Williams综合征是以特殊面容(鼻小、鼻孔前倾、虹膜发育不良等)、生长发育异常及各种心血管畸形为特征。最常见的畸形是瓣上型主动脉狭窄和外周性肺动脉狭窄,其他可有:肺动脉瓣狭窄、间隔缺损和外周性全身动脉狭窄。对这些畸形的自然病程的研究尚较少。病人和方法10例患儿(男6例)均具Williams综合征的典型特征。他们均作两次心导管检查,插管 Williams syndrome is characterized by a particular face (nasal small, nostril forward, iris dysplasia, etc.), growth and development abnormalities and a variety of cardiovascular malformations. The most common deformities are aortic valve stenosis and peripheral pulmonary stenosis, other may have: pulmonary valve stenosis, septal defect and peripheral arterial stenosis. The natural history of these deformities is still relatively small. Patients and Methods Ten patients (6 males) had typical features of Williams syndrome. They both performed cardiac catheterization and intubation