Journal of Pediatric Cardiology and Cardiac Surgery

Online ISSN: 2433-1783 Print ISSN: 2433-2720
Japanese Society of Pediatric Cardiology and Cardiac Surgery
Japanese Society of Pediatric Cardiology and Cardiac Surgery Academy Center, 358-5 Yamabuki-cho, Shinju-ku, Tokyo 162-0801, Japan
Journal of Pediatric Cardiology and Cardiac Surgery 5(2): 104-108 (2021)
doi:10.24509/jpccs.20-042

Case ReportCase Report

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

1Department of Pediatrics, Graduate School of Medical Sciences, Kyushu University ◇ Fukuoka, Japan

2Department of Cardiovascular Medicine, Faculty of Medical Sciences, Kyushu University ◇ Fukuoka, Japan

3Department of Cardiovascular Surgery, Kyushu University Hospital ◇ Fukuoka, Japan

受付日:2020年11月26日Received: November 26, 2020
受理日:2021年5月20日Accepted: May 20, 2021
発行日:2021年7月1日Published: July 1, 2021
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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.

Key words: Williams syndrome; infective endocarditis; mitral regurgitation

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