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Clinical characterization of cardiovascular abnormalities associated with feline mucopolysaccharidosis I and VI
M M Sleeper1, C M Kusiak, F S Shofer
1Section of Cardiology, Department of Clinical Studies, University of Pennsylvania School of Veterinary Medicine, Philadelphia, PA, USA. sleeper@vet.upenn.edu
Insights
Cardiovascular abnormalities like aortic and mitral valve thickening were found in cats with mucopolysaccharidosis (MPS) I and VI. These feline models show progressive cardiac changes with age, similar to human MPS patients.
Area of Science:
- Veterinary Cardiology
- Genetics
- Lysosomal Storage Diseases
Background:
- Lysosomal storage diseases (LSDs) are inherited metabolic disorders.
- Mucopolysaccharidosis (MPS) I and MPS VI are LSDs affecting multiple organs, including the cardiovascular system.
- Understanding cardiovascular manifestations in affected animals is crucial for disease management and therapeutic development.
Purpose of the Study:
- To characterize cardiovascular abnormalities in cats with MPS I and MPS VI.
- To assess the impact of age on the severity of cardiac lesions in these feline models.
- To compare cardiovascular findings in affected cats with those in human MPS patients.
Main Methods:
- Physical examination, electrocardiography (ECG), and echocardiography were performed on 18 cats with MPS I and 10 cats with MPS VI.
- A control group of 12 unaffected cats underwent similar comprehensive examinations.
- ECG was conducted on all MPS I cats and 9 MPS VI cats.
Main Results:
- No cardiovascular abnormalities were detected during physical examinations.
- While ECG intervals were normal, sinus arrhythmia was more prevalent in affected cats.
- Echocardiography revealed significant aortic valve thickening, regurgitation, aortic root dilation, and mitral valve thickening.
- The severity of these cardiac lesions increased with age in affected cats.
Conclusions:
- Cats with MPS I and MPS VI exhibit progressive cardiovascular abnormalities, primarily affecting the aortic and mitral valves and the ascending aorta.
- Myocardial changes were not observed in the studied population.
- These feline models demonstrate cardiovascular findings analogous to those in human MPS patients, making them valuable for preclinical therapeutic testing.
Objective:
The purpose of this study was to define the cardiovascular abnormalities present in young and adult cats affected with the lysosomal storage diseases mucopolysaccharidosis (MPS) I and MPS VI.
Method:
Eighteen cats affected with MPS I and 10 cats affected with MPS VI were evaluated by physical examination, electrocardiography and echocardiography. Electrocardiography (ECG) was performed on all MPS I and 9 of the MPS VI cats. Twelve unaffected cats underwent complete examinations for comparison purposes.
Results:
No cardiovascular abnormalities were noted on physical examination. Measured ECG intervals were normal in affected cats; however, sinus arrhythmia was noted more frequently than in the unaffected cats. Significant echocardiographic abnormalities included aortic valve thickening, regurgitation and aortic root dilation. Significant mitral valve thickening was also noted. The severity of changes increased in older affected cats.
Conclusion:
As affected animals increased in age, more cardiac abnormalities were found with increasing severity. Significant lesions included the mitral and aortic valves and ascending aorta, but myocardial changes were not recognized. MPS I and MPS VI cats have similar cardiovascular findings to those seen in children and constitute important models for testing new MPS therapies.
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