Fabry Disease

NB

usually indistinguishable from symmetric HCM

can be asymmetric

LGE

mostly in the basal inferolateral LV wall
mostly mid myocardial (mesocardial)
subendocardium mostly spared

FABRY DISEASE
“Delayed enhanced images of 64-year-old heterozygotic woman with Fabry’s disease–related cardiac hypertrophy. Minimal thinning of basal segment of left ventricular inferolateral wall is associated with thick mesocardial striae of delayed enhancement shown on short-axis delayed enhanced images.”
De Cobelli et al Delayed-Enhanced Cardiac MRI for Differentiation of Fabry’s Disease from Symmetric Hypertrophic Cardiomyopathy
AJR Volume 192, Issue 3 2009
LGE in FABRY DISEASE
“40-year-old man with Fabry’s disease–related hypertrophy. Long-axis delayed enhanced images show typical pattern of delayed enhancement: thick striae involving inferolateral wall of basal segment of left ventricle in mesocardial distribution. Note sparing of subendocardial layer.
De Cobelli et al Delayed-Enhanced Cardiac MRI for Differentiation of Fabry’s Disease from Symmetric Hypertrophic Cardiomyopathy”
AJR Volume 192, Issue 3 2009
LGE in FABRY DISEASE
“40-year-old man with Fabry’s disease–related hypertrophy. Short -axis delayed enhanced images show typical pattern of delayed enhancement: thick striae involving inferolateral wall of basal segment of left ventricle in mesocardial distribution. Note sparing of subendocardial layer.”
De Cobelli et al Delayed-Enhanced Cardiac MRI for Differentiation of Fabry’s Disease from Symmetric Hypertrophic Cardiomyopathy”
AJR Volume 192, Issue 3 2009

 

 

 

need endomyocardial biopsy

Read More: https://www.ajronline.org/doi/10.2214/AJR.08.1201

 

References and Links

Radiopaedia

Wiki

 De Cobelli  et al, Delayed-Enhanced Cardiac MRI for Differentiation of Fabry’s Disease from Symmetric Hypertrophic Cardiomyopathy  American Journal of Roentgenology  Volume 192, Issue 3 2009