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Cardiovascular changes in mucopolysaccharidoses in Taiwan
Ming-Ren Chen1, Shuan-Pei Lin, Haw-Kwei Hwang
1Division of Cardiology, Mackay Memorial Hospital, Mackay Medicine, Nursing and Management College, Taipei, Taiwan. mingren@ms2.mmh.org.tw
Acta Cardiologica
|March 23, 2005
Summary
Mucopolysaccharidoses (MPS) patients often develop progressive cardiovascular abnormalities, including valve issues and septal hypertrophy. Regular cardiac evaluations are crucial for managing these patients as they live longer.
Area of Science:
- Cardiology
- Genetics
- Pediatrics
Background:
- Mucopolysaccharidoses (MPS) are a group of rare genetic disorders.
- Cardiovascular complications are a significant cause of morbidity and mortality in MPS.
- Long-term survival in MPS patients necessitates a better understanding of cardiac involvement.
Purpose of the Study:
- To retrospectively analyze cardiovascular findings in MPS patients.
- To describe the progression of cardiac abnormalities with age.
- To emphasize the importance of routine cardiac assessment in MPS management.
Main Methods:
- Retrospective review of 37 MPS patients' medical records (1991-2002).
- Analysis of physical findings, electrocardiography (ECG), and echocardiography data.
- Correlation of cardiac findings with patient age and MPS type.
Main Results:
- Cardiovascular abnormalities were common and progressed with age, despite mild clinical symptoms.
- Most frequent findings included mitral/aortic valve abnormalities and interventricular septal hypertrophy (especially in types I, II, VI).
- No arrhythmias were observed; one patient showed ischemic changes due to cardiomyopathy.
Conclusions:
- Cardiovascular disease is a progressive and significant concern in MPS.
- Regular and complete cardiac evaluations are essential for the routine management of MPS patients.
- Early detection and monitoring of cardiac issues can improve outcomes and survival rates.