Amyloid in the cardiovascular system: a review

Abstract

The cardiovascular system is a common target of amyloidosis. This review presents the current clinical and diagnostic approach to amyloidosis, with the emphasis on cardiovascular involvement. It summarises recent nomenclature, classification, and pathogenesis of amyloidosis. In addition, non-invasive possibilities are discussed, together with endomyocardial biopsies in the diagnosis of cardiac amyloidosis. Finally, recent advances in treatment and prognostic implications are presented.

Figure 1

Example of a heart with amyloidosis. (A) Macroscopic view showing nodules with amyloid (arrows). (B) Microscopic view showing eosinophilic areas containing amyloid (asterisks); original magnification, ×100. (C) Microscopic view showing eosinophilic bands, containing amyloid, around individual cardiomyocytes (arrows); original magnification, ×100. (D) Microscopic view (same figure as C) showing apple green birefringance under polarised light; original magnification, ×100. (E) An intramyocardial artery showing Congo red stained amyloid (arrow); original magnification, ×400. (F) An intramyocardial artery (same figure as E) showing apple green birefringance under polarised light; original magnification, ×400. (G) Amyloidosis is characterised by the deposition of fine fibrillar material (asterisk) in and around cardiomyocytes (arrow); original magnification, ×10 000. (H) Amyloidosis is characterised by the deposition of fine fibrillar material (asterisk) around small vessels; arrow, endothelial cell; original magnification, ×15 000.