Background: Type B interrupted aortic arch with ventricular septal defect is a complex congenital heart defect that may have associated left ventricular outflow tract obstruction. Surgical management has evolved from a two-stage approach to the currently favored single-stage approach. The following data summarize our experience with the two-stage approach over a 15-year period.
Methods: Between 1980 and 1995, 27 consecutive patients with type B interrupted aortic arch and ventricular septal defect underwent surgical management using the two-stage approach. There were 15 girls and 12 boys; 21 patients had the DiGeorge syndrome.
Results: Stage I was performed at a median age of 4 days. Twenty-six (96%) of 27 patients survived first-stage palliation. One patient survived stage I palliation but died before undergoing stage II. Twenty-five patients underwent second-stage repair at a median age of 6 weeks (range, 1 to 46 weeks). There were 2 early deaths and 1 late death. Actuarial analysis demonstrates 1- and 5-year survival rates of 85% and 81%, respectively. Twenty-two survivors have been followed up for an average of 8 +/- 2 years. Freedom from reoperation for arch graft enlargement has been 86% at 3 years and 55% at 5 years. Freedom from reoperation for left ventricular outflow tract obstruction has been 82% at both 3 and 5 years.
Conclusions: The two-stage approach can achieve good mid- to long-term palliation of patients with type B interrupted aortic arch and ventricular septal defect. These results should provide a reference from which to gauge the long-term success of the single-stage approach.