Echocardiographic diagnosis of congenital supravalvular aortic stenosis in children / 中华超声影像学杂志

ABSTRACT

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 16 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.