Statements in which the resource exists as a subject.
PredicateObject
rdf:type
lifeskim:mentions
pubmed:issue
5
pubmed:dateCreated
2007-7-24
pubmed:abstractText
The Williams-Beuren syndrome is the association of elf-like facies, mental retardation with cardiovascular anomalies, the most common of which is supravalvular aortic stenosis. This lesion may be focal or associated with hypoplasia of the distal aorta. The treatment is surgical and the role of interventional cardiological treatment is poorly defined. The authors report the case of a child with typical Williams-Beuren syndrome. An initial, very localised surgical aortic repair was performed at 3 months of age for a discrete supravalvular aortic stenosis. Two months later, a second operation was required for a new stenosis of the distal anastomosis associated with marked hypoplasia of the aortic arch. The progressive constitution of an isthmic coarctation led to the percutaneous implantation of a stent followed by two balloon dilatations. Only the first two endoluminal procedures successfully reduced the transisthmic pressure gradient. An antihypertensive treatment was given and regular echocardiography allows monitoring of the adaptation of the left ventricle.
pubmed:language
fre
pubmed:journal
pubmed:citationSubset
IM
pubmed:chemical
pubmed:status
MEDLINE
pubmed:month
May
pubmed:issn
0003-9683
pubmed:author
pubmed:issnType
Print
pubmed:volume
100
pubmed:owner
NLM
pubmed:authorsComplete
Y
pubmed:pagination
466-9
pubmed:dateRevised
2009-2-13
pubmed:meshHeading
pubmed:year
2007
pubmed:articleTitle
[The Williams-Beuren syndrome: reconstruction of the thoracic aorta combining surgery and endovascular treatment].
pubmed:affiliation
Cardiologie pédiatrique, Hôpital des Enfants, Toulouse.
pubmed:publicationType
Journal Article, English Abstract, Case Reports