Aortic valve replacement in a patient with morquio syndrome

Cardiac involvement in Morquio syndrome, mucopolysaccharidosis IV, is characterized by aortic and mitral valve thickening and infiltration of the coronary arteries. There are few reports concerning surgical interventions in patients with mucopolysaccharidoses. We report a case of a patient affected...

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Bibliographic Details
Published inThe Heart surgery forum Vol. 11; no. 2; p. E96
Main Authors Nicolini, Francesco, Corradi, Domenico, Bosio, Silvia, Gherli, Tiziano
Format Journal Article
LanguageEnglish
Published United States 2008
Subjects
Adult
Aortic Valve Stenosis - diagnosis
Aortic Valve Stenosis - surgery
Heart Valve Prosthesis
Humans
Male
Mucopolysaccharidosis IV - diagnosis
Mucopolysaccharidosis IV - surgery
Prosthesis Implantation - methods
Treatment Outcome
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Summary:Cardiac involvement in Morquio syndrome, mucopolysaccharidosis IV, is characterized by aortic and mitral valve thickening and infiltration of the coronary arteries. There are few reports concerning surgical interventions in patients with mucopolysaccharidoses. We report a case of a patient affected by Morquio syndrome who underwent aortic valve replacement surgery for severe aortic valve stenosis, with an uneventful postoperative course. Cardiac surgery appears safe and feasible in these patients and improves the quality of life, even if the future prognosis related to the underlying disorder should be considered.
ISSN:1522-6662
DOI:10.1532/HSF98.20071197