Intravascular ultrasound assessment of coronary artery involvement in Fabry disease
Language English Country United States Media print-electronic
Document type Journal Article, Research Support, Non-U.S. Gov't
- MeSH
- Endothelium, Vascular pathology MeSH
- Fabry Disease complications diagnosis diagnostic imaging MeSH
- Fibroblasts metabolism MeSH
- Coronary Angiography methods MeSH
- Coronary Vessels diagnostic imaging pathology MeSH
- Middle Aged MeSH
- Humans MeSH
- Coronary Artery Disease complications diagnosis diagnostic imaging MeSH
- Aged MeSH
- Case-Control Studies MeSH
- Trihexosylceramides metabolism MeSH
- Ultrasonography MeSH
- Check Tag
- Middle Aged MeSH
- Humans MeSH
- Male MeSH
- Aged MeSH
- Female MeSH
- Publication type
- Journal Article MeSH
- Research Support, Non-U.S. Gov't MeSH
- Names of Substances
- globotriaosylceramide MeSH Browser
- Trihexosylceramides MeSH
AIM: We used intravascular ultrasound (IVUS) to characterize coronary artery involvement in patients with Fabry disease (FD). METHODS: Nine FD patients (5 women) were matched to 10 control patients (5 women) chosen from our IVUS database. Standard volumetric IVUS analyses were performed along with assessment of plaque echodensity. RESULTS: Plaques in FD patients were diffuse and hypoechogenic compared with more focal and more echogenic lesions in control patients. Echogenicity of plaques was significantly lower in FD patients (median 30.7 +/- 12.9 vs 55.9 +/- 15.7, p = 0.0052, mean 37.2 +/- 15.6 vs 66.2 +/- 13.3, p = 0.0014). Diffusiveness was assessed as differences between mean and median plaque burden versus the plaque burden in each of the analysed cross-sections. These differences were lower in FD vs controls (5.8 +/- 4.8 vs 8.7 +/- 6.6, p < 0.001 for mean, and 5.8 +/- 4.9 vs 8.8 +/- 7.3, p < 0.001 for median) indicating a more diffuse involvement. The occurrence of lipid cores was significantly higher in FD patients than in controls (2.4 +/- 1.5 vs 1.0 +/- 0.94, p = 0.02). CONCLUSION: IVUS showed diffuse hypoechogenic plaques in patients with FD. The explanation may be higher lipid content in plaques and accumulation of glycosphingolipid in smooth-muscle and endothelial cells.
2nd Department of Internal Medicine 1st Faculty of Medicine Charles University Prague Czech Republic
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