HOME PRODUCTS SERVICES COMPANY CONTACT FAQ

Supravalvular Aortic Stenosis (Aortic Supravalvular Stenosis)

Subscribe to New Research on Supravalvular Aortic Stenosis

A pathological constriction occurring in the region above the AORTIC VALVE. It is characterized by restricted outflow from the LEFT VENTRICLE into the AORTA.

Also Known As:

Aortic Supravalvular Stenosis; Aortic Stenosis, Supravalvular; Aortic Supravalvular Stenoses; Stenoses, Aortic Supravalvular; Stenosis, Aortic Supravalvular; Stenosis, Supravalvular Aortic; Supravalvular Stenoses, Aortic; Supravalvular Stenosis, Aortic

Networked: 130 relevant articles (0 outcomes, 8 trials/studies)

Disease Context: Research Results

Cardiovascular Diseases: 37259
- Heart Diseases: 18762
  - Heart Valve Diseases: 960
    - Aortic Valve Stenosis: 2889
      - Supravalvular Aortic Stenosis: 130
        Williams Syndrome: 341
        eisenberg type Supravalvar aortic stenosis
  - Ventricular Outflow Obstruction: 94
    - Aortic Valve Stenosis: 2889
      - Supravalvular Aortic Stenosis: 130
        eisenberg type Supravalvar aortic stenosis

Related Diseases

1.	Williams Syndrome (Williams-Beuren Syndrome)
2.	Cutis Laxa
3.	Vascular Diseases (Vascular Disease)
4.	Atherosclerotic Plaque (Atheroma)
5.	Pathologic Constriction (Stenosis)

Experts

1.	Mecham, Robert P: 10 articles (12/2011 - 05/2003)
2.	Knutsen, Russell H: 7 articles (12/2011 - 11/2003)
3.	Faury, Gilles: 5 articles (01/2012 - 11/2003)
4.	Li, Dean Y: 5 articles (02/2008 - 11/2003)
5.	Urbán, Z: 4 articles (11/2001 - 06/2000)
6.	Pezet, Mylène: 3 articles (01/2012 - 11/2003)
7.	Wagenseil, Jessica E: 3 articles (12/2009 - 09/2005)
8.	Starcher, Barry: 3 articles (12/2009 - 11/2003)
9.	Urban, Zsolt: 3 articles (02/2007 - 11/2005)
10.	Jacob, Marie-Paule: 2 articles (08/2014 - 02/2008)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Supravalvular Aortic Stenosis:

