caa a22 4500 47711279X CHVBK 20180405111614.0 cr unu---uuuuu 170330e19960701xx s 000 0 eng 10.1007/BF02559995 doi (NATIONALLICENCE)springer-10.1007/BF02559995 Combined aortic and mitral valve replacement in an adult with mucopolysaccharidosis (Maroteaux-Lamy syndrome) [Elektronische Daten] [Mitsuhiro Hachida, Masaki Nonoyama, Yukihiro Bonkohara, Naoji Hanayama, Hitoshi Koyanagi] Summary: A 41-year-old man with aortic stenosis, mitral stenosis, and tricuspid regurgitation due to Maroteaux-Lamy syndrome underwent aortic and mitral valve replacement with tricuspid annuloplasty. The annular diameter of the aortic and mitral valves was extremely small. The valve prostheses were 19 mm in diameter in the aortic position and 25 mm in the mitral position. Histologically, the valves showed thickening and hyalinization of the collagen fibers, and the presence of foamy cells that contained a large quantity of pure acid mucopolysaccharide. On an echocardiographic examination performed 2 years after the surgery, the peak systolic gradient of the trans-aortic valve was 18 mmHg at rest, and 26 mmHg during exercise. Careful observation of the residual pressure gradient will be needed. The patient's present New York Heart Association (NYHA) status is grade I and he has returned to work. Springer-Verlag, 1996 Valve disease nationallicence Mucopolysaccharidosis nationallicence Mitral valve stenosis nationallicence Aortic valve stenosis nationallicence Hachida Mitsuhiro Department of Cardiovascular Surgery, The Heart Institute of Japan, Tokyo Women's Medical College, 8-1 Kawada-cho, Shinjuku-ku, 162, Tokyo, Japan aut Nonoyama Masaki Department of Cardiovascular Surgery, The Heart Institute of Japan, Tokyo Women's Medical College, 8-1 Kawada-cho, Shinjuku-ku, 162, Tokyo, Japan aut Bonkohara Yukihiro Department of Cardiovascular Surgery, The Heart Institute of Japan, Tokyo Women's Medical College, 8-1 Kawada-cho, Shinjuku-ku, 162, Tokyo, Japan aut Hanayama Naoji Department of Cardiovascular Surgery, The Heart Institute of Japan, Tokyo Women's Medical College, 8-1 Kawada-cho, Shinjuku-ku, 162, Tokyo, Japan aut Koyanagi Hitoshi Department of Cardiovascular Surgery, The Heart Institute of Japan, Tokyo Women's Medical College, 8-1 Kawada-cho, Shinjuku-ku, 162, Tokyo, Japan aut Heart and Vessels Springer-Verlag 11/4(1996-07-01), 215-217 0910-8327 11:4<215 1996 11 380 https://doi.org/10.1007/BF02559995 text/html Onlinezugriff via DOI 1 research-article jats NATIONALLICENCE 856 40 https://doi.org/10.1007/BF02559995 text/html Onlinezugriff via DOI NATIONALLICENCE 700 1- Hachida Mitsuhiro Department of Cardiovascular Surgery, The Heart Institute of Japan, Tokyo Women's Medical College, 8-1 Kawada-cho, Shinjuku-ku, 162, Tokyo, Japan aut NATIONALLICENCE 700 1- Nonoyama Masaki Department of Cardiovascular Surgery, The Heart Institute of Japan, Tokyo Women's Medical College, 8-1 Kawada-cho, Shinjuku-ku, 162, Tokyo, Japan aut NATIONALLICENCE 700 1- Bonkohara Yukihiro Department of Cardiovascular Surgery, The Heart Institute of Japan, Tokyo Women's Medical College, 8-1 Kawada-cho, Shinjuku-ku, 162, Tokyo, Japan aut NATIONALLICENCE 700 1- Hanayama Naoji Department of Cardiovascular Surgery, The Heart Institute of Japan, Tokyo Women's Medical College, 8-1 Kawada-cho, Shinjuku-ku, 162, Tokyo, Japan aut NATIONALLICENCE 700 1- Koyanagi Hitoshi Department of Cardiovascular Surgery, The Heart Institute of Japan, Tokyo Women's Medical College, 8-1 Kawada-cho, Shinjuku-ku, 162, Tokyo, Japan aut NATIONALLICENCE 773 0- Heart and Vessels Springer-Verlag 11/4(1996-07-01), 215-217 0910-8327 11:4<215 1996 11 380 Metadata rights reserved Springer special CC-BY-NC licence nationallicence BK010053 XK010053 XK010000 NATIONALLICENCE NATIONALLICENCE NL-springer