Infective Endocarditis Associated with Mitral Regurgitation in Two Cases of Williams Syndrome

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Williams syndrome (WS) is a congenital anomaly affecting various organs, especially cardiac tissues. While supravalvular aortic stenosis is common during childhood, mitral regurgitation usually develops during adulthood. To the best of our knowledge, only two reports have demonstrated infective endocarditis (IE) associated with mitral valve regurgitation in WS. We report two cases of IE in adult patients with WS. Case 1 was a 30-year-old woman presenting with dyspnea and remittent fever, severe mitral regurgitation, and atrial fibrillation. She had dental abnormalities and received dental care without prophylactic antibiotics 2 weeks before admission. Blood cultures were positive for Streptococcus gordonii, which is sensitive to penicillin G and ampicillin. She was successfully treated with antibiotics and surgical mitral valve plasty. Case 2 was a 34-year-old woman with IE following urinary tract infection. Methicillin-susceptible Staphylococcus aureus was the causative agent of IE. Mitral valve plasty was performed after 6-weeks of treatment with antibiotics. WS are at a high risk of bacteremia because of dental abnormalities. Mitral valve prolapse and regurgitation develop during adulthood, which might be associated with the onset of IE in patients with this disease.