LAUSR

Cardiac amyloidosis (CA) is an uncommon form of infiltrative cardiomyopathy that presents with progressive heart failure (HF) and has a poor prognosis. The disease process is characterized by the extracellular deposition of amyloid fibril deposits which typically are either monoclonal light chain (AL) or transthyretin (ATTR) proteins. Progressive increases in wall thickness are associated with greater impairment of left ventricular function and can be identified with echocardiography or cardiac magnetic resonance imaging (CMRI). However, the clinical diagnosis may be missed due to the lack of specificity of these structural changes or delayed pending confirmatory tissue biopsy. Clinical presentation usually includes symptoms of heart failure but may include angina which has been attributed to small vessel disease. New therapies are being developed for ATTR amyloidosis and include micro-RNA inhibitors that interrupt the production of amyloid proteins and molecular stabilizers of the transthyretin tetramer such as tafamidis. Although imaging with Tc-labeled bone tracers has been described for imaging CA for many years, recent studies have demonstrated high diagnostic accuracy for ATTR CA. Early and accurate diagnosis has increasing clinical importance with the possibility of these new and specific treatments.