Journal article
Intravascular ultrasound assessment of coronary artery involvement in Fabry disease
Journal of inherited metabolic disease, Vol.31(6), pp.753-760
12/2008
DOI: 10.1007/s10545-008-0794-0
PMID: 18998239
Abstract
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.
Details
- Title: Subtitle
- Intravascular ultrasound assessment of coronary artery involvement in Fabry disease
- Creators
- T. Kovarnik - Charles UniversityG. S. Mintz - Cardiovascular Research FoundationD. Karetova - Charles UniversityJ. Horak - Charles UniversityJ. Bultas - Charles UniversityR. Skulec - Charles UniversityH. Skalicka - Charles UniversityM. Aschermann - Charles UniversityM. Elleder - Charles UniversityA. Linhart - Charles University
- Resource Type
- Journal article
- Publication Details
- Journal of inherited metabolic disease, Vol.31(6), pp.753-760
- Publisher
- Wiley
- DOI
- 10.1007/s10545-008-0794-0
- PMID
- 18998239
- ISSN
- 0141-8955
- eISSN
- 1573-2665
- Number of pages
- 8
- Grant note
- VZ-MSM 0021620806 / Ministry of Education, Youth and Physical Education of the Czech Republic; Ministry of Education, Youth & Sports - Czech Republic MSM 0021620817 / Charles University Prague Research
- Language
- English
- Date published
- 12/2008
- Academic Unit
- Electrical and Computer Engineering
- Record Identifier
- 9984627306302771
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