Valvular heart disease in four patients with Maroteaux-Lamy syndrome.

@article{Tan1992ValvularHD,
  title={Valvular heart disease in four patients with Maroteaux-Lamy syndrome.},
  author={Christie Tan and Hartzell Vernon Schaff and Fletcher A. Miller and William D. Edwards and Pamela S Karnes},
  journal={Circulation},
  year={1992},
  volume={85 1},
  pages={188-95}
}
BACKGROUND Maroteaux-Lamy syndrome is a lysosomal storage disease of mucopolysaccharide metabolism (MPS type VI) that may involve the mitral and aortic valves. Affected patients have other skeletal and oropharyngeal malformations that complicate anesthetic and surgical management. METHODS AND RESULTS The present report describes the clinical, echocardiographic, and pathological findings in four patients with Maroteaux-Lamy syndrome. Two of three siblings underwent successful double-valve… CONTINUE READING
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