Cardiology
General Cardiology
Cardiac Structural Involvement in MucopolysaccharidosesRigante D. · Segni G.Department of Pediatrics, Università Cattolica Sacro Cuore,
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Article / Publication Details
Received: March 26, 2002
Accepted: April 07, 2002
Published online: September 26, 2002
Issue release date: September 2002
Number of Print Pages: 3
Number of Figures: 0
Number of Tables: 1
ISSN: 0008-6312 (Print)
eISSN: 1421-9751 (Online)
For additional information: https://www.karger.com/CRD
Abstract
Mucopolysaccharidoses (MPS) are lysosomal storage disorders due to impaired glycosaminoglycan degradation. Cardiac involvement is present in most patients with MPS although its clinical impact is still undetermined. Cardiovascular abnormalities were evaluated in 39 patients with MPS aged 4–22 years. Valvular lesions and different forms of cardiac involvement were detected. The most common lesion was thickening of the mitral valve with regurgitation or stenosis, regardless of the MPS type. Mitral valve thickening was observed in 23 patients, aortic valve thickening in 11 patients and congestive heart failure in only 1 patient with MPS III. The most severe changes were registered for MPS types I and II. Complete cardiological investigation should be routinely warranted in every patient inflicted with MPS.
© 2002 S. Karger AG, Basel
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Article / Publication Details
Received: March 26, 2002
Accepted: April 07, 2002
Published online: September 26, 2002
Issue release date: September 2002
Number of Print Pages: 3
Number of Figures: 0
Number of Tables: 1
ISSN: 0008-6312 (Print)
eISSN: 1421-9751 (Online)
For additional information: https://www.karger.com/CRD
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