Tetralogy of Fallot: favorable outcome of nonneonatal transatrial, transpulmonary repair.

This report describes our experience with 366 patients who had a transatrial, transpulmonary repair of tetralogy of Fallot between December 1980 and December 1991. Included in this group are patients with tetralogy of Fallot plus atrioventricular septal defect as well as patients displaying all degrees of aortic override (in the presence of subaortic ventricular septal defect and right ventricular outflow tract obstruction). Median age was 15.3 months and median weight, 12.3 kg. Of the 366 patients, 72% required a pericardial patch to reconstruct the main pulmonary artery or right ventricular outflow tract. Serious coronary anomalies were seen in 11 patients, without influencing surgical approach. There were two hospital deaths (0.5%; 70% confidence limits, 0.2% to 1.2%). Actuarial survival was 97.5% at 42 months (95% confidence limits, 95% to 99%) reflecting four late deaths over 1,129 patient-years of follow-up. Postoperative cardiac catheterization studies were performed in 61 patients at a mean follow-up interval of 23 months. Mean right ventricular/left ventricular systolic pressure ratio after repair was 0.46 (standard deviation, 0.28), and mean gradient across the right ventricular outflow tract was 15 mm Hg (standard deviation, 24 mm Hg). Actuarial freedom from reoperation for any reason has been 95% (95% confidence limits, 92% to 97%) at 5-year and 10-year follow-up. These early and medium-term results encourage us to continue with transatrial, transpulmonary repair of tetralogy of Fallot. We believe that this approach has an operative risk similar to or lower than transventricular repair, and that it will result in better preservation of right ventricular function in the long term.