title: Aortic valve replacement in a patient with morquio syndrome.
abstract: 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.
authors.affiliation: Department of Cardiac Surgery, University of Parma, Parma, Italy. francesco.nicolini@unipr.it
authors.forename: Francesco
authors.initials: F
authors.lastname: Nicolini
authors.forename: Domenico
authors.initials: D
authors.lastname: Corradi
authors.forename: Silvia
authors.initials: S
authors.lastname: Bosio
authors.forename: Tiziano
authors.initials: T
authors.lastname: Gherli
mesh_terms: D000328:Adult; D001024:Aortic Valve Stenosis / Q000175:diagnosis* / Q000601:surgery*; D006350:Heart Valve Prosthesis; D006801:Humans; D008297:Male; D009085:Mucopolysaccharidosis IV / Q000175:diagnosis* / Q000601:surgery*; D019919:Prosthesis Implantation / Q000379:methods*; D016896:Treatment Outcome