A clinico-morphologic study was performed on 1120 patients who underwent aortic valve replacement at the Department of Medical and Surgical Cardiology, 2nd University Medical School of Naples, Naples, Italy, from January 1981 through December 1991. In 69 cases the aortic valve was incompetent due to a non-inflammatory aortic root disease such as myxomatous infiltration of the cusps and or aortic root dilatation. Among these patients males were prevalent (male/female ratio = 2.2). The mean age was 37 +/- 7.5 years. A floppy mitral valve was diagnosed in 16 cases while in one a left atrial myxoma was found. The patients were divided into 3 groups: Group 1-29 patients with aortic root dilatation and normal cusps; Group 2-25 patients with aortic root dilatation and myxomatous infiltration of aortic cusps (floppy aortic valve); and Group 3-15 patients with floppy aortic valve and undilated aortic root. At the gross examination the cusps of the patients in Groups 2 and 3 were redundant, thin, soft and gelatinous. The histology showed myxomatous infiltration with disruption of the fibrous layer. In patients with aortic root dilatation the histology of the aortic root fragments showed a cystic medial necrosis. Deep correlation was found between the root dilatation and the grade of aortic wall cystic medial necrosis. Cusp's diastasis was the cause of aortic regurgitation in patients with aortic root dilatation, while cusp prolapse caused aortic incompetence in presence of the floppy aortic valve and undilated aortic root.(ABSTRACT TRUNCATED AT 250 WORDS)