Supravalvular Aortic Stenosis (Aortic Supravalvular Stenosis)

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102 relevant articles (0 outcomes, 8 trials/studies) found for this Disease

Description: 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

Disease Context: Research Results

Cardiovascular Diseases: 18509

Heart Diseases: 12346

Heart Valve Diseases: 641

Aortic Valve Stenosis: 1625

Supravalvular Aortic Stenosis: 102

Williams Syndrome: 185

Ventricular Outflow Obstruction: 74

Aortic Valve Stenosis: 1625

Supravalvular Aortic Stenosis: 102

Related Diseases

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

Experts

1.	Urbán, Z: 3 articles (11/2001 - 06/2000)
2.	Davis, E C: 2 articles (11/2001 - 06/2000)
3.	Boyd, C D: 2 articles (11/2001 - 06/2000)
4.	Ishibashi, Nobuyuki: 1 article (01/2007)
5.	Isomatsu, Yukihisa: 1 article (01/2007)
6.	Aoki, Mitsuru: 1 article (01/2007)
7.	Watanabe, Manabu: 1 article (01/2007)
8.	Fujiwara, Tadashi: 1 article (01/2007)
9.	Kim, Youngho: 1 article (08/2006)
10.	Yoo, Han-Wook: 1 article (08/2006)

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)" 11/01/2008 - "Elastin loss-of-function mutations in humans have been linked to the autosomal-dominant disease supravalvular aortic stenosis, which is characterized by stenotic lesions in both the systemic and pulmonary circulations" 10/01/2007 - "RESULTS: Forty out of 71 patients were detected to have Elastin gene locus microdeletion, 25 patients (25/40, 62.5%) had at least one cardiac anomaly; among these patients, supravalvular aortic stenosis (SVAS) was diagnosed in 18 patients (18/25, 72%) and 6 of them had complex abnormalities" 07/01/2007 - "Some of these properties are also observed in humans with supravalvular aortic stenosis, a disease caused by functional heterozygosity of the elastin gene" Order ALL the reference details at left...
2.	SASIBA 11/01/1997 - "BACKGROUND AND AIM OF THE STUDY: Supravalvular aortic stenosis (SAS) has been surgically treated by either single-sinus or bisinus patch repair" 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)" Order ALL the reference details at left...
3.	ReninIBA 01/01/2002 - "CONCLUSION: Our study demonstrates that in rats with severe experimental supravalvular aortic stenosis only renal renin gene expression is stimulated" Order ALL the reference details at left...
4.	LDL Receptors (LDL Receptor)IBA 08/11/1998 - "CONCLUSIONS: 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." Order ALL the reference details at left...
5.	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." Order ALL the reference details at left...
6.	DiamondIBA 05/01/1990 - "A traditional diamond shaped patch aortoplasty was used to relieve supravalvular aortic stenosis in 1 case and an extended aortoplasty was used in last 4 cases" 09/01/1977 - "Enlargement of the aorta with a diamond-shaped patch of the noncoronary sinus of Valsalva may not be sufficient in severe cases of supravalvular aortic stenosis" Order ALL the reference details at left...
7.	CalciumIBA 05/01/1975 - "Only 32% of the patients have evidence of supravalvular aortic stenosis and not one of them has had documented hypercalcemia, including eight patients who had a serum calcium determination in the first year of life" 06/01/1976 - "Supravalvular aortic stenosis-infantile hypercalcaemia syndrome: in vitro hypersensitivity to vitamin D2 and calcium." Order ALL the reference details at left...
8.	Calcitonin (Calcitonin, Eel)FDA Link 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." Order ALL the reference details at left...
9.	Triad FDA Link 01/01/1975 - "The diagnosis was based upon the triad of symptoms: 1) supravalvular aortic stenosis, 2) typical appearance of the patient, 3) considerable mental disability." 03/01/2005 - "Our results indicate that the triad of symptoms consisting of infantile hypercalcemia, dysmorphic facies, and supravalvular aortic stenosis, which until recently was considered fundamental for Williams syndrome diagnosis, is not usually present and does not lead to an early diagnosis" Order ALL the reference details at left...
10.	fibrillinIBA 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" 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" Order ALL the reference details at left...

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" Order ALL the reference details at left...
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" Order ALL the reference details at left...
3.	Angioplasty (Angioplasties) 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]" Order ALL the reference details at left...
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." Order ALL the reference details at left...
5.	Balloon Dilatation 09/01/1994 - "Balloon dilatation was performed in two cases of supravalvular aortic stenosis with variable results" 09/01/1994 - "Balloon dilatation of supravalvular aortic stenosis: a report of two cases." 11/01/1989 - "Percutaneous transluminal balloon dilatation in supravalvular aortic stenosis." Order ALL the reference details at left...

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