Aortic and Mitral Valve Involvement in Maroteaux-Lamy Syndrome VI: Surgical Implications in the Enzyme Replacement Therapy Era

Salvatore Torre; Mauro Scarpelli; Alessandro Salviati; Ebba Buffone; Giuseppe Faggian; Giovanni Battista Luciani

doi:10.1016/j.athoracsur.2015.11.062

Aortic and Mitral Valve Involvement in Maroteaux-Lamy Syndrome VI: Surgical Implications in the Enzyme Replacement Therapy Era

Ann Thorac Surg. 2016 Jul;102(1):e23-5. doi: 10.1016/j.athoracsur.2015.11.062.

Authors

Salvatore Torre¹, Mauro Scarpelli², Alessandro Salviati², Ebba Buffone³, Giuseppe Faggian¹, Giovanni Battista Luciani⁴

Affiliations

¹ Section of Cardiac Surgery, Department of Surgery, University of Verona, Verona, Italy.
² Section of Neurology, Department of Neurological Sciences and Movement Sciences, University of Verona, Verona, Italy.
³ Division of Neurology, Bussolengo Hospital, Bussolengo, Verona, Italy.
⁴ Section of Cardiac Surgery, Department of Surgery, University of Verona, Verona, Italy. Electronic address: [email protected].

PMID: 27343522
DOI: 10.1016/j.athoracsur.2015.11.062

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.

Publication types

Case Reports

MeSH terms

Adult
Aortic Valve / surgery*
Aortic Valve Stenosis / surgery
Enzyme Replacement Therapy
Female
Humans
Mitral Valve / surgery*
Mitral Valve Stenosis / surgery
Mucopolysaccharidosis VI / complications*
Mucopolysaccharidosis VI / therapy