Amyloid and the cardiovascular system: A review of pathogenesis and pathology with clinical correlations

The process of amyloidogenesis may complicate diverse disease states. It may be systemic and have serious clinical sequelae; in other circumstances, it is a localized phenomenon and functionally insignificant. In many cases its manifestations may be predictable, with knowledge of the responsible protein. Perhaps no organ system better exemplifies this than the cardiovascular one in which amyloid may form from precursor proteins as varied as immunoglobulin light chains, serum amyloid-A protein, transthyretin and its variants, atrial natriuretic factor, β2-microglobulin, and others. This review describes the state of knowledge in relation to cardiovascular amyloidosis, with particular emphasis on what is currently known about the pathogenesis of the process and the related pathology of the various anatomic components of the cardiovascular system.