[Surgical treatment of Williams syndrome combined with cardiovascular disease].
OBJECTIVE
To introduce the experience of diagnosis and surgical treatment of Williams syndrome combined with cardiovascular disease.


METHODS
Between October 1996 and June 2003, 8 patients of Williams syndrome with cardiovascular disease were admitted in Fuwai hospital. Seven patients underwent surgical correction. One didn't undergo surgical procedure. There were 6 male and 2 female ranging from 1.5 to 12.0 years old (medium age 6.4). Three had localized type supravalvular aortic stenosis and 5 diffused type supravalvular aortic stenosis. In them, 2 patients were combined with peripheral pulmonary stenosis. Single patch aortoplasty were performed in 6 cases, and inverted bifurcated patch aortoplasty in one patient.


RESULTS
One patient died and one patient suffered renal insufficiency. In the early postoperative period, the mean speed of flow was reduced to 1.7 m/s from 4.6 m/s, and the mean systolic pressure gradient was reduced from 91 mm Hg to 18 mm Hg. Six patients were followed up 16 to 91 months. There were 5 cases in NYHA function class I, and one in class II.


CONCLUSIONS
Satisfied result can be achieved in surgical treatment of Williams syndrome with supravalvular aortic stenosis, but it is not in combined with peripheral pulmonary stenosis.
Meng
Qiang
Q
Department of Surgery, Cardiovascular Institute and Fuwai Hospital, Chinese Academy of Medical Sciences, Peking Union Medical College, Beijing 100037, China. qmeng@sina.com
Sun
Li-zhong
LZ
Chang
Qian
Q
Zhu
Jun-ming
JM
Wang
Shui-yun
SY
Hu
Sheng-shou
SS
D021921:Aortic Stenosis, Supravalvular / Q000150:complications / Q000151:congenital / Q000601:surgery*; D002648:Child; D002675:Child, Preschool; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D008297:Male; D011666:Pulmonary Valve Stenosis / Q000150:complications / Q000151:congenital / Q000601:surgery*; D016896:Treatment Outcome; D018980:Williams Syndrome / Q000150:complications / Q000601:surgery*