ABC Heart Fail Cardiomyop 2021; 1(2): 84-85

ATTRv: a Multisystemic Disease Requires a Multiprofessional Approach

Carolina Lavigne Moreira, Wilson Marques Júnior

DOI: 10.36660/abchf.20210026

Pathogenic variants of the transtiretin gene (TTRv) result in the production of amyloidogenic fibrils that deposit in the extracellular space of many tissues, resulting in amyloidogenic multisystemic disease (ATTRv). Several tissues may be affected in different combinations, including the peripheral nerves and the cardiac system, which are usually the main targets, but also the kidneys, eyes, gastrointestinal system, central nervous system and musculoskeletal structures.

ATTRv is a progressive disease that results in death around 8 to 15 years after the onset of the disease, even more rapidly when the cardiac system is involved early. However, the natural history of the disease has changed with the introduction of treatments that, by different mechanisms, decrease the production of amyloid fibrils.

[…]