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Valve surgery in a mucopolysaccharidosis type I patient: early prosthetic valve endocarditis

Rodolfo V Rocha1, Rene J Alvarez, Christian A Bermudez

  • 1Department of Cardiothoracic Surgery, University of Pittsburgh Medical Center, Pittsburgh, PA, USA.

European Journal of Cardio-Thoracic Surgery : Official Journal of the European Association for Cardio-Thoracic Surgery
|August 9, 2011
PubMed

Insights

Mucopolysaccharidosis (MPS) patients with Hurler-Scheie syndrome (MPS I) may develop heart valve issues. This case highlights complications after double-valve replacement and reviews surgical considerations for MPS I.

Area of Science:

  • Cardiovascular Medicine
  • Rare Genetic Disorders
  • Lysosomal Storage Diseases

Background:

  • Mucopolysaccharidosis (MPS) are rare genetic disorders resulting from enzyme deficiencies causing glycosaminoglycan accumulation.
  • Hurler-Scheie syndrome (MPS I), a subtype of MPS, involves a deficiency in the enzyme α-L-iduronidase.
  • Cardiac valvular abnormalities are a known complication of MPS I due to progressive glycosaminoglycan storage.

Observation:

  • This report details an unusual case of a double-valve replacement in an MPS I patient.
  • The patient experienced early infective endocarditis, necessitating surgical reintervention.
  • Few surgical heart valve interventions have been documented in MPS I patients.

Findings:

  • The case illustrates the potential complications, such as infective endocarditis, following valve surgery in MPS I patients.
  • A comprehensive literature review on valve surgery in MPS I patients is presented.
  • Key surgical considerations, including valve selection and infection prevention strategies, are summarized.

Implications:

  • This study underscores the complex management of cardiac issues in MPS I patients.
  • It emphasizes the need for careful surgical planning and vigilant post-operative care.
  • The findings contribute to understanding and improving outcomes for valve interventions in this rare genetic disorder.

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