H-repair in supravalvular aortic stenosis [PDF]
Christoph Haller+4 more
europepmc +5 more sources
We report an extremely rare case of Williams–Beuren (WB) syndrome in a 10-year-old boy with supravalvular aortic stenosis (SVAS) secondary to a large fibrocalcific eccentric shelf in the ascending aorta with fibrocalcific severe supravalvular pulmonary ...
Debasish Das+5 more
doaj +2 more sources
Supravalvular aortic stenosis repair: surgical training of 2 repair techniques using 3D-printed models. [PDF]
There are a number of techniques used in the surgical treatment of supravalvular aortic stenosis with excellent results; however, junior surgeons' experience may be limited due to its rare nature.
Hussein N, Honjo O, Barron DJ, Yoo SJ.
europepmc +2 more sources
Management of supravalvular aortic stenosis with the Doty technique, in a South American pediatric center. [PDF]
Supravalvular aortic stenosis (SVAS) is a congenital malformation associated with Williams-Beuren syndrome. It is characterized by severe stenosis of the sinotubular junction. The smooth muscle layer is thickened due to an alteration of the Elastin gene.
Yagual-Gutiérrez EB+5 more
europepmc +2 more sources
Anesthetic considerations in supravalvular aortic stenosis: A case series
Supravalvular aortic stenosis (SVAS), characterized by elastin arteriopathy, presents either as a localized narrowing at the sinotubular junction or as a diffuse form with additional involvement of the ascending aorta, aortic arch, and its branches ...
Virendra Kumar +3 more
doaj +2 more sources
Letter regarding "Aortopulmonary fistula in a Warmblood mare associated with an aortic aneurysm and supravalvular aortic stenosis". [PDF]
We read with interest the case report “ Aortopulmonary fistula in a Warmblood mare associated with an aortic aneurysm and supravalvular aortic stenosis ” .
Guglielmini C+3 more
europepmc +2 more sources
Supravalvular aortic stenosis in a twin. [PDF]
A case of subvalvular aortic stenosis in one of a set of dissimilar twins is reported. The case is discussed in terms of the aetiological factors involved and supports the view that supravalvular aortic stenosis is not environmental and may be genetic in origin.
K.F. Hossack, G Neilson
openaire +4 more sources
Identification and characterization of novel elastin gene mutations in eleven families with supravalvular aortic stenosis [PDF]
Background: Supravalvular aortic stenosis (SVAS) is a rare congenital heart disease affecting approximately 1 in 25,000 live births. In some patients it is accompanied by pulmonary artery stenosis, particularly of pulmonary artery branches.
Jianrong Zhou+13 more
doaj +2 more sources
Supravalvular aortic stenosis in William′s syndrome
We report supravalvular aortic stenosis in a 12 year old patient who presented with mental retardation, malformed teeth, broad lower lips, pectus carinatum, clinodactyly, kyphoscoliosis with symptoms of shortness of breath. On examination presence of better volume pulse in right radial artery with ejection systolic murmur best heard in right 2(nd ...
Chander Mohan Mittal, Bishav Mohan
openaire +5 more sources
Familial hypercholesterolemia supravalvular aortic stenosis and extensive atherosclerosis [PDF]
Familial hypercholesterolemia is an autosomally dominant disorder caused by various mutations in low-density lipoprotein receptor genes. This can lead to premature coronary atherosclerosis and cardiac-related death.
Rajpal Prajapati, Vikas Agrawal
doaj +2 more sources