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.>.
Keywords: Enterococci; Infective endarteritis; Supravalvular aortic stenosis.