Mid-term results after surgical repair of congenital supravalvular aortic stenosis by extended aortoplasty

Objectives: Congenital supravalvar aortic stenosis (SVAS) is a rare arteriopathy associated with Williams-Beuren syndrome (WBS) and other elastin gene deletions. Our objectives were to review the mid-term outcome of SVAS repair with extended aortoplasty.

Methods: Congenital SVAS repairs from 2001 to 2010 were retrospectively reviewed. Follow-up records, reintervention and reoperation data, and most recent echocardiograms were obtained.

Results: From 2001 to 2010, 21 patients (15 male) underwent surgical repair of SVAS by extended aortoplasty with autologous pretreted pericardium a modification of „Doty“ technique. Mean age was 3.1±4.2 years. WBS was diagnosed in 14 patients. There was no early mortality but one late death. At latest follow-up (mean follow-up, 4.3±2.9 years; range 1 month to 108 months). Echocardiograms revealed a peak Doppler gradient across the aortic outflow tract of 15±8mm Hg. All patients had minimal to mild aortic insufficiency. No reoperation no re-intervention was required.

Conclusions: Extended aortoplasty provides excellent mid-term relief of supravalvular aortic stenosis and, in addition, restores the aortic root geometry to a much more anatomical configuration. It can be done at no greater operative risks. Midterm results are excellent.