Abstract

RUBENS FIGUEROA, Jesús De; MARHX, Alfonso; LOPEZ TERRAZAS, Javier  and  PALACIOS MACEDO, Alexis. Supravalvular aortic stenosis associated to infectious endocarditis and cerebral vascular disease in a patient with Williams-Beuren Syndrome. Arch. Cardiol. Méx. [online]. 2015, vol.85, n.4, pp.292-295. ISSN 1665-1731.  https://doi.org/10.1016/j.acmx.2014.12.002.

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.

Keywords : Williams-Beuren syndrome; Infective endocarditis; Supravalvular aortic stenosis; Mexico.

        · abstract in Spanish     · text in English     · English ( pdf )