小儿先天性主动脉瓣上狭窄的超声诊断

目的 探讨小儿先天性主动脉瓣上狭窄(SVAS)的超声心动图特点及其诊断价值.方法 超声心动图在多个切面上检查诊断为主动脉瓣上狭窄的31例息儿,与心导管、手术及基因检测相对照.超声重点观察主动脉瓣、瓣上、主动脉弓降部、肺动脉瓣、主肺动脉及其左右分支、冠状动脉的超声改变.结果 沙漏样环形狭窄26例,全段管型狭窄4例,隔膜型狭窄1例.极轻度狭窄狭窄处的最大瞬时压差(△P)<25 mm Hg(1 mm Hg=0.133 kPa)]2例,轻度狭窄(△p 25～49 mm Hg)20例,中度狭窄(△P 50～75 mm Hg)5例,重度狭窄(△P>75 mm Hg)4例.19例患儿基因检测诊断为Williams综合征.合并主动脉瓣狭窄3例,其中1例首次检查时漏诊.合并肺动脉狭窄10例,占32.26%.其中肺动脉瓣狭窄6例,左、右肺动脉狭窄3例,左右肺动脉分叉处狭窄1例.合并冠状动脉扩张6例.结论 胸骨旁及心尖五腔切面为诊断SVAS较好的切面,SVAS好发于Williams综合征的患儿,32.26%的患儿伴有肺动脉狭窄.对于SVAS患儿需常规检查冠状动脉的改变.

Echocardiographic diagnosis of congenital supravalvular aortic stenosis in children

Objective To study the echocardiographic characteristics and its diagnosis value on congenital supravalvular aortic stenosis (SVAS) in children. Methods Thirty-one patients with SVAS diagnosed by multiplane echocardiography were enrolled in the study. Their echocardiographic characteristics were compared with cardiac catheterization, operation, and gene detection results. Echocardiographic changes were mainly observed in aortic valve, supravalve, descending aortic arch, pulmonary artery valve, main pulmonary artery and its branches,and coronary artery. Results Of the 31 patients,26 had hourglass type SVAS,4 hypoplastic type,and 1 membranous type; 2 patients had extremely mild stenosis (defined as a Doppler gradient <25 mm Hg) ,20 mild (25～49 mm Hg) ,5 moderate (50～75 mm Hg) ,and 4 severe C>75 mm Hg) ones. Nineteen patients were diagnosed with Williams syndrome by gene detection. Three patients were associated with aortic valve stenosis including one missed at the initial diagnosis; 10(32.26%) patients with pulmonary stenosis, including pulmonary valve stenosis in 6, left and right pulmonary artery stenosis in 3 ,and branch stenosis in 1:6 patients with coronary stenosis. Conclusions The sternal border and five chamber apical views are the best to detect SVAS. Williams syndrome patients are prone to SVAS.Pulmonary stenosis echocardiography forms a great proportion of the SVAS patients. Routine examination is necessary for coronary stenosis in cases of SVAS.