title: The histological basis of late gadolinium enhancement cardiovascular magnetic resonance in a patient with Anderson-Fabry disease.
abstract: 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.
authors.affiliation: CMR Unit, Royal Brompton Hospital, London, United Kingdom. James@moonmail.co.uk
authors.forename: James C
authors.initials: JC
authors.lastname: Moon
authors.forename: Mary
authors.initials: M
authors.lastname: Sheppard
authors.forename: Emma
authors.initials: E
authors.lastname: Reed
authors.forename: Phillip
authors.initials: P
authors.lastname: Lee
authors.forename: Perry M
authors.initials: PM
authors.lastname: Elliott
authors.forename: Dudley J
authors.initials: DJ
authors.lastname: Pennell
mesh_terms: 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
chemical_list: D003287:Contrast Media; D019786:Gadolinium DTPA