REPAIR OF TETRALOGY OF FALLOT IN THE FIRST 6 MONTHS OF LIFE - TRANSATRIAL VERSUS TRANSVENTRICULAR APPROACH

Background. This report describes our experience with primary correcti on of tetralogy of Fallot in infants. Methods. Fifty-one consecutive i nfants younger than 6 months underwent primary correction of tetralogy of Fallot between January 1978 and October 1994. Mean age at repair w as 4.2 months. Four were neonates. Correction was accomplished through a right ventriculotomy in the first consecutive 22 patients (43%; gro up A); since 1991, a combined transatrial-transpulmonary approach was used in 29 consecutive patients (57%; group B). A transannular patch w as necessary in 33 infants (65%) 16 of group A (73%) and 17 of group B (59%). Results. There was one early death from possible left anterior descending coronary artery distortion in group A and no deaths in gro up B. Two patients required early reoperation for systemic-to-pulmonar y artery collateral ligation (postoperative day 6) and permanent pacem aker implantation (postoperative day 30). There were no late deaths. A ll 50 survivors are currently asymptomatic and in New York Heart Assoc iation class I. Three patients required late reoperations 36 months, 3 0 months, and 13 months after repair for (1) subaortic stenosis and dy sfunctioning dysplastic mitral valve, (2) residual pulmonary artery br anch stenosis, and (3) residual right ventricular outflow obstruction. Four patients underwent balloon dilation and stent insertion (1 patie nt) for peripheral pulmonary artery stenosis 1.5 year to 12 years (mea n, 5 years) after initial repair. Actuarial freedom from need for rein tervention at 4 years was 78.4% in group A and 85.7% in group B. Two-d imensional and Doppler echocardiographic follow-up studies showed a re sidual mild to moderate pulmonary artery branch stenosis in 4 patients in group A, and a recurrent subaortic stenosis in 1 patient in group A. Right ventricular peak systolic pressure was less than 40 mm Hg in all but 3 asymptomatic patients who had a residual pulmonary artery br anch stenosis. Right ventricular end-systolic and end-diastolic volume s showed larger volumes and reduced ejection fraction in group A compa red with group B. Conclusions. This limited experience with repair of tetralogy of Fallot in patients less than 6 months of age demonstrates that the transatrial-transventricular approach is possible in neonate s and young infants with a very low mortality and morbidity and also a low incidence of residual lesions. Follow-up echocardiographic data s uggest that right ventricular function is better preserved in those pa tients who underwent the transatrial-transpulmonary repair.