title: Aortic and Mitral Valve Involvement in Maroteaux-Lamy Syndrome VI: Surgical Implications in the Enzyme Replacement Therapy Era.
abstract: Open-heart operations in patients with mucopolysaccharidoses are exceedingly rare and pose distinct clinical challenges. Few reports exist of valve replacement in type VI mucopolysaccharidosis, mostly entailing combined mitral and aortic valve replacement. Here reported is the case of a young woman with mitral and aortic valve disease, in whom the surgical procedure was confined to the aortic valve. The rationale behind this strategy, particularly in light of the benefits offered by specific enzyme replacement therapy of type VI mucopolysaccharidosis, is discussed.
authors.affiliation: Section of Cardiac Surgery, Department of Surgery, University of Verona, Verona, Italy.
authors.forename: Salvatore
authors.initials: S
authors.lastname: Torre
authors.affiliation: Section of Neurology, Department of Neurological Sciences and Movement Sciences, University of Verona, Verona, Italy.
authors.forename: Mauro
authors.initials: M
authors.lastname: Scarpelli
authors.affiliation: Section of Neurology, Department of Neurological Sciences and Movement Sciences, University of Verona, Verona, Italy.
authors.forename: Alessandro
authors.initials: A
authors.lastname: Salviati
authors.affiliation: Division of Neurology, Bussolengo Hospital, Bussolengo, Verona, Italy.
authors.forename: Ebba
authors.initials: E
authors.lastname: Buffone
authors.affiliation: Section of Cardiac Surgery, Department of Surgery, University of Verona, Verona, Italy.
authors.forename: Giuseppe
authors.initials: G
authors.lastname: Faggian
authors.affiliation: Section of Cardiac Surgery, Department of Surgery, University of Verona, Verona, Italy. Electronic address: giovanni.luciani@univr.it.
authors.forename: Giovanni Battista
authors.initials: GB
authors.lastname: Luciani
mesh_terms: D000328:Adult; D001021:Aortic Valve / Q000601:surgery*; D001024:Aortic Valve Stenosis / Q000601:surgery; D056947:Enzyme Replacement Therapy; D005260:Female; D006801:Humans; D008943:Mitral Valve / Q000601:surgery*; D008946:Mitral Valve Stenosis / Q000601:surgery; D009087:Mucopolysaccharidosis VI / Q000150:complications* / Q000628:therapy