The histological basis of late gadolinium enhancement cardiovascular magnetic resonance in a patient with Anderson-Fabry disease

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Moon, J. C. ; Sheppard, M. ; Reed, E. ; Lee, P. ; Elliott, P. M. ; Pennell, D. J. (2006)
  • Publisher: TAYLOR & FRANCIS INC
  • Subject: 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.
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