Congenital Aortic Stenosis Resulting From a Unicommissural Valve

Abstract

Clinical, electrocardiographic, phonocardiographic, radiographic, hemodynamic, and anatomic findings are presented in 21 adult patients with stenotic unicommissural aortic valves. Distinction between congenitally unicuspid and bicuspid aortic valves before operation or autopsy was not possible. Although the basic structure of the valve may render it inherently stenotic, the age at which a murmur was first noted (average, 19 years), the duration of a known murmur (average, 25 years), and the age of onset of first symptoms of left ventricular outflow obstruction (average, 41 years) strongly suggest that stenosis at least in part is acquired. The relationship of the true and false commissures to the coronary arterial ostia could be determined with certainty in 12 patients. The basic division of the aortic valve into left, right, and noncoronary cusps is maintained, but the raphes do not extend to the valve orifice. Because the aortic valve is attached to the ascending aorta at only one point (the true commissure), which is at the level of the orifice, valvotomy is hazardous, and valve replacement appears indicated when operative treatment becomes necessary in the adult patient with a stenotic unicommissural aortic valve.