected transposition.

Since June, 1989, 44 patients with congenitally corrected transposition associated with AV discordance who were younger than 16 years-underwent anatomical correction, or double-switch operation; 4 of the patients (9.1%) died while still hospitalized. Although double-switch operation is a time-consuming procedure, and longer follow-up is warranted, it is best indicated for CTGA, especially in the presence of systemic tricuspid regurgitation, or in cases with poor systemic RV function. Preoperative tricuspid regurgitation improved spontaneously without surgical intervention on the valve, and mere unloading of the anatomical RV was proven sufficient to control the regurgitation in our series. Postoperative ventricular function can be favorably compared with that of the conventional Rastelli procedure in the complete transposition of the great arteries. Combined atrial switch and arterial switch procedures were performed in nine cases, and eight surviving patients were currently doing well in NYHA functional class 1 at the time this report was written. This combination of anatomical repair should be considered for cases with CTGA associated with VSD and pulmonary hypertension. Two types of anatomical repair that the author first described, combined arterial switch and the Senning procedure, and combined atrial switch and direct anastomosis between the RV and the distal pulmonary artery, seemed to be preferable methods for performing anatomical correction in CTGA.