Infective Endocarditis in a Patient with Williams’ Syndrome: Case Report

An 18-year-old male was admitted to Seoul National University Hospital for the evaluation of fever and chill on February 3, 1988. On physical examination, his face showed a characteristic “elfin” facial appearance. His face was characterized by abnormalities of dental development, a broad overhanging upper lip, high arched palate and gum hypertrophy. He also showed mental retardation. Cardiac catheterization with selective cineangiocardiography demonstrated a supravalvular aortic narrowing, grade 2 aortic insufficiency, and moderately dilated proximal coronary arteries with normal distribution without an intraluminal narrowing. There was a systolic pressure gradient (55mmHg) between the aortic root and ascending aorta distal to a stenotic segment. MRI showed a supravalvular aortic stenosis. Vegetation was not found on echocardiograpghy. Unidentified G(−) rods were isolated in 3 out of 9 bottles in blood culture test. He was treated with Na-penicillin and gentamicin for 28 days.