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KMID : 0364019930260020135
Korean Journal of Thoracic and Cardiovascular Surgery
1993 Volume.26 No. 2 p.135 ~ p.140
Surgical Treatment of Supravalvular Aortic Stenosis
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Abstract
Supravalvular aortic stenosis is an uncommon, congenital narrowing of ascending aorta just above aortic valve.
Eleven patients underwent an aortoplasty to relieve supravalvular aortic stenosis at Sejong General Hospital from July 1985 to December 1991. Age ranged from 5 to 14 years(median 9 years). There were 7 male and 4 female patients. Seven patients
had
characteristics of Williams' syndrome including elfin face and mental retardation. All patients had localized, hourglass type but 4 patients had atypical findings. Preoperative left ventricula-aortic pressure gradient ranged from 40 to 190
mmHg(mean 88
mmHg).
To relieve severe supravalvular aortic stenosis, extended aortoplasty was used in 7 patients and standard aortoplasty in 4 patients. Postoperative pressure gradient ranged from 0 to 40 mmHg(mean 16.6mmHg). Follow-up pressure gradient with Doppler
ranged
from 0 to 88 mmHg(mean 32.5mmHg). (Korean J Thoracic Cardiovas Surg 1994; 27:135-40)
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