1.	ElastinIBA 08/31/2007 - "The results of this study confirm that reestablishing normal elastin levels is a logical objective for treating diseases of elastin insufficiency such as supravalvular aortic stenosis. " 11/15/1998 - "Through a series of molecular genetic studies, we demonstrated that loss-of-function mutations in one elastin allele cause an inherited obstructive arterial disease, supravalvular aortic stenosis (SVAS). " 12/01/2012 - "Supravalvular aortic stenosis is a systemic elastin (ELN) arteriopathy that disproportionately affects the supravalvular aorta. " 12/01/2012 - "Supravalvular aortic stenosis: elastin arteriopathy." 10/02/2012 - "Supravalvular aortic stenosis (SVAS) is caused by mutations in the elastin (ELN) gene and is characterized by abnormal proliferation of vascular smooth muscle cells (SMCs) that can lead to narrowing or blockage of the ascending aorta and other arterial vessels. "
2.	Coarctation of the aortaIBA 06/01/1981 - "Two-dimensional echocardiographic studies were performed in 23 patients with coarctation of the aorta, 5 with interruption of the aortic arch, 6 with supravalvular aortic stenosis, 3 with truncus arteriosus, 2 with anomalous origin of the right pulmonary artery from the ascending aorta and 2 with aorto-pulmonary septal defect. " 08/01/2015 - "A 1-year-old boy with a bicuspid aortic valve, who had undergone successful repair of coarctation of the aorta by extended end-to-end direct anastomosis at the age of 1 month, was found to have mild supravalvular aortic stenosis involving the left coronary ostium. " 01/01/2013 - "Shone complex is a rare combination of valvular or supravalvular aortic stenosis, supra-valvular mitral membrane, parachute mitral valve, and coarctation of the aorta. " 11/01/1993 - "Supravalvular aortic stenosis is described along with coarctation of the aorta. " 01/01/1984 - "He had the unusual combination of supravalvular aortic stenosis, long segment coarctation of the aorta, aortic hypoplasia and a high bifurcation of the abdominal aorta. "
3.	ReninIBA 01/01/2002 - "Our study demonstrates that in rats with severe experimental supravalvular aortic stenosis only renal renin gene expression is stimulated. "
4.	LDL Receptors (LDL Receptor)IBA 08/11/1998 - "This study not only demonstrates the utility of MRI for detecting and characterizing aortic root atherosclerotic plaque and supravalvular aortic stenosis in HFH patients but also suggests that the LDL receptor plays a direct or indirect role in aortic mural development and vascular growth."
5.	SASIBA 08/01/2009 - "Discrete supravalvular aortic stenosis (SAS) is known to involve the whole aortic root. " 07/01/2005 - "Steinberg method is a modification of Doty extended aortoplasty for supravalvular aortic stenosis (SAS). " 11/01/2004 - "We experienced a successful surgical case of extended aortoplasty by means of Doty's method (two sinus reconstruction) for congenital supravalvular aortic stenosis (SAS). " 11/01/2003 - "Stenotic lesion of the left coronary artery is an unnoticed but complicating feature of supravalvular aortic stenosis (SAS). " 01/01/2003 - "The authors present their experience in surgical treatment of children with supravalvular aortic stenosis (SAS). "
6.	fibrillinIBA 12/01/2009 - "Even though elastin and fibrillin-1 are the major structural components of elastic fibers, mutations in elastin and fibrillin-1 lead to narrowing of large arteries in supravalvular aortic stenosis and dilation of the ascending aorta in Marfan syndrome, respectively. " 01/01/1994 - "The relation between Marfan syndrome and fibrillin mutations and that between supravalvular aortic stenosis and William syndromes and elastin mutations are reviewed, as is the presence of microdeletions in 22q11 in DiGeorge syndrome, velocardiofacial syndrome, and nonsyndromic patients with conotruncal malformations. " 01/01/2012 - "An alteration of the elastin (Eln) or fibrillin (Fbn) gene products leads to severe genetic pathologies of the cardiovascular system, such as supravalvular aortic stenosis, or Williams Beuren syndrome--in which elastin deficiency induces aortic stenoses--or Marfan syndrome, in which on the contrary fibrillin-1 deficiency promotes the appearance of aortic aneurysms. " 01/01/2013 - "Severe heritable elastic fibre diseases are caused by mutations in elastic fibre components; for example, mutations in elastin cause supravalvular aortic stenosis and autosomal dominant cutis laxa, mutations in fibrillin-1 cause Marfan syndrome and Weill-Marchesani syndrome, and mutations in fibulins-4 and -5 cause autosomal recessive cutis laxa. " 11/19/1999 - "Both morphometric parameters analyzed by using automated image analysis and immunofluorescence labeling with monoclonal antibodies against elastin and fibrillin 1 showed a disorganized pre-elastic (oxytalan and elaunin) and mature elastic fibers in the dermis of 10 Williams syndrome patients compared with five healthy children and one patient with isolated supravalvular aortic stenosis. "
7.	Vitamin D FDA LinkGeneric 01/01/1969 - "The relationship between vitamin D and the craniofacial and dental anomalies of the supravalvular aortic stenosis syndrome." 10/01/1967 - "[On the relation of Vitamin D, administered to gravidas and children, and an abnormality syndrome manifested by supravalvular aortic stenosis]." 05/01/1967 - "Vitamin D as a cause of the supravalvular aortic stenosis syndrome." 10/01/1966 - "Congenital supravalvular aortic stenosis, idiopathic hypercalcemia, and vitamin D."
8.	CholesterolIBA 01/01/2007 - "The main complication of HMZ FH is supravalvular aortic stenosis, which is caused by cholesterol deposits on the aortic valve and in the ascending aorta. " 04/01/1983 - "The degree of supravalvular aortic stenosis correlated with the serum cholesterol level but not with patient age. "
9.	Calcitonin (Calcitonin, Eel)FDA LinkGeneric 05/01/1988 - "Exclusion of calcitonin as a candidate gene for the basic defect in a family with autosomal dominant supravalvular aortic stenosis." 01/01/1992 - "Exclusion of calcitonin/alpha-CGRP gene defect in a family with autosomal dominant supravalvular aortic stenosis."
10.	Sirolimus (Rapamycin)FDA Link 05/01/2013 - "In cell cultures, rapamycin inhibited prolonged mTOR activation and enhanced proliferation of SMC derived from patients with supravalvular aortic stenosis and with Williams syndrome. "

