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Mitral Valve Repair and Cryo-Maze Procedure in Ehlers-Danlos Syndrome

Department of Cardiovascular Surgery, National Cardiovascular Center, Osaka, Japan

For reprint information contact: Junjiro Kobayashi, MD Tel: 81 6 6833 5012 Fax: 81 6 6872 7486 Email: jkobayas{at}hsp.ncvc.go.jp, Department of Cardiovascular Surgery, National Cardiovascular Center, 5-7-1 Fujishirodai, Suita, Osaka 565–8565, Japan.

A 48-year-old man presented with typical features of Ehlers-Danlos syndrome, such as soft and hyperextensible skin, subcutaneous nodules, clubbed feet, and a mild degree of pigeon chest. In addition, his past history revealed congenital dislocation of the hip joint, retinal detachment, and repeated episodes of congestive heart failure due to severe mitral regurgitation and atrial fibrillation. We subjected the patient to surgical repair of the mitral valve combined with the maze procedure, which proved successful.