Document Detail

Progressive discrete subaortic stenosis developed after successful primary repair of the supravalvular aortic stenosis in Williams syndrome.
MedLine Citation:
PMID:  19653183     Owner:  NLM     Status:  MEDLINE    
Discrete subaortic stenosis is a rare, late complication of the surgical repair of congenital heart defects. Secondary subaortic stenosis late after surgical repair of supravalvular aortic stenosis in Williams syndrome has not previously been described. A 20 year-old female patient with Williams syndrome became symptomatic eight years after subaortic membrane resection. A discrete subaortic stenosis was identified by echocardiography. She was indicated for a Konno operation with a 19-mm Sorin SL valve inserted in the aortic position, along with ventricular septal defect closure with a pericardial patch. She produced a transient slight hypocalcemia after the operation. She was discharged home in a good condition on the fourth post-operative day. Intracardiac morphological and hemodynamic factors have been suggested as responsible for such a late complication. Our case study suggests the need for regular follow-up in patients with Williams syndrome, even after primary surgical repair.
Shi-Min Yuan; Rafael Kuperstein; Jacob Lavce
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Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  Cardiology journal     Volume:  16     ISSN:  1897-5593     ISO Abbreviation:  -     Publication Date:  2009  
Date Detail:
Created Date:  2009-08-04     Completed Date:  2009-10-21     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  101392712     Medline TA:  Cardiol J     Country:  Poland    
Other Details:
Languages:  eng     Pagination:  368-71     Citation Subset:  IM    
Departments of Cardiac and Thoracic Surgery, The Chaim Sheba Medical Center, Tel Hashomer, Israel.
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MeSH Terms
Aortic Stenosis, Supravalvular / etiology,  surgery*
Discrete Subaortic Stenosis / etiology*,  ultrasonography
Disease Progression
Postoperative Complications*
Williams Syndrome / complications*
Young Adult

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