title: Supravalvular aortic stenosis associated to infectious endocarditis and cerebral vascular disease in a patient with Williams-Beuren Syndrome.
abstract: The Williams-Beuren syndrome is a rare genetic disease characterized by: (a) typical facial features; (b) psychomotor retardation with a specific neurocognitive profile; (c) cardiovascular condition and (d) likely transient hypocalcemia in infancy. The objective of this study was to describe the clinic evolution and diagnosis of patient with this syndrome that was associated with endocarditis caused by Streptococcus parasanguis in the ascending aorta and an aneurism located in the fronto-temporal area, which produced a parenchymal hematoma in the left lobe, and subarachnoid hemorrhage. He was treated with ceftriaxone and dicloxacillin. Then we proceeded to correct the aneurysm and perform vegetation resection in aortic arteries with supravalvular aortic stenosis correction. The evolution after one year has been favorable and is currently without neurologic sequelae. A 5-year-old male patient presented a diagnosis of supravalvular aortic stenosis. After cardiac catheterization was performed, he presented a fever and right side paresis. The echocardiogram showed multiple vegetations in the ascendant aortic arch and the supraortic arteries. The blood cultures reported S. parasanguis. The magnetic resonance showed a subarachnoid hemorrhage with an aneurysm and a hematoma.
authors.affiliation: Instituto Nacional de Pediatría, México, D.F., Mexico. Electronic address: derubens@hotmail.com.
authors.forename: Jesús
authors.initials: J
authors.lastname: De Rubens Figueroa
authors.affiliation: Instituto Nacional de Pediatría, México, D.F., Mexico.
authors.forename: Alfonso
authors.initials: A
authors.lastname: Marhx
authors.affiliation: Instituto Nacional de Pediatría, México, D.F., Mexico.
authors.forename: Javier
authors.initials: J
authors.lastname: López Terrazas
authors.affiliation: Instituto Nacional de Pediatría, México, D.F., Mexico.
authors.forename: Alexis
authors.initials: A
authors.lastname: Palacios Macedo
mesh_terms: D021921:Aortic Stenosis, Supravalvular / Q000209:etiology*; D002561:Cerebrovascular Disorders / Q000150:complications*; D002675:Child, Preschool; D004697:Endocarditis, Bacterial / Q000150:complications*; D006801:Humans; D008297:Male; D013290:Streptococcal Infections / Q000150:complications*; D018980:Williams Syndrome / Q000150:complications*
keywords: Endocarditis infecciosa; Estenosis supravalvular aórtica; Infective endocarditis; Mexico; México; Supravalvular aortic stenosis; Síndrome de Williams–Beuren; Williams–Beuren syndrome