Diagnostic Modalities in the Detection of Cardiac Amyloidosis

Cardiac amyloidosis (CA) results mainly from the infiltration of the myocardium by either immunoglobulin light-chain fibrils (AL) or transthyretin fibrils (ATTR), causing restrictive cardiomyopathy and eventually death if untreated. AL derives from monoclonal immunoglobulin light chains produced by plasma cell clones in the bone marrow, while ATTR is the misfolded form of hepatically derived transthyretin (TTR) protein and can be hereditary (ATTRv) or wild-type (ATTRwt). Over the last decade, improvements in diagnostic imaging and better clinical awareness have unleashed a notable presence of CA in the community, especially ATTR in the elderly population. These multimodality imaging modalities include echocardiography, cardiac magnetic resonance, and radionuclide scintigraphy with bone-avid tracers. There has been remarkable progress in the therapeutic landscape as well, and there are disease-modifying therapies available now that can alter the course of the disease and improve survival if initiated at an early stage of the disease. There remains an unmet need for detecting this disease accurately and early so that these patients can benefit the most from newly emerging therapies.