[Hemodynamic results of surgery for congenital supravalvular aortic stenoses (author's transl)].

Abstract

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.