PubMed1987
Supravalvular aortic stenosis (SAS) is an unusual form of progressive left ventricular outflow tract (LVOT) obstruction, frequently associated with others vascular and physical anomalies. Between July 1980 and July 1986 eight such patients have been operated upon. In seven patients patch aortoplasty (according to McGoon or Doty technique) relieved the stenosis; in one, a two years old boy, with associated severe hypoplasia of the ascending aorta and aortic arch, a left ventricle (LV) - abdominal aorta valved conduit was associated to aortoplasty. One patient died in the operating room with a severe anterior myocardial infarction; seven patients are in good clinical condition at a mean follow-up of 32 months. The aortic supravalvular gradient was dramatically reduced in all patients according with Doppler echocardiography. In our mind, patients with SAS would benefit from an early surgical repair, because of progressive involvement of the aortic valve, coronary arteries and LV. The surgical technique is strictly related to the anatomical situations. Per and postoperative management should rely on the pathophysiology peculiar to this malformation (especially about coronary perfusion). Taking care to these points, the surgical results at a mid term follow-up are now very satisfactory in this cardiac malformation.