Successful mitral valve replacement in a patient with Ehlers-Danlos syndrome type VI.

A 40-year-old patient with Ehlers-Danlos syndrome type VI (ocular type) had mitral regurgitation due to mitral valve prolapse. Because the patient's tissue was fragile, we replaced the mitral valve with a reinforced prosthetic valve to prevent paravalvular leakage. The excised mitral leaflet showed significant myxomatous change and decrease in collagen fibers. We believe this is the first report of cardiac surgery in a patient with Ehlers-Danlos syndrome type VI.