Intravascular ultrasound assessment of coronary artery involvement in Fabry disease.
OBJECTIVE
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).


CONCLUSIONS
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.
Kovarnik
T
T
Second Department of Internal Medicine, First Faculty of Medicine, Charles University, Prague, Czech Republic. tkovarnik@yahoo.com
Mintz
G S
GS
Karetova
D
D
Horak
J
J
Bultas
J
J
Skulec
R
R
Skalicka
H
H
Aschermann
M
M
Elleder
M
M
Linhart
A
A
D000368:Aged; D016022:Case-Control Studies; D017023:Coronary Angiography / Q000379:methods; D003324:Coronary Artery Disease / Q000150:complications / Q000175:diagnosis* / Q000000981:diagnostic imaging*; D003331:Coronary Vessels / Q000000981:diagnostic imaging* / Q000473:pathology*; D004730:Endothelium, Vascular / Q000473:pathology; D000795:Fabry Disease / Q000150:complications / Q000175:diagnosis* / Q000000981:diagnostic imaging*; D005260:Female; D005347:Fibroblasts / Q000378:metabolism; D006801:Humans; D008297:Male; D008875:Middle Aged; D014281:Trihexosylceramides / Q000378:metabolism; D014463:Ultrasonography
D014281:Trihexosylceramides; C018549:globotriaosylceramide