Congenital aortic stenosis with hypoplasia of the left sinus of Valsalva. Anatomic reconstruction of the aortic root.

Evaluation of the aortic root in 13 patients with congenital aortic stenosis aged 2.5 to 24 years (mean 8.3 years) has revealed morphologic characteristics of asymmetry of the aortic root caused by a small (hypoplastic) left sinus of Valsalva associated with a supravalvular ridge above the left coronary ostium and dysplasia of the aortic valve. The asymmetry resulted in folding and buckling of the left aortic cusp. The aortic valve was classified as bicuspid in 11 of the 13 patients. Preoperative aortography was characteristic and revealed the diagnosis in all patients. The average left ventricle-aorta systolic pressure gradient was 81 mm Hg. Operative repair consisted of an oblique aortotomy extended in a spiral fashion to the right and posteriorly into the left sinus of Valsalva. Seven patients had further mobilization of the posterior commissure with a second incision to the right of the commissure into the noncoronary sinus. Slightly fused valve commissures were opened in 12 patients. Aortic root reconstruction was accomplished with a spiral Dacron patch and posterior commissural repositioning. Follow-up catheterization at 9 to 35 months (mean 24 months) in five patients demonstrated an average outflow tract systolic gradient of 28 mm Hg and a more symmetrical appearance of the aortic root.