PMID- 9119812
OWN - NLM
STAT- MEDLINE
DCOM- 19970418
LR  - 20191102
IS  - 0910-8327 (Print)
IS  - 0910-8327 (Linking)
VI  - 11
IP  - 4
DP  - 1996
TI  - Combined aortic and mitral valve replacement in an adult with 
      mucopolysaccharidosis (Maroteaux-Lamy syndrome).
PG  - 215-7
AB  - 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.
FAU - Hachida, M
AU  - Hachida M
AD  - Department of Cardiovascular Surgery, Heart Institute of Japan, Tokyo Women's 
      Medical College, Japan.
FAU - Nonoyama, M
AU  - Nonoyama M
FAU - Bonkohara, Y
AU  - Bonkohara Y
FAU - Hanayama, N
AU  - Hanayama N
FAU - Koyanagi, H
AU  - Koyanagi H
LA  - eng
PT  - Case Reports
PT  - Journal Article
PL  - Japan
TA  - Heart Vessels
JT  - Heart and vessels
JID - 8511258
SB  - IM
MH  - Adult
MH  - Aortic Valve
MH  - Aortic Valve Stenosis/diagnosis/etiology/*surgery
MH  - Aortography
MH  - Cardiac Catheterization
MH  - Echocardiography
MH  - Follow-Up Studies
MH  - Heart Valve Prosthesis/*methods
MH  - Humans
MH  - Male
MH  - Mitral Valve
MH  - Mitral Valve Stenosis/diagnosis/etiology/*surgery
MH  - Mucopolysaccharidoses/*complications
MH  - Syndrome
EDAT- 1996/01/01 00:00
MHDA- 1996/01/01 00:01
CRDT- 1996/01/01 00:00
PHST- 1996/01/01 00:00 [pubmed]
PHST- 1996/01/01 00:01 [medline]
PHST- 1996/01/01 00:00 [entrez]
AID - 10.1007/BF02559995 [doi]
PST - ppublish
SO  - Heart Vessels. 1996;11(4):215-7. doi: 10.1007/BF02559995.