Delayed-enhanced cardiac MRI for differentiation of Fabry's disease from symmetric hypertrophic cardiomyopathy.
OBJECTIVE
Fabry's disease may be difficult to differentiate from symmetric hypertrophic cardiomyopathy. Our aim was to compare the myocardial location and distribution patterns of delayed enhancement between patients with Fabry's disease who are affected by symmetric myocardial hypertrophy and patients with symmetric hypertrophic cardiomyopathy in order to identify a specific sign to best differentiate the two diseases.


CONCLUSIONS
Patients with Fabry's disease-related hypertrophy showed left ventricular (LV) delayed enhancement with a typical and consistently found pattern characterized by the involvement of the inferolateral basal or mid basal segments and a mesocardial distribution that spared the subendocardium. This pattern seems to be specific to Fabry's disease; in fact, patients with symmetric hypertrophic cardiomyopathy had variable locations and distributions of delayed enhancement. These observations may contribute to identifying Fabry's disease as a specific cause of symmetric hypertrophy.
De Cobelli
Francesco
F
Department of Radiology, San Raffaele Scientific Institute and Vita-Salute University, Via Olgettina 60, 20132, Milan, Italy. decobelli.francesco@hsr.it
Esposito
Antonio
A
Belloni
Elena
E
Pieroni
Maurizio
M
Perseghin
Gianluca
G
Chimenti
Cristina
C
Frustaci
Andrea
A
Del Maschio
Alessandro
A
D002312:Cardiomyopathy, Hypertrophic / Q000175:diagnosis* / Q000503:physiopathology; D003287:Contrast Media; D003937:Diagnosis, Differential; D000795:Fabry Disease / Q000175:diagnosis* / Q000503:physiopathology; D005260:Female; D019786:Gadolinium DTPA; D006801:Humans; D007090:Image Interpretation, Computer-Assisted; D008279:Magnetic Resonance Imaging / Q000379:methods*; D008297:Male; D008875:Middle Aged
D003287:Contrast Media; D019786:Gadolinium DTPA