A case of surgical Correction for Mitral Insufficiency and Supravalvular Aortic Stenosis with Williams syndrome

Pediatric Cardiology and Cardiac Surgery
Vol.25 No.5 2009 (691-694)

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Takashi Ono,¹⁾ Shigehiro Morishima,¹⁾ Makoto Nakazawa,²⁾ Masatomo Honda,³⁾ and Yoshimichi Kudo⁴⁾

Departments of ¹⁾Pediatric Cardiovascular Surgery, ²⁾Pediatric and Lifelong Congenital Cardiology Institute, ³⁾Cardiovascular Center, and ⁴⁾Pediatrics, Southern Tohoku General Hospital, Koriyama, Japan

Abstract

Although it has been recognized that supravalvular aortic stenosis is part of Williams syndrome, concomitant mitral insufficiency is rare. Furthermore, simultaneous surgery of supravalvular aortic stenosis and mitral insufficiency is extremely rare. We successfully performed single-patch augmentation for supravalvular aortic stenosis and mitral valve repair in an 8-year-old girl with Williams syndrome. The patient had been diagnosed with supravalvular aortic stenosis and Williams syndrome with ultrasound echocardiography and with clinical features such as elfin face and mental retardation. Her heart murmur increased at the age of 8 years, and cardiac catheterization revealed grade III mitral regurgitation and a pressure gradient of 30 mmHg at the left ventricular outflow. Mitral valve repair was performed by posterior commissural closure combined with annuloplasty. We were planning aortoplasty using the Doty technique for supravalvular aortic stenosis. However, because the ostium of the right coronary artery was close to the commissure between the left and right coronary sinuses, supravalvular aortic stenosis was repaired by single patch augmentation with incision into the non-coronary sinus. The pressure gradient at the left ventricular outflow tract was reduced, and mitral regurgitation improved after the operation.