title: Severe coronary artery disease in the absence of supravalvular stenosis in a patient with Williams syndrome.
abstract: Williams syndrome is a complex syndrome comprising developmental abnormalities, craniofacial dysmorphic features, and cardiac anomalies. The most common cardiac anomaly is supravalvular aortic stenosis. We report a case of a 6-year-old girl with Williams syndrome who presented with decompensated heart failure due to ischemic cardiomyopathy. Her only significant cardiac anomaly was severe stenosis of the left main coronary artery. She subsequently died despite surgical revascularization. Isolated coronary anomalies are rare in Williams syndrome but should be considered especially in the presence of heart failure or ischemia.
authors.affiliation: Pediatric Cardiology Department, Green Lane Hospital, Private Bag 92024, Auckland 1003, New Zealand. vanpelt@ihug.co.nz
authors.forename: N C
authors.initials: NC
authors.lastname: van Pelt
authors.forename: N J
authors.initials: NJ
authors.lastname: Wilson
authors.forename: G
authors.initials: G
authors.lastname: Lear
mesh_terms: D021921:Aortic Stenosis, Supravalvular / Q000175:diagnosis; D002648:Child; D001026:Coronary Artery Bypass; D023921:Coronary Stenosis / Q000150:complications* / Q000175:diagnosis* / Q000503:physiopathology / Q000601:surgery; D003330:Coronary Vessel Anomalies / Q000503:physiopathology / Q000601:surgery; D004452:Echocardiography; D004562:Electrocardiography; D005260:Female; D006333:Heart Failure / Q000209:etiology / Q000503:physiopathology / Q000601:surgery; D006801:Humans; D012720:Severity of Illness Index; D018487:Ventricular Dysfunction, Left / Q000503:physiopathology / Q000601:surgery; D018980:Williams Syndrome / Q000150:complications* / Q000175:diagnosis* / Q000503:physiopathology / Q000601:surgery