Combined Supravalvular Aortic and Pulmonic Stenosis

Abstract

The association of supravalvular aortic stenosis and postvalvular pulmonic stenosis is described in three patients. In addition to the clinical findings resulting from obstruction to both ventricular outflow tracts, other unusual features were also present, such as a difference in the pulses and blood pressure of the upper extremities and absence of poststenotic dilatation of the ascending aorta. Diagnosis was established at cardiac catheterization by the demonstration of important pressure gradients in the aorta and in the pulmonary arteries with characteristic and almost identical pressure tracings on both sides. The pathologic lesions demonstrated in our patients by selective angiocardiography, surgery, and autopsy are discussed. The possibility of a common etiology for both entities is suggested.