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

J Cardiovasc Magn Reson. 2006;8(3):479-82. doi: 10.1080/10976640600605002.


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.

Publication types

  • Case Reports
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Analysis of Variance
  • Cardiovascular Diseases / pathology*
  • Contrast Media
  • Fabry Disease / pathology*
  • Fatal Outcome
  • Gadolinium DTPA
  • Humans
  • Magnetic Resonance Imaging, Cine*
  • Middle Aged
  • Statistics, Nonparametric


  • Contrast Media
  • Gadolinium DTPA