The histological basis of late gadolinium enhancement cardiovascular magnetic resonance in a patient with Anderson-Fabry disease.
Anderson-Fabry Disease (AFD) is a storage disease that mimics hypertrophic cardiomyopathy. Late gadolinium enhancement (LGE) by cardiovascular magnetic resonance occurs in approximately 50% of patients in the basal inferolateral LV wall, but how an intracellular storage disease causes focal LGE is unknown. We present a whole-heart histological validation that LGE is caused by focal myocardial collagen scarring. This scarring may be the substrate for electrical re-entry and sudden arrhythmic death. The reasons for this distribution of fibrosis are unclear, but may reflect inhomogeneous left ventricular wall stress.
Moon
James C
JC
CMR Unit, Royal Brompton Hospital, London, United Kingdom. James@moonmail.co.uk
Sheppard
Mary
M
Reed
Emma
E
Lee
Phillip
P
Elliott
Perry M
PM
Pennell
Dudley J
DJ
D000704:Analysis of Variance; D002318:Cardiovascular Diseases / Q000473:pathology*; D003287:Contrast Media; D000795:Fabry Disease / Q000473:pathology*; D017809:Fatal Outcome; D019786:Gadolinium DTPA; D006801:Humans; D019028:Magnetic Resonance Imaging, Cine; D008875:Middle Aged; D018709:Statistics, Nonparametric
D003287:Contrast Media; D019786:Gadolinium DTPA