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.
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References
Shieken RM, Kerber RE, Ionasescu VV, Zellweger H (1975) Cardiac manifestations of the mucopolysaccharidoses. Circulation 52:700–705
Wilson CS, Mankin HT, Pluth JR (1980) Aortic stenosis and mucopolysaccharidosis. Ann Int Med 92:496–498
Butman SM, Karl L, Copeland JG (1989) Combined aortic and mitral valve replacement in an adult with Scheie's Disease. Chest 96:209–210
Maroteaux P, Leveque B, Marie J, Lamy M (1963) Une nouvelle dysostose avec elimination urinaire de chondroitine-sulfate B. Presse Med 71:1849–1852.
Young ID, Harper PS (1982) Mild for of Hunter's syndrome: Clinical delineation based on 31 cases. Arch Dis Child 57:828–836
Herd JK, Subramanian S, Robinson H (1973) Type III mucopolysaccharidosis: Report of a case with severe mitral valve involvement. J Pediatr 82:101–104
Tan CT, Schaff HV, Miller FA, Edwards WD, Karnes PS (1992) Valvular heart disease in four patients with Maroteaux-Lamy syndrome. Circulation 85:188–195
Nelson J, Shields MD, Mulholland HC (1990) Cardiovascular studies in the mucopolysaccharidoses. J Med Genet 27:94–100
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Hachida, M., Nonoyama, M., Bonkohara, Y. et al. Combined aortic and mitral valve replacement in an adult with mucopolysaccharidosis (Maroteaux-Lamy syndrome). Heart Vessels 11, 215–217 (1996). https://doi.org/10.1007/BF02559995
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DOI: https://doi.org/10.1007/BF02559995