Objective: Intracardiac malformations associated with coarctation and aortic arch hypoplasia have traditionally been repaired in 2 stages, with a high mortality rate. We review our experience with single-stage biventricular repair of intracardiac defects associated with aortic arch hypoplasia by means of pulmonary homograft patch aortoplasty.
Methods: Between October 1988 and October 1997, 39 of 40 consecutive patients underwent single-stage biventricular repair for aortic arch obstruction and associated intracardiac defects. The median age at operation was 17 days and the mean weight was 3.71 +/- 1.09 kg. Nineteen patients had either dextrotransposition of the great arteries or the Taussig-Bing anomaly. Sixteen patients had multiple left-sided obstructive lesions (2 cases of critical aortic stenosis, 3 of subaortic stenosis and ventricular septal defect, and 11 of hypoplastic left heart complex). One patient had an associated complete atrioventricular septal defect. Four patients had only an associated ventricular septal defect. Through a median sternotomy, the hypoplastic aortic arch was enlarged with a pulmonary homograft patch in 36 patients. In 4 patients an extended end-to-end anastomosis was performed.
Results: There were 2 early deaths (5%) and 2 late deaths (5%). One late death was not cardiac related. The mean follow-up time was 36 months (range 1 month-9 years). The recoarctation rate after pulmonary homograft patch aortoplasty was 8. 3%, but after exclusion of those patients with associated left-sided obstructive lesions this decreased to 0%. No aneurysm formation in the aorta has occurred. The actuarial survival at 8 years is 89% +/- 10%.
Conclusions: Single-stage biventricular repair of aortic arch obstruction and associated intracardiac defects can achieve excellent survival. We recommend pulmonary homograft patch aortoplasty because it achieves complete relief of anatomic afterload with a tension-free anastomosis and low incidence of recoarctation.