The histological basis of late gadolinium enhancement cardiovascular magnetic resonance in a patient with Anderson-Fabry disease
Moon, J. C.
Elliott, P. M.
Pennell, D. J.
- Publisher: TAYLOR & FRANCIS INC
cardiomyopathy, hypertrophy, magnetic resonance imaging, Anderson-Fabry disease, ONSET HYPERTROPHIC CARDIOMYOPATHY, PREVALENCE
mesheuropmc: cardiovascular diseases
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.