Imaging of cardiovascular malformations in Williams syndrome
LI Shi-guo, ZHAO Shi-hua, JIANG Shi-liang, HUANG Lian-jun, XU Zhong-ying, LING Jian, ZHENG Hong, YAN Chao-wu, LU Jin-guo
Published 2008-09-10
Cite as Chin J Radiol, 2008,42(09): 916-918. DOI: 10.3321/j.issn:1005-1201.2008.09.005
Abstract
Objective To evaluate the imaging methods for cardiovascular malformations in Williams syndrome(WS).Methods Thirteen cases of WS(7 males and 6 females)aged 10 months to 13 years were involved in this study.All patients underwent chest X-ray radiography,electrocardiography.echocardiography and physical examination.3 cases underwent electronic beam computed tomography (EBCT),cardiac catheterization and angiography were performed in 8 cases.Results Twelve patients were referred to our hespital for cardiac murmur and 1 cnse for cyanosis after birth.7 patients were found with "elfin-like"facial features.6 patients with pulmonary arterial stenosis.2 Cases with patent ductus arteriosus.2 case8 with 8evere pulmonary hypertension and 1 case with total endocardial cushion defect.Sudden death occurred in 2 patients during and after catlleterization.respectively. Conclusions Conventional angiography is the golden standard for the diagnosis of cardiovascular malformations in WS.Noninvnsive methods such as MSCT and MRI should be suggested because of the risk of sudden death in conventional angiography.
Key words:
Williams syndrome; Cardiovascular abnormalities; Diagnostic imaging; Death,sudden
Contributor Information
LI Shi-guo
Department of Radiology,Cardiovascular Institute and Fuwai Hospital,Peking Union Medical College,Chinese Academy of Medical Sciences,Beijing 100037,China
ZHAO Shi-hua
Department of Radiology,Cardiovascular Institute and Fuwai Hospital,Peking Union Medical College,Chinese Academy of Medical Sciences,Beijing 100037,China
JIANG Shi-liang
Department of Radiology,Cardiovascular Institute and Fuwai Hospital,Peking Union Medical College,Chinese Academy of Medical Sciences,Beijing 100037,China
HUANG Lian-jun
Department of Radiology,Cardiovascular Institute and Fuwai Hospital,Peking Union Medical College,Chinese Academy of Medical Sciences,Beijing 100037,China
XU Zhong-ying
Department of Radiology,Cardiovascular Institute and Fuwai Hospital,Peking Union Medical College,Chinese Academy of Medical Sciences,Beijing 100037,China
LING Jian
Department of Radiology,Cardiovascular Institute and Fuwai Hospital,Peking Union Medical College,Chinese Academy of Medical Sciences,Beijing 100037,China
ZHENG Hong
Department of Radiology,Cardiovascular Institute and Fuwai Hospital,Peking Union Medical College,Chinese Academy of Medical Sciences,Beijing 100037,China
YAN Chao-wu
Department of Radiology,Cardiovascular Institute and Fuwai Hospital,Peking Union Medical College,Chinese Academy of Medical Sciences,Beijing 100037,China
LU Jin-guo
Department of Radiology,Cardiovascular Institute and Fuwai Hospital,Peking Union Medical College,Chinese Academy of Medical Sciences,Beijing 100037,China