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Replacement of the Aortic Valve in a Patient with Mucolipidosis III

Published online by Cambridge University Press:  24 September 2009

Linda H. Cripe ,
Stephanie M. Ware  and
Robert B. Hinton
Linda H. Cripe*
Affiliation:
The Heart Institute, Cincinnati Children’s Hospital Medical Center and The University of Cincinnati College of Medicine, Cincinnati, Ohio
Stephanie M. Ware
Affiliation:
The Heart Institute, Cincinnati Children’s Hospital Medical Center and The University of Cincinnati College of Medicine, Cincinnati, Ohio
Robert B. Hinton
Affiliation:
The Heart Institute, Cincinnati Children’s Hospital Medical Center and The University of Cincinnati College of Medicine, Cincinnati, Ohio
*
Correspondence to: Linda H. Cripe, MD, Division of Pediatric Cardiology, Cincinnati Children’s Hospital Medical Center, 3333 Burnet Avenue, Cincinnati, OH 45229-3039. Phone: 513-636-5476; Fax: 513-636-1875; E-mail: [email protected]
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Abstract

We report replacement of the aortic valve in a patient aged 9 years with mucolipidosis III and severe aortic insufficiency. Histopathology demonstrated abnormalities of the matrix and lysosomal inclusion bodies. As life expectancy increases for patients with lysosomal storage disorders, approaches to intervention for valvar disease become increasingly important.

Keywords

Cardiac surgeryaortic valvemetabolic disease
Type
Brief Reports
Information
Cardiology in the Young , Volume 19 , Issue 6 , December 2009 , pp. 641 - 643
Copyright
Copyright © Cambridge University Press 2009

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