Therapies and Procedures

1.	Plasmapheresis 08/01/1978 - "In one (Case 1) plasmapheresis was performed at fortnightly intervals over a period of 18 months; in the other (Case 2) the necessity for surgical relief of an associated supravalvular aortic stenosis resulted in premature termination of the trial. "
2.	Transplants (Transplant) 04/01/1996 - "In this case of severe diffuse supravalvular aortic stenosis in a child, we used the combination of an apicoaortic conduit followed 6 years later by aortic valve replacement, replacement of the ascending aorta and aortic arch, and an ascending to thoracic descending aorta bypass graft." 01/01/1994 - "(3) In diffuse supravalvular aortic stenosis, aortic enlargement should be extended into the ascending aorta or beyond as required to relieve the gradient; some patients may require a graft or conduit.(ABSTRACT TRUNCATED AT 400 WORDS)" 01/01/1981 - "Surgical repair of the diffuse form of supravalvular aortic stenosis, (tubular hypoplasia of ascending aorta) is a difficult problem because complete relief of the pressure gradient is not achieved by the insertion of a prosthetic graft across the non coronary sinus of Valsalva. " 01/01/2007 - "Modified Myers and coronary artery bypass grafting using free internal thoracic artery graft for complicated supravalvular aortic stenosis." 12/01/1982 - "Surgical treatment of severe discrete supravalvular aortic stenosis by placement of a patch graft across the narrowed area of aorta at one point has been associated with a substantial incidence of residual stenosis. "
3.	Angioplasty (Angioplasty, Transluminal) 10/01/2010 - "Twelve years have passed since a patient with William's syndrome underwent a surgery with a patch angioplasty for the diffuse supravalvular aortic stenosis and deformities of the neck branch arteries. " 04/01/1999 - "Surgical angioplasty of left main coronary stenosis complicating supravalvular aortic stenosis." 12/01/1991 - "In a 9-year-old girl with supravalvular aortic stenosis combined with severe left main coronary artery trunk obstruction (Williams syndrome), the repair for both lesions was performed successfully by simultaneous extended aortoplasty and direct coronary angioplasty using a single double-flanged pericardial patch." 10/01/1982 - "[Secondary mediastinitis and septicemia following patch-angioplasty of supravalvular aortic stenosis: a case report]."
4.	Stents 04/01/2004 - "This report shows the findings observed in a 7-year-old boy patient having severe supravalvular aortic stenosis who was treated by stent implantation. " 04/01/2004 - "Acute aortic insufficiency complicating stent treatment of supravalvular aortic stenosis: successful release of trapped leaflets by wiring the stent." 11/01/1996 - "Tailored stent treatment for severe supravalvular aortic stenosis." 01/01/2016 - "Percutaneous treatment of supravalvular aortic stenosis (SVAS) by means of balloon dilation and stent deployment has been rarely reported in the literature. " 01/01/2016 - "Disseminated Peripheral Mycotic Aneurysms and Septic Embolizations Related to an Infected Stent Deployed for Restenosis of Surgically Repaired Supravalvular Aortic Stenosis."
5.	Coronary Artery Bypass (Coronary Artery Bypass Surgery) 07/01/1983 - "Aortic aspergilloma resulting in supravalvular aortic stenosis after aortocoronary bypass surgery." 01/01/2007 - "Modified Myers and coronary artery bypass grafting using free internal thoracic artery graft for complicated supravalvular aortic stenosis."