Cardiac Amyloidosis: Typing, Diagnosis, Prognosis and Management

Amyloidosis is uncommon, with age-adjusted incidences of between 6.1 and 10.5 per million person-years,[1] and an estimated 1275 to 3200 new cases occurring annually in the United States.[1, 2] The contemporary understanding of amyloidosis points to a group of complex sys‐ temic disorders involving the extracellular deposition of misfolded proteinaceous material in many organs, most commonly the kidneys, heart, liver, central and peripheral nervous sys‐ tems.[2-4] The normal function of tissues is altered, and end-organ dysfunction usually ensues. Cardiac amyloidosis can be isolated to the heart, but it often coexists with disease elsewhere in the body.[4, 5] Cardiac manifestations may predominate the clinical presentation or may be subclinical and detected on routine investigation of a patient presenting with non-cardiac com‐ plaints.[5] The presence and relative prominence of cardiac involvement in the clinical picture is dependent on the type of amyloidosis and severity of amyloid infiltration in the tissue.[5]