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

Abstract：

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.

journal_name

Interact Cardiovasc Thorac Surg

journal_title

Interactive cardiovascular and thoracic surgery

authors

Bakhtiary F,Amer M,Etz CD,Dähnert I,Wilhelm Mohr F,Bellinghausen W,Kostelka M

doi

10.1093/icvts/ivt236

subject

Has Abstract

pub_date

2013-10-01 00:00:00

pages

688-90

issue

4

eissn

1569-9293

issn

1569-9285

pii

ivt236

journal_volume

17

pub_type

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