title: Mid-term outcome after surgical repair of congenital supravalvular aortic stenosis by extended aortoplasty.
abstract: OBJECTIVE
  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.
authors.affiliation: Department of Cardiac Surgery, University of Leipzig, Heart Center, Leipzig, Germany.
authors.forename: Farhad
authors.initials: F
authors.lastname: Bakhtiary
authors.forename: Mohammed
authors.initials: M
authors.lastname: Amer
authors.forename: Christian D
authors.initials: CD
authors.lastname: Etz
authors.forename: Ingo
authors.initials: I
authors.lastname: Dähnert
authors.forename: Friedrich
authors.initials: F
authors.lastname: Wilhelm Mohr
authors.forename: Wilfried
authors.initials: W
authors.lastname: Bellinghausen
authors.forename: Martin
authors.initials: M
authors.lastname: Kostelka
mesh_terms: D001021:Aortic Valve / Q000000981:diagnostic imaging / Q000601:surgery*; D064592:Autografts; D006348:Cardiac Surgical Procedures / Q000009:adverse effects / Q000401:mortality; D002648:Child; D002675:Child, Preschool; D015150:Echocardiography, Doppler; D005260:Female; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D010496:Pericardium / Q000637:transplantation; D012189:Retrospective Studies; D013997:Time Factors; D016896:Treatment Outcome; D018980:Williams Syndrome / Q000175:diagnosis / Q000401:mortality / Q000601:surgery*
keywords: Outcome; Supravalvular aortic stenosis; Williams–Beuren syndrome