Infective endarteritis in a case of supravalvular aortic stenosis

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.