de Vivie E R, Björnstad P G, Rastan H, Beuren A J, Koncz J
Thoraxchir Vask Chir. 1978 Oct;26(5):357-62. doi: 10.1055/s-0028-1096653.
Supravalvular aortic stenosis is either a syndrome combined with typical face characteristics mental retardation and peripheral pulmonary artery stenosis or it occurs as an isolated congenital heart defect. The diagnosis was confirmed in 137 patients by means of catheterization and angiocardiography; 35 pediatric and 10 adult patients were considered to be candidates for corrective surgery because of the severity of their disease. Age varied from 3 to 32 years. 7 patients (15,5%) died. Recatheterization was performed in 12 children 5,6 +/- 4,1 years after surgery. Left ventricular pressures were decreased from 184,1 +/- 28,4 to 155,5 +/- 32,1 mm Hg, and the pressure gradients between left ventricle and the aorta fell from 101,2 +/- 19,7 to 29,1 +/- 23,1 mm Hg. The postoperative values varied between 0 and 80 mm Hg. Operative results are discussed in relation to the anatomical type malformation and the operative technique.
瓣上主动脉狭窄要么是一种伴有典型面部特征、智力发育迟缓及外周肺动脉狭窄的综合征,要么作为一种孤立的先天性心脏缺陷出现。通过心导管检查和心血管造影术确诊了137例患者;35例儿科患者和10例成年患者因病情严重被视为矫正手术的候选对象。年龄从3岁至32岁不等。7例患者(15.5%)死亡。12名儿童在术后5.6±4.1年进行了再次心导管检查。左心室压力从184.1±28.4降至155.5±32.1毫米汞柱,左心室与主动脉之间的压力梯度从101.2±19.7降至29.1±23.1毫米汞柱。术后值在0至80毫米汞柱之间变化。结合解剖类型畸形和手术技术对手术结果进行了讨论。