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

Abstract

OBJECTIVESCongenital 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.METHODSCongenital SVAS repairs from 2001 to 2010 were retrospectively reviewed. The follow-up records, reintervention and reoperation data and most recent echocardiograms were obtained.RESULTSFrom 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.CONCLUSIONSExtended 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. © The Author 2013. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.