title: Valve surgery in a mucopolysaccharidosis type I patient: early prosthetic valve endocarditis.
abstract: Mucopolysaccharidosis (MPS) are rare genetic disorders, caused by enzymatic defects that lead to abnormal glycosaminoglycan metabolism and its accumulation. Hurler-Scheie syndrome (MPS I) is associated with a deficiency of the lysosomal enzyme α-L-iduronidase. Enzymatic replacement with intravenous laronidase is a frequently utilized therapeutic option. In patients with MPS I, progressive glycosaminoglycan storage in the heart can lead to valvular abnormalities; however, few surgical heart valve interventions have been reported in MPS I patients. We present an unusual case of a double-valve replacement in an MPS I patient, complicated by early infective endocarditis requiring surgical reintervention. We also present a comprehensive literature review of valve surgery in patients with MPS I and a brief summary of the most relevant surgical considerations, including valve selection and infection prevention.
authors.affiliation: Department of Cardiothoracic Surgery, University of Pittsburgh Medical Center, Pittsburgh, PA, USA.
authors.forename: Rodolfo V
authors.initials: RV
authors.lastname: Rocha
authors.forename: Rene J
authors.initials: RJ
authors.lastname: Alvarez
authors.forename: Christian A
authors.initials: CA
authors.lastname: Bermudez
mesh_terms: D001024:Aortic Valve Stenosis / Q000601:surgery; D004696:Endocarditis / Q000601:surgery*; D005260:Female; D006349:Heart Valve Diseases / Q000601:surgery*; D006350:Heart Valve Prosthesis; D019918:Heart Valve Prosthesis Implantation; D006801:Humans; D008875:Middle Aged; D008946:Mitral Valve Stenosis / Q000601:surgery; D008059:Mucopolysaccharidosis I / Q000601:surgery*; D016459:Prosthesis-Related Infections / Q000601:surgery*; D012086:Reoperation