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Unicuspid Aortic Valve in a 34 year old woman


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Contributor: Surita Sidhu MD and Ashraf Ghobashy MD
 

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34 year old female, diagnosed at age 10 with Turner's syndrome and known congenital aortic stenosis presented with new onset dyspnea on exertion.

Physical exam showed characteristic features of Turner's syndrome including short stature and mild neck webbing. Cardiac exam revealed a 3/6 crescendo/ decrescendo systolic ejection murmur. Lungs were clear, no jugular venous distention or carotid bruits. On transthoracic echocardiogram she had a thickened aortic valve with restricted mobility and moderately severe aortic stenosis. She had moderate aortic regurgitation as well as left atrial prominence and an ejection fraction of 75%. Cardiac catheterization revealed severe aortic stenosis with post-stenotic dilation as well as a hypertrophied left ventricle and normal coronaries.

Intraoperative transesophageal echocardiography showed a thickened and calcified unicuspid single commissure . The aorta was also calcified at the base and posterior wall and the ascending aorta was enlarged at 4.2 cm. A St. Jude aortic valve was placed. The patient's postoperative course was uncomplicated.

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June 6, 2000 (CJ)
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