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  • Title: [Surgical treatment of Williams syndrome combined with cardiovascular disease].
    Author: Meng Q, Sun LZ, Chang Q, Zhu JM, Wang SY, Hu SS.
    Journal: Zhonghua Wai Ke Za Zhi; 2005 May 15; 43(10):644-6. PubMed ID: 16008933.
    Abstract:
    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. CONCLUSION: 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.
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