Document Detail

Twenty-year surgical experience with congenital supravalvar aortic stenosis.
MedLine Citation:
PMID:  19379894     Owner:  NLM     Status:  MEDLINE    
BACKGROUND: Congenital supravalvar aortic stenosis (SVAS) is an arteriopathy associated with Williams-Beuren syndrome and other elastin gene deletions. Our objectives were to review outcomes of congenital SVAS repair and to compare prosthetic patch repair techniques to all-autologous slide aortoplasty. METHODS: Congenital SVAS repairs from 1988 to 2008 were retrospectively reviewed. Peak instantaneous gradients were estimated by Doppler interrogation. Variables were compared by either Student's t test or Fisher's exact test. Risk factors were analyzed by chi(2) test. Survival was estimated by the Kaplan-Meier method. RESULTS: Of 25 primary SVAS repairs, there were 10 all-autologous slide aortoplasties and 15 prosthetic patch aortoplasties. The prosthetic patch group included the Doty technique (n = 9), patch-augmented slide aortoplasty (n = 3), modified Brom technique (n = 1), interposition graft (n = 1), and two-sinus patch with transverse arch augmentation (n = 1). There was 1 early and 1 late death. Cumulative survival for all patients was 96% at 5 and 10 years. Event-free survival did not differ between groups (p = 0.481). There were 2 late reoperations (both were prosthetic patch patients with bicuspid aortic valve: 1 with recurrent aortic valve stenosis and 1 with aortic insufficiency). Bicuspid aortic valve was the only risk factor for reoperation (p = 0.003). Three patients weighing less than 10 kg with diffuse disease underwent attempted slide aortoplasty: 2 required patch augmentation and 1 had a recurrent gradient in less than 1 year postoperatively. CONCLUSIONS: Outcomes after SVAS repair were good by any technique. No advantage to all-autologous slide aortoplasty was apparent at current follow-up. Based on our experience, slide aortoplasty is not recommended for small patients with diffuse disease.
Daniel J Scott; David N Campbell; David R Clarke; Steven P Goldberg; Daniel R Karlin; Max B Mitchell
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Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  The Annals of thoracic surgery     Volume:  87     ISSN:  1552-6259     ISO Abbreviation:  Ann. Thorac. Surg.     Publication Date:  2009 May 
Date Detail:
Created Date:  2009-04-21     Completed Date:  2009-05-14     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  15030100R     Medline TA:  Ann Thorac Surg     Country:  Netherlands    
Other Details:
Languages:  eng     Pagination:  1501-7; discussion 1507-8     Citation Subset:  AIM; IM    
The Children's Hospital Heart Institute and University of Colorado Denver Health Sciences Center, Aurora, Colorado, USA.
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MeSH Terms
Aorta, Thoracic / surgery
Aortic Stenosis, Supravalvular / mortality,  surgery*,  ultrasonography
Aortic Valve Stenosis / mortality,  surgery*,  ultrasonography
Child, Preschool
Heart Defects, Congenital / mortality,  surgery*,  ultrasonography
Hemodynamics / physiology
Prosthesis Implantation / methods,  mortality
Retrospective Studies
Survival Analysis
Treatment Outcome
Williams Syndrome / surgery,  ultrasonography
Young Adult

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine

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