Document Detail


Valve surgery in a mucopolysaccharidosis type I patient: early prosthetic valve endocarditis.
MedLine Citation:
PMID:  21820914     Owner:  NLM     Status:  Publisher    
Abstract/OtherAbstract:
Mucopolysaccharidosis (MPS) are rare genetic disorders, caused by enzymatic defects that lead to abnormal glycosaminoglycan metabolism and its accumulation. Hurler-Scheie syndrome (MPS I) is associated with a deficiency of the lysosomal enzyme α-l-iduronidase. Enzymatic replacement with intravenous laronidase is a frequently utilized therapeutic option. In patients with MPS I, progressive glycosaminoglycan storage in the heart can lead to valvular abnormalities; however, few surgical heart valve interventions have been reported in MPS I patients. We present an unusual case of a double-valve replacement in an MPS I patient, complicated by early infective endocarditis requiring surgical reintervention. We also present a comprehensive literature review of valve surgery in patients with MPS I and a brief summary of the most relevant surgical considerations, including valve selection and infection prevention.
Authors:
Rodolfo V Rocha; Rene J Alvarez; Christian A Bermudez
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Publication Detail:
Type:  JOURNAL ARTICLE     Date:  2011-8-4
Journal Detail:
Title:  European journal of cardio-thoracic surgery : official journal of the European Association for Cardio-thoracic Surgery     Volume:  -     ISSN:  1873-734X     ISO Abbreviation:  -     Publication Date:  2011 Aug 
Date Detail:
Created Date:  2011-8-8     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  8804069     Medline TA:  Eur J Cardiothorac Surg     Country:  -    
Other Details:
Languages:  ENG     Pagination:  -     Citation Subset:  -    
Copyright Information:
Copyright © 2011 European Association for Cardio-Thoracic Surgery. Published by Elsevier B.V. All rights reserved.
Affiliation:
Department of Cardiothoracic Surgery, University of Pittsburgh Medical Center, Pittsburgh, PA, USA.
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