Linked Life Data

20662229

Source:http://linkedlifedata.com/resource/pubmed/id/20662229

Statements in which the resource exists as a subject.
PredicateObject
rdf:type
lifeskim:mentions
pubmed:issue
7
pubmed:dateCreated
2010-7-28
pubmed:abstractText
A 17-year-old female patient complaining of chest pain was transferred to our hospital to treat annuloaortic ectasia associated with severe aortic valve insufficiency. She underwent a valve sparing operation with graft replacement of the ascending aorta and the proximal portion of the aortic arch. After the 1st operation, she was diagnosed with Loeys-Dietz syndrome (LDS), which is recently described as an autosomal dominant aortic aneurysm syndrome caused by heterozygous mutations in the transforming growth factor-beta receptor type 1 and 2 genes. Only 2 months after the 1st operation, she developed heart failure due to recurrence of aortic regurgitation. Computed tomography (CT) scan showed an expansion of the aortic arch. Total arch replacement using the elephant trunk technique and aortic valve replacement were performed successfully 4 months after the 1st operation. The rapidly progressive nature of the aortic disease in patients with LDS underscores the importance of meticulous surveillance of the entire aorta and the need for early surgical management.
pubmed:language
jpn
pubmed:journal
pubmed:citationSubset
IM
pubmed:status
MEDLINE
pubmed:month
Jul
pubmed:issn
0021-5252
pubmed:author
pubmed:issnType
Print
pubmed:volume
63
pubmed:owner
NLM
pubmed:authorsComplete
Y
pubmed:pagination
527-30
pubmed:meshHeading
pubmed:year
2010
pubmed:articleTitle
[Marfan-like aortic aneurysm syndrome; A surgical experience with Loeys-Dietz syndrome].
pubmed:affiliation
Department of Cardiovascular Surgery, Kyoto 2nd Red Cross Hospital, Kyoto, Japan.
pubmed:publicationType
Journal Article, English Abstract, Case Reports