title: Fatal severe coronary artery stenosis in Williams syndrome: decision making using late gadolinium enhancement cardiovascular MRI.
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.
authors.affiliation: 1Royal Brompton and Harefield NHS Foundation Trust,Sydney Street,London,United Kingdom.
authors.forename: Inga
authors.initials: I
authors.lastname: Voges
authors.affiliation: 1Royal Brompton and Harefield NHS Foundation Trust,Sydney Street,London,United Kingdom.
authors.forename: Rodney C
authors.initials: RC
authors.lastname: Franklin
authors.affiliation: 1Royal Brompton and Harefield NHS Foundation Trust,Sydney Street,London,United Kingdom.
authors.forename: Ricardo
authors.initials: R
authors.lastname: Wage
authors.affiliation: 1Royal Brompton and Harefield NHS Foundation Trust,Sydney Street,London,United Kingdom.
authors.forename: Sonya V
authors.initials: SV
authors.lastname: Babu-Narayan
mesh_terms: D021921:Aortic Stenosis, Supravalvular / Q000000981:diagnostic imaging* / Q000503:physiopathology*; D023921:Coronary Stenosis / Q000000981:diagnostic imaging* / Q000503:physiopathology*; D003657:Decision Making; D017809:Fatal Outcome; D005260:Female; D005471:Fluoroscopy; D005682:Gadolinium; D006801:Humans; D007223:Infant; D008279:Magnetic Resonance Imaging; D010166:Palliative Care; D018980:Williams Syndrome / Q000150:complications*
chemical_list: D005682:Gadolinium
keywords: Williams syndrome; cardiovascular MRI; coronary artery abnormalities; supravalvar aortic stenosis