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Supravalvular aortic stenosis in the adult : a case presentation with unique associated features

dc.contributor.authorPrzybojewski, J. Z.
dc.contributor.authorStewart, R. I.
dc.contributor.authorDe Wet Lubbe, J. J.
dc.date.accessioned2011-03-18T14:59:48Z
dc.date.available2011-03-18T14:59:48Z
dc.date.issued1981-05
dc.identifier.citationPrzybojewski, J. Z., Stewart, R. I., De Wet Lubbe, J. J. 1981. Supravalvular aortic stenosis in the adult : a case presentation with unique associated features. SA medical journal, 23 May: 796-803.
dc.identifier.issn2078-5135 (online) 0256-9574 (print)
dc.identifier.urihttp://hdl.handle.net/10019.1/8064
dc.descriptionThe original publication is available at http://www.samj.org.za
dc.description.abstractA 42-year-old symptomatic woman with possible adult Williams's syndrome (mental retardation, 'elfin facies' and supravalvular aortic steosis (SVAS)) is documented. This patient displayed many unique features in addition to the severe SVAS (peak systolic gradient 96 mmHg): there was an associated persistent left superior vena cava draining into the coronary sinus, mitral valve prolapse (Barlow's syndrome) and complete right bundle-branch block on ECG. Peripheral pulmonary artery stenosis was absent. M-mode echocardiography in the adult with SVAS is described for the first time in the literature, as is the use of the 60° cross-sectional scan. Both these non-invasive procedures proved of value in the diagnosis. The degree of subendocardial ischaemia, as determined by the 'endocardial viability ratio', was calculated and the possible mechanisms producing angina pectoris with 'coronary artery hypertension' in this condition are discussed. The literature on associated mitral valve abnormalities in SVAS is reviewed. A successful surgical result was obtained; the various forms of operation are outlined.en_ZA
dc.language.isoen
dc.publisherHealth and Medical Publishing Group -- HMPG
dc.subjectAortic valve -- Stenosisen_ZA
dc.titleSupravalvular aortic stenosis in the adult : a case presentation with unique associated featuresen_ZA
dc.typeArticle
dc.description.versionPublishers' version
dc.rights.holderThe authors


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