Abstract

FIGURE 1. Gross image of the aorta showing irregular mural vegetation adjacent to an area of mural necrosis and early aneurysm formation. (Original magnification, 1.53.) CLINICAL SUMMARY A 5-month-old boy presented with fever, tachycardia, and a systolic heart murmur. At 1 month of age, he had been treated for congenital aortic stenosis (AS) with balloon aortic valvoplasty. Six weeks before the present illness, a catheterization was performed to assess the residual left ventricular outflow tract gradient and pulmonary vascular bed. During the procedure, there was no suspicion of an intimal tear or dissection. This study and a subsequent echocardiogram showed mild AS and trace aortic insufficiency. After a lumbar puncture, antibiotics were initiated for a presumptive diagnosis of meningitis. Two days later, there was seizure activity, and imaging showed a subacute infarction in the right middle cerebral artery distribution. Blood culture results were positive for Enterococcus faecalis. An echocardiogram revealed thickened aortic valve leaflets with vegetation adjacent to the left cusp and an abnormal appearance of the ascending aorta, suggesting aortitis with intraluminal hematoma, dissection, or pseudoaneurysm. Brain magnetic resonance imaging demonstrated an evolving infarction in right middle cerebral artery distribution. The patient was seizure free and had no focal neurologic findings. Surgical intervention was planned to replace the aortic valve and ascending aorta. At the time of the operation, the entire ascending aorta appeared thickened and inflamed (Figures 1 and 2). Aortic cannulation was performed more distal than usual in the transverse arch to allow for high aortic crossclamp place-

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