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Vasospastic angina in a patient with Fabry's disease who showed normal coronary angiographic findings
1Department of Internal Medicine (IV), Jikei University School of Medicine, Tokyo, Japan.
Insights
Fabry
Area of Science:
- Cardiology
- Genetics
- Cell Biology
Background:
- Coronary artery disease in Fabry's disease is typically linked to cellular deposits causing stenosis.
- Understanding the mechanisms of cardiac involvement in Fabry's disease is crucial for patient management.
Observation:
- A case study of a Fabry's disease patient revealed severe coronary vasospasms during angiography.
- These vasospasms occurred without evidence of coronary artery stenosis.
- Myocardial biopsy showed characteristic lamellated deposits within endothelial cells of myocardial capillaries.
Findings:
- Glyco-sphingolipid deposits in coronary endothelial cells may induce functional disorders.
- Endothelial cell dysfunction, rather than organic stenosis, can precipitate coronary artery spasms.
Implications:
- This case highlights a potential mechanism for coronary vasospasm in Fabry's disease.
- It suggests that endothelial cell dysfunction plays a key role in cardiac manifestations.
- Further research into functional cardiac abnormalities in Fabry's disease is warranted.
Abstract:
It has been reported that coronary diseases in patients with Fabry's disease are induced by deposits in endothelial cells and coronary smooth muscle cells. Most of those are ischemia due to stenosis. This report describes a case of patient with Fabry's disease who showed severe vasospasms without coronary artery stenosis during acetylcholine loaded coronary angiography. In this case, a myocardial biopsy revealed that the deposits in the endothelial cells of the myocardial capillaries were lamellated appearance. Recently, it is reported that endothelial cell damage could be an important cause of coronary vasospasm. This case suggests that the some sort of functional disorder was induced by glyco-sphingolipid deposits in the coronary endothelial cells, and that this might have led to coronary artery spasms without the organic stenosis of coronary arteries.