Document Detail

Aortic root replacement for annuloaortic ectasia in Shprintzen-Goldberg syndrome: a case report.
MedLine Citation:
PMID:  9130129     Owner:  NLM     Status:  MEDLINE    
Annuloaortic ectasia due to Shprintzen-Goldberg syndrome (SGS) is reported. A 10-year-old boy was admitted to our hospital for evaluation of chest pain. On admission, he was diagnosed as SGS on the basis of his various anomalies. Two-dimensional echocardiography showed a bicuspid aortic valve and marked annular dilatation, Doppler flow studies revealed severe aortic regurgitation, and retrograde aortography showed severe aortic regurgitation with annular dilatation. Successful aortic root replacement was performed; subsequent histologic examination of the ascending aorta demonstrated cystic medial necrosis. In conclusion, SGS is a generalized connective tissue dysplasia, with clinical manifestations of cardiovascular lesions similar to those in Marfan syndrome. Aortic root replacement was successfully performed; however, recurrence of aortic aneurysms outside of the ascending aorta should be carefully observed. Surgical treatment for cardiovascular disorders may be necessary to save the life of patients with SGS.
S Fukunaga; H Akashi; K Tayama; H Kawano; K Kosuga; S Aoyagi
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Publication Detail:
Type:  Case Reports; Journal Article; Review    
Journal Detail:
Title:  The Journal of heart valve disease     Volume:  6     ISSN:  0966-8519     ISO Abbreviation:  J. Heart Valve Dis.     Publication Date:  1997 Mar 
Date Detail:
Created Date:  1997-06-27     Completed Date:  1997-06-27     Revised Date:  2006-05-04    
Medline Journal Info:
Nlm Unique ID:  9312096     Medline TA:  J Heart Valve Dis     Country:  ENGLAND    
Other Details:
Languages:  eng     Pagination:  181-3     Citation Subset:  IM    
Second Department of Surgery, Kurume University School of Medicine, Japan.
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MeSH Terms
Abnormalities, Multiple* / diagnosis,  physiopathology
Aorta / surgery
Aortic Valve / abnormalities*,  surgery
Aortic Valve Insufficiency / complications,  diagnosis,  surgery*
Cardiac Surgical Procedures / methods*
Disease-Free Survival
Echocardiography, Doppler
Marfan Syndrome* / diagnosis

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

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