Gross anatomic, histologic and ultrastructural studies were made on 32 floppy aortic valves (FAVs) resected at the time of aortic valvular replacement for aortic regurgitation. Patients with the FAVs had relatively long clinical courses and had severe aortic regurgitation with mild symptoms of heart failure. The sizes of the mechanical valves implanted in the patients with FAVs were not large, indicating that the aortic regurgitation in these patients was not worsened by dilatation of the aortic ring. Two types of FAVs were recognized grossly, according to whether they showed abnormal cuspal thickening or thinning. Accumulations of myxoid material in the spongiosa were found in all FAVs, regardless of cuspal gross morphology. Histologically, the collagen fibers were sparse and irregularly arranged and elastic fibers were disrupted and finely granular in the myxomaotus areas of FAVs. Ultrastructurally, the myxomatous material consisted of numerous star-shaped proteoglycan granules associated with spiraling collagen fibrils and abnormal elastic fibers. Numerous spiraling collagen fibrils were observed especially at the border area of myxomatous change that extended from the spongiosa into the fibrosa. Abnormal elastic fibers had either a granular appearance of their amorphous components without microfibrils, or irregularly arranged masses of microfibrils without amorphous components. These abnormalities of connective tissue components, resulting from defective formation and/or increased degradation were similar to those in floppy mitral valves, and were related to the floppiness of cardiac valves.