Document Detail

Combined aortic and mitral valve replacement in a child with mucopolysaccharidosis type I: a case report.
MedLine Citation:
PMID:  19455897     Owner:  NLM     Status:  MEDLINE    
Mucopolysaccaridosis syndromes are metabolic disorders that are inherited in an autosomal recessive pattern, and demonstrate cardiovascular involvement that, on rare occasions, results in surgery. The case is presented of a 12-year-old boy with symptoms and signs of congestive heart failure who was referred to the authors' department for severe mitral and aortic valvular stenosis. The patient was shown to have the somatic characteristics of mucopolysaccharidosis, while urinalysis indicated the presence of chondroitin sulfate B. Mitral and aortic valve replacements were carried out, using bileaflet mechanical valves with a standard median sternotomy and cardiopulmonary bypass. A bileaflet preservation technique was adopted during mitral valve replacement to provide better hemodynamics. Severe cardiac involvement in mucopolysaccharidosis requiring cardiac surgery in pediatrics has been rarely documented. A low-profile bileaflet mechanical valve, enabling a larger-sized valve insertion, is preferred in order to avoid patient-prosthesis mismatch during somatic growth of the patient.
Onur S Goksel; Helin El; Emin Tireli; Enver Dayioglu
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Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  The Journal of heart valve disease     Volume:  18     ISSN:  0966-8519     ISO Abbreviation:  J. Heart Valve Dis.     Publication Date:  2009 Mar 
Date Detail:
Created Date:  2009-05-21     Completed Date:  2009-06-19     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  9312096     Medline TA:  J Heart Valve Dis     Country:  England    
Other Details:
Languages:  eng     Pagination:  214-6     Citation Subset:  IM    
Istanbul University, Istanbul Medical Faculty, Department of Cardiovascular Surgery, Istanbul, Turkey.
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MeSH Terms
Aortic Valve Stenosis / etiology,  surgery*
Cardiopulmonary Bypass
Heart Valve Prosthesis*
Mitral Valve Stenosis / etiology,  surgery*
Mucopolysaccharidosis I / complications*
Severity of Illness Index

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