Berry综合征的超声诊断

目的 探讨Berry综合征的超声诊断价值,分析其超声表现类型.方法 回顾性分析6例Berry综合征患者的超声图像,并结合文献分析超声表现的类型.结果 6例患者中,5例超声诊断经手术(3例)或心血管造影(2例)证实,另1例于超声诊断后14 d死亡.综合征畸形包括远端主一肺动脉间隔缺损、右肺动脉起自升主动脉、主动脉弓离断(A型合并动脉导管未闭5例)或狭窄(1例)伴室间隔完整.6例患者根据超声表现可分为两组:A组(3例)表现为间隔近乎缺如,左右肺动脉开口远离,右肺动脉血供主要来自升主动脉,主动脉弓离断;B组(3例)表现为左右肺动脉开口紧邻,联合部骑跨于远端间隔缺损上,右肺动脉血供分别来自升主动脉和主肺动脉,主动脉弓狭窄或离断.结论 经胸超声心动图能准确诊断Berry综合征的各种组合畸形,是术前诊断的首选方法.

Echocardiographic diagnosis of Berry syndrome

Objective To explore the value of transthoracic echocardiography in diagnosis of Berry syndrome and analyze the sonographic typing.Methods The echocardiographic results of 6 Berry patients were reviewed retrospectively.Their sonographic typing were also analyzed connected with the reports in the literature.Results The ultrasound diagnosis of 5cases were confirmed by surgery(3)or angiography(3) and the remain one infant was died 14 days after the echocardiographic procedure.The malformations of Berry syndrome consisted of the distal aorto-pulmonary septal defect(APSD),aortic origin of the right pulmonary artery(RPA),interruption of the aortic arch(5 cases,type A with patent ductus arteriosus)or coarctation of the aortic isthmus(1 case)with intact ventricular septum.In 6 cases,there were 2 sonographic types:(1)Type A(3 cases),the aorto-pulmonary septum was almost absent and the left and right pulmonary arteries remained widely separated,the blood of the RPA came almost from the aorta,the aortic arch was interrupted.(2)Type B(3 cases),the left and right pulmonary arteries remained adjacent and the bifurcation straddled the distal APSD,the blood of the RPA came from the aorta and the pulmonary trunk separately,the aortic arch was coarctated or interrupted. Conclusions The transthoracic echocardiography can be used to evaluate all containing malformations of Berry syndrome and it may be the first diagnostic choice.