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

OBJECTIVES: Congenital supravalvular aortic stenosis (SVAS) is a rare arteriopathy associated with the Williams-Beuren syndrome (WBS) and other elastin gene deletions. Our objective was to review the mid-term outcomes of SVAS repair with extended aortoplasty.

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

RESULTS: From 2001 to 2010, 21 patients (15 males) underwent surgical repair of SVAS by extended aortoplasty with autologous pretreated pericardium, which is a modification of the Doty technique. The mean age was 3.1 ± 4.2 years. WBS was diagnosed in 14 of the patients. There was no early mortality, but one late death was observed. At the latest follow-up (mean follow-up, 4.3 ± 2.9 years; range, 1-108 months), echocardiograms revealed a peak Doppler gradient across the aortic outflow tract of 15 ± 8 mmHg. The majority of the patients had minimal to mild aortic insufficiency. No reoperation or reintervention was required.

CONCLUSIONS: Extended aortoplasty provides excellent mid-term relief of SVAS and, in addition, reshapes the aortic root geometry to a much more favourable anatomical configuration. It can be performed without any increase in operative risks. The mid-term results are excellent.