Amyloid transthyretin cardiomyopathy

Abstract:

Amyloidosis is an infiltrative disease affecting different organ systems, with worst outcomes in patients with amyloid cardiomyopathy. Transthyretin amyloidosis is characterized by deposition of transthyretin tetramer as a result of gene mutation or ageing process. Transthyretin deposits in the heart result in severe thickening of myocardium, conduction tissue disturbances, and lead to symptoms and signs of heart failure. Recently, the accuracy and efficacy of non-invasive imaging methods in diagnosis of transthyretin amyloid cardiomyopathy has been rising, allowing for easier and faster diagnosis. Great advancements have also been achieved in the development of disease-modifying agents for patients with transthyretin amyloid cardiomyopathy.

Key words:
amyloidosis; cardiomyopathy; heart failure; radionuclide imaging; tafamidis