Setting the stage for the next step in cardiac amyloidosis imaging: Serial quantitative studies to assess disease activity

Excerpt

The term “amyloid” refers to misfolded proteins that tend to aggregate into fibrils, usually with a beta-pleated structural conformation.1 The latter imparts the characteristic apple-green birefringence when stained with Congo-red and viewed under polarized light. The term is attributed to Rudolf Virchow who used it to describe the observed “lardaceous” infiltration of tissues, which was incorrectly thought to be a substance derived from starch (from latin “amylum”).2 Due to already established widespread use, the nomenclature persisted even after the proteinaceous origin of amyloid was subsequently identified. The deposition of amyloid proteins is the basis of several recognized organ-specific and systemic amyloidosis syndromes, neurodegenerative disorders, and prion diseases.3 …