From July 1982 through September 1994, 19 children had operative treatment of subaortic stenosis associated with an atrioventricular septal defect. Specific diagnosis were septum primum defects in 7, Rastelli type A defects in 6, transitional defects in 4, inlet ventricular septal defect with malattached chordae in 1, and tetralogy of Fallot with Rastelli type C defect in 1. Twenty-seven operations for subaortic stenosis were performed. Surgical treatment of the outlet lesion was performed at initial atrioventricular septal defect repair in 3 children and in the remaining 16 from 1.2 to 13.1 years (mean 4.9 years, median 3.9 years) after repair. Eighteen of the 19 children had fibrous resection and myectomy for relief of obstruction. Seven children had an associated left atrioventricular valve procedure. One child received an apicoaortic conduit. Seven children (36.8%) required 8 reoperations for previously treated subaortic stenosis. Time to the second procedure was 2.8 to 7.4 years (mean 4.9 years). Follow-up is 0.4 to 14.0 years (median 5.6 years). Six-year actuarial freedom from reoperation is 66% +/- 15%. The angle between the plane of the outlet septum and the plane of the septal crest was measured in 10 normal hearts (86.4 +/- 13.7) and 10 hearts with atrioventricular septal defects (22.2 +/- 26.0; p < 0.01). The outflow tract can be effectively shortened, widened, and the angle increased toward normal by augmenting the left side of the superior bridging leaflet and performing a fibromyectomy.
Conclusion: Standard fibromyectomy for subaortic stenosis in children with atrioventricular septal defects leads to a high rate of reoperation. Leaflet augmentation and fibromyectomy may decrease the likelihood of reoperation.