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Fatal severe coronary artery stenosis in Williams syndrome: decision making using late gadolinium enhancement cardiovascular MRI

Published online by Cambridge University Press:  13 March 2017

Inga Voges ,
Rodney C. Franklin ,
Ricardo Wage  and
Sonya V. Babu-Narayan
Inga Voges*
Affiliation:
Royal Brompton and Harefield NHS Foundation Trust, Sydney Street, London, United Kingdom
Rodney C. Franklin
Affiliation:
Royal Brompton and Harefield NHS Foundation Trust, Sydney Street, London, United Kingdom
Ricardo Wage
Affiliation:
Royal Brompton and Harefield NHS Foundation Trust, Sydney Street, London, United Kingdom
Sonya V. Babu-Narayan
Affiliation:
Royal Brompton and Harefield NHS Foundation Trust, Sydney Street, London, United Kingdom NIHR Cardiovascular Biomedical Research Unit, Royal Brompton and Harefield NHS Foundation Trust, National Heart and Lung Institute, Imperial College, London, United Kingdom
*
Correspondence to: I. Voges, Royal Brompton and Harefield NHS Foundation Trust, Sydney Street, London SW15 2DF, United Kingdom. Tel: +00 49 (0)78 8171 0045; Fax: 0207 351 8816; E-mail: i.voges2@rbht.nhs.uk
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Abstract

Williams syndrome is a well-recognised congenital disorder characterised by cardiovascular, connective tissue, and central nervous system abnormalities. Coronary artery abnormalities are seen in patients with supravalvar aortic stenosis, but end-stage ischaemic heart disease is rare. We report a case of end-stage ischaemic heart disease due to severe coronary arterial stenosis, highlighting how cardiovascular MRI contributed to the management.

Keywords

Williams syndromecoronary artery abnormalitiescardiovascular MRIsupravalvar aortic stenosis
Type
Brief Report
Information
Cardiology in the Young , Volume 27 , Issue 7 , September 2017 , pp. 1398 - 1401
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Copyright
© Cambridge University Press 2017 

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