| Aortic root replacement for annuloaortic ectasia in Shprintzen-Goldberg syndrome: a case report. | |
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MedLine Citation:
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PMID: 9130129 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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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. |
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Authors:
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S Fukunaga; H Akashi; K Tayama; H Kawano; K Kosuga; S Aoyagi |
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Publication Detail:
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Type: Case Reports; Journal Article; Review |
Journal Detail:
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Title: The Journal of heart valve disease Volume: 6 ISSN: 0966-8519 ISO Abbreviation: J. Heart Valve Dis. Publication Date: 1997 Mar |
Date Detail:
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Created Date: 1997-06-27 Completed Date: 1997-06-27 Revised Date: 2006-05-04 |
Medline Journal Info:
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Nlm Unique ID: 9312096 Medline TA: J Heart Valve Dis Country: ENGLAND |
Other Details:
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Languages: eng Pagination: 181-3 Citation Subset: IM |
Affiliation:
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Second Department of Surgery, Kurume University School of Medicine, Japan. |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Abnormalities, Multiple*
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diagnosis,
physiopathology Aorta / surgery Aortic Valve / abnormalities*, surgery Aortic Valve Insufficiency / complications, diagnosis, surgery* Cardiac Surgical Procedures / methods* Child Disease-Free Survival Echocardiography, Doppler Humans Male Marfan Syndrome* / diagnosis |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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