[Mitral stenosis secondary to Hurler's syndrome]

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Left Atrial Stenosis Induced Pulmonary Venous Arterialization and Group 2 Pulmonary Hypertension in Rat
08:34

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Published on: November 18, 2018

S Kraiem1, D Lahidheb, N Chehaibi

  • 1Service de cardiologie, hôpital Habib Thameur, 8, av. Ali Ben Ayed, Monfleury, Tunis 1008, Tunisie.

Archives Des Maladies Du Coeur Et Des Vaisseaux
|March 27, 2001
PubMed

Insights

Mitral stenosis is a rare complication of Hurler syndrome (mucopolysaccharidosis type I-Hurler). This case highlights mucopolysaccharide deposits causing severe mitral stenosis, necessitating valve replacement in a 16-year-old patient.

Area of Science:

  • Cardiology
  • Genetics
  • Pediatrics

Background:

  • Valvular disease is common in mucopolysaccharidosis type I-Hurler (MPS I-H).
  • Severe mitral stenosis is an exceptionally rare manifestation of MPS I-H.
  • Early diagnosis and characteristic morphological features are crucial for managing MPS I-H.

Observation:

  • A 16-year-old female with Hurler syndrome, diagnosed at age 4, presented with dyspnea.
  • Morphological features included gargoylism, macroglossia, and limited joint flexion.
  • Severe mitral stenosis with hemodynamic compromise was identified via imaging.

Findings:

  • The patient required mitral valve replacement due to severe mitral stenosis.
  • Anatomopathological examination confirmed mucopolysaccharide deposition as the etiology.
  • This case underscores the potential for rare cardiac complications in MPS I-H.

Implications:

  • Highlights the importance of cardiac monitoring in MPS I-H patients, even with rare presentations.
  • Suggests mucopolysaccharide deposition can lead to severe valvular dysfunction.
  • Informs clinical management strategies for Hurler syndrome patients with cardiac involvement.

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