Myxomatous mitral valve chordae. II: Selective elevation of glycosaminoglycan content.

BACKGROUND AND AIM OF THE STUDY: Chordal rupture in myxomatous mitral valves is the leading cause of leaflet prolapse and regurgitation. Increased glycosaminoglycan (GAG) content has been reported in these valves. Therefore, the biochemical differences between myxomatous and control mitral valve chordae were investigated. METHODS: The contents of hexuronic acid, DNA, water, and collagen in chordae from 45 myxomatous valves and 10 control valves were measured. Collagen and hexuronic acid quantities were normalized to wet and dry weights, and to DNA content. Different GAG classes were measured using fluorophore-assisted carbohydrate electrophoresis (FACE). RESULTS: Myxomatous chordae contained significantly more GAGs than controls after quantities were normalized for wet weight, dry weight, and DNA content. The FACE assay showed that the myxomatous chordae contained significantly more chondroitin/dermatan 6-sulfate when normalized to both wet and dry weight, and slightly more hyaluronan. In contrast to leaflets, which contain predominantly hyaluronan, the predominant GAG class in chordae was chondroitin/dermatan sulfate. Keratan sulfate, a GAG class previously unreported in valve tissues, was also discovered in the chordae. Myxomatous chordae contained more water and less collagen than control chordae, but equal quantities of DNA when normalized for wet weight. CONCLUSION: Cells in the chordae of myxomatous valves may produce more GAGs than cells in the chordae of control valves. The resulting accumulation of GAGs and bound water likely gives myxomatous valves their characteristic thickening and floppy, gelatinous nature, and may account for their reported mechanical weaknesses.