Abstract Bicuspid aortic valve is the most common form of congenital heart disease (prevalence of 0.5 to 2.0%). These patients develop infective endocarditis at significantly higher rates than the general population; furthermore, they are at increased risk of developing infective endocarditis at a younger age owing to intrinsic factors of the aortic valve, but demonstrate better long–term survival after surgical treatment. This case report concerns a 33–year–old caucasian man who presented to the emergency department for persistent fever. He had no significant past medical history, except for a known bicuspid aortic (type 0) valve. The laboratory test demonstrated an active infection and blood cultures were positive, so we performed a transthoracic echocardiogram showing a mobile mass on the aortic valve, associated to a severe aortic regurgitation. Hence, we decided to perform a transesophageal echocardiogram that documented a bicuspid aortic valve with an extensive endocarditic vegetation on anterior leaflet, cusp prolapse and a non–homogenous peri–valvular area, involving the mitral–aortic junction, compatible with abscess. Perivalvular extension of infective endocarditis is the most frequent cause of uncontrolled infection and is associated with a poor prognosis so, as suggested by ESC Guidelines, this clinical case represented an indication for early surgery. The patient was transferred to cardiac surgery department where he underwent surgery. Extensive debridement of the endocarditic lesion was performed; the mitral–aortic junction was reconstructed by suturing a double patch of heterologous pericardium. The defect resulting from the debridement beneath the left coronary ostium was also reconstructed using an additional sutured pericardial patch; then, an Inspiris Resilia size 23 prosthesis was implanted. Transesophageal echocardiography revealed good functioning of the aortic valve prosthesis with mild mitral insufficiency. Meanwhile, blood cultures resulted positive for Staphylococcus Aureus coagulase–negative, so specific antibiotic therapy was started.
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