MRI characterization of myocardial tissue in patients with Fabry's disease.
OBJECTIVE
Fabry's disease is a multisystem X-linked disorder of lysosomal metabolism frequently associated with left ventricular (LV) hypertrophy. In this study, we aimed to assess whether myocardial T2 relaxation time determined by a black blood multiecho multishot MRI sequence could be used to evaluate cardiac involvement in patients with Fabry's disease.


CONCLUSIONS
Myocardial T2 relaxation time is prolonged in patients with Fabry's disease compared with that of hypertrophic patients and healthy control subjects. MRI may be useful for the characterization of myocardial tissue in patients with Fabry's disease.
Imbriaco
Massimo
M
Department of Radiology, University Federico II, Via Pansini 5, Via Posillipo 196, Napoli 80123, Italy. mimbriaco@hotmail.com
Spinelli
Letizia
L
Cuocolo
Alberto
A
Maurea
Simone
S
Sica
Giacomo
G
Quarantelli
Mario
M
Pisani
Antonio
A
Liuzzi
Raffaele
R
Cianciaruso
Bruno
B
Sabbatini
Massimo
M
Salvatore
Marco
M
D000293:Adolescent; D000328:Adult; D009202:Cardiomyopathies / Q000150:complications* / Q000473:pathology*; D000795:Fabry Disease / Q000150:complications* / Q000473:pathology*; D005240:Feasibility Studies; D005260:Female; D006801:Humans; D008279:Magnetic Resonance Imaging / Q000379:methods*; D008297:Male; D008875:Middle Aged