Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0014118 (endocarditis)
 15,629 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Over the last 22 years, two children were operated on for supraventricular aortic stenosis at the Institute of Cardiovascular Diseases in Bratislava. Both cases presented a localized form of supravalvular aortic stenosis. Simple elipsoid flaps were used without extended aortoplasty. One of the two children, a 12-year-old boy with Williams' syndrome died of endocarditis in the early postoperative period. In the 10-year-old girl with familial supravalvular aortic stenosis the operation was successful, although the defect was combined with supravalvular muscular obstruction. The authors emphasize the possibility of choice between two surgical procedures according to the localization of the stenosis with respect to the valvular apparatus.
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PMID:[Surgical treatment of supravalvular aortic stenosis]. 139 37

A 17-year-old man with supravalvular aortic stenosis associated with Williams syndrome was admitted to our hospital for intensive treatment for intractable infective endocarditis. The patient had a history of percutaneous balloon valvuloplasty for aortic stenosis in 1992. He was well until late in 1999, when he had a high temperature after dental work-up. The diagnosis was infective endocarditis but antibiotic therapy was not effective. He was transferred to our clinic. Transthoracic echocardiography demonstrated bicuspid aortic valve, supraaortic stenosis, mitral valve prolapse with severe regurgitation and scattered vegetations on the anterior mitral and aortic valves. In addition, transesophageal echocardiography showed innumerable mobile vegetations located from Valsalva's sinus to the descending aorta. Aortic root and arch replacement with a homograft and mitral valve replacement with an artificial valve were successfully performed to eliminate the infective endocarditis. In the present patient, the flow jet across the supraaortic stenosis seemed to cause a predisposition to severe endocarditis.
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PMID:[Intractable infective endocarditis associated with supraaortic stenosis in Williams syndrome: a case report]. 1216 46

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.
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PMID:Supravalvular aortic stenosis associated to infectious endocarditis and cerebral vascular disease in a patient with Williams-Beuren Syndrome. 2588 7

Supravalvular aortic stenosis is a less common form of left ventricular outflow tract obstruction (LVOTO); commonest being the valvular aortic stenosis followed by valvular and subvalvular forms respectively. Most of the supravalvular aortic stenosis is associated with Williams syndrome; isolated supravalvular aortic stenosis is further rarer. We present a case of isolated SVAS with infective endocarditis (1.6) as the cause of pyrexia of unknown origin (PUO).
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PMID:Isolated Supravalular Aortic Stenosis with Infective Endocarditis presenting as Pyrexia of Unknown Origin. 2879 78

Supravalvular aortic stenosis is the rarest form of congenital left ventricular outflow tract obstruction. It can be associated with Williams syndrome or it may be an isolated anomaly. Although infective endocarditis is common in valvular aortic stenosis, in supravalvular aortic stenosis infective endocarditis/endarteritis is rarely reported. We report a case of infective endarteritis in a case of supravalvular aortic stenosis due to resistant enterococci, causing prolonged fever. Blood culture-directed antibiotic therapy cured the infection. <Learning objective: Infective endarteritis in supravalvular aortic stenosis is a rarely reported but treatable entity. Although it may respond to empirical antibiotics, occasionally some resistant organism, causing infective endarteritis, may not respond to empirical therapy. Multiple blood culture with culture-directed antibiotic administration may be lifesaving, as in our index case.>.
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PMID:Infective endarteritis in a case of supravalvular aortic stenosis. 3054 28