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Cardiovascular abnormalities in Taiwanese patients with mucopolysaccharidosis

Shan-Miao Lin1, Hsiang-Yu Lin2, Chih-Kuang Chuang3

  • 1Department of Medicine, Mackay Medical College, New Taipei City, Taiwan; Department of Pediatrics, Mackay Memorial Hospital, Taipei, Taiwan; Mackay Junior College of Medicine, Nursing and Management, Taipei, Taiwan.

Molecular Genetics and Metabolism
|March 7, 2014
PubMed
Abstract

Insights

Mucopolysaccharidoses (MPS) patients frequently experience cardiovascular issues like thickened heart walls and valve problems. Early detection via echocardiography and electrocardiography is crucial for managing these inherited metabolic diseases.

Area of Science:

  • Cardiology
  • Genetics
  • Metabolic Disorders

Background:

  • Mucopolysaccharidoses (MPS) are rare inherited metabolic diseases.
  • MPS can lead to significant damage in various organs, including the heart.

Purpose of the Study:

  • To evaluate cardiovascular involvement in Taiwanese patients with MPS.
  • To analyze cardiac manifestations based on MPS type and patient age.

Main Methods:

  • Retrospective review of medical records for 60 MPS patients (2000-2012).
  • Analysis of echocardiogram and electrocardiogram data.
  • Cardiac parameters assessed by MPS type and age.

Main Results:

  • Type II was the most frequent MPS type (43%).
  • Common cardiac findings included thickened interventricular septum (55%), asymmetric septal hypertrophy (42%), and mitral valve prolapse (33%).
  • Valvular issues and cardiac hypertrophy were more prevalent in MPS I, II, and VI and progressed with age.

Conclusions:

  • Anatomical cardiovascular changes are common in all MPS types, particularly MPS I, II, and VI.
  • Cardiovascular abnormalities progress with age.
  • Echocardiography and electrocardiography are valuable tools for early detection and long-term follow-up of MPS patients.
Keywords:
CardiovascularEchocardiographyElectrocardiographyMucopolysaccharidosisTaiwan

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