Abstract
Supravalvular aortic stenosis (sup AS) is the least common type of congenital left ventricular outflow tract (LVOT) obstruction. In the most common type of sup AS, there is a fixed aortic narrowing originating from just above the sinus of Valsalva which extends along a variable length of the aorta (hourglass type); the membranous type is the result of a fibrous or fibromuscular semicircular diaphragm with a small central opening in the lumen of the aorta, and diffuse hypoplasia of the ascending aorta illustrates the third type [1].
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References
Aboulhosn J, Child JS. Left ventricular outflow obstruction: subaortic stenosis, bicuspid aortic valve, supravalvar aortic stenosis, and coarctation of the aorta. Circulation. 2006;114:2412–22.
Micale L, Turturo MG, Fusco C, et al. Identification and characterization of seven novel mutations of elastin gene in a cohort of patients affected by supravalvular aortic stenosis. Eur J Hum Genet. 2010;18:317–23.
Warnes CA, Williams RG, Bashore TM, et al. ACC/AHA 2008 guidelines for the management of adults with congenital heart disease: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Writing Committee to Develop Guidelines on the Management of Adults With Congenital Heart Disease). J Am Coll Cardiol. 2008;52(23):e143–263.
Pober BR. Williams–Beuren syndrome. N Engl J Med. 2010;362:239–52.
Bonow RO, Carabello BA, Chatterjee K, et al. ACC/AHA 2006 guidelines for the management of patients with valvular heart disease: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Writing Committee to Revise the 1998 Guidelines for the Management of Patients With Valvular Heart Disease) developed in collaboration with the Society of Cardiovascular Anesthesiologists, endorsed by the Society for Cardiovascular Angiography and Interventions and the Society of Thoracic Surgeons. J Am Coll Cardiol. 2006;48:e1–148.
Otto CM, editor. Textbook of clinical echocardiography. 4th ed. Philadelphia: Saunders/Elsevier; 2009.
Rosenhek R, Baumgartner H. Aortic stenosis. In: Otto CM, Bonow RO, editors. Valvular heart disease: a companion to Braunwald’s heart disease. Philadelphia: Saunders/Elsevier; 2009. p. 127–54.
Vahanian A, Baumgartner H, Bax J, et al. Guidelines on the management of valvular heart disease: the Task Force on the Management of Valvular Heart Disease of the European Society of Cardiology. Eur Heart J. 2007;28:230.
Mc Elhinney DB, Petrossian E, Tworetzky W, et al. Issues and outcomes in the management of supravalvar aortic stenosis. Ann Thorac Surg. 2000;69(2):562–7.
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Color flow Doppler study is helpful for identifying the level of the stenosis. In the supra aortic stenois turbulent flow originate above the AV (MPG 2576 kb)
Transthoracic 2D and color Doppler study in apical 5 chamber view showing a discrete membrane above the Aortic valve (AV) that turbulent flow begins above the AV (MPG 2674 kb)
Transthoracic 2D and color Doppler study in apical 5 chamber view showing a discrete membrane above the Aortic valve (AV) that turbulent flow begins above the AV (MPG 2674 kb)
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Sadeghpour, A., Alizadehasl, A. (2014). Supravalvular Aortic Stenosis. In: Sadeghpour, A., Kyavar, M., Alizadehasl, A. (eds) Comprehensive Approach to Adult Congenital Heart Disease. Springer, London. https://doi.org/10.1007/978-1-4471-6383-1_37
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DOI: https://doi.org/10.1007/978-1-4471-6383-1_37
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