Supravalvar stenosis of the pulmonary artery.

Abstract
The clinical and hemodynamic data in 14 patients with different types of supravalvar pulmonary stenosis are analyzed. In 9 patients the condition was associated with other cardiovascular anomalies. In 4 patients left-heart catheterization revealed the presence of aortic valvar stenosis in 2, coarctation of the aorta in 1, and aortic insufficiency in 1 patient. These associated conditions make the clinical symptomatology very complicated and a correct diagnosis can only be made by combining pressure curves, oximetry, intracardiac phonography, and selective angiocardiography. The practical importance of pre-operative diagnosis of supravalvar pulmonary stenosis in the surgical management of the so-called "associated lesions" is discussed.