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Cardiac amyloidosis: An Update on Pharmacotherapy

May 26, 2023 from 9:30am EDT to 10:00am EDT

Cardiac amyloidosis is associated with restrictive cardiomyopathy caused by the extracellular deposition of unstable proteins in the myocardium. These unstable proteins undergo misfolding, aggregation, and deposition as amyloid fibrils. Two mechanisms contribute to the genesis of unstable proteins. First, the abnormal clonal proliferation of plasma cells results in misfolding of the monoclonal immunoglobulin light chains causing Light-Chain (AL) Amyloidosis. Although light-Chain (AL) Amyloidosis is considered a hematologic malignancy, it is often investigated and diagnosed by a cardiologist as patients initially present with alterations in cardiac functions. Once the diagnosis of light-Chain (AL) Amyloidosis is confirmed, it is treated with anti-plasma cell chemotherapy, and the therapy is guided by oncologists. The second mechanism of protein misfolding results in transthyretin (TTR) amyloidosis (ATTR),  and is caused by abnormal protein synthesis in the liver that is typically involved in the transportation of the hormone thyroxine and retinol-binding protein. Since transthyretin amyloid cardiomyopathy (ATTR-CM) is diagnosed, treated, and followed up by cardiologists, it has paramount relevance to cardiovascular nurses. The purpose of this presentation is to review currently approved ATTR disease-modifying therapies and discuss approaches to ATTR monitoring and surveillance. We will also provide updated information regarding approved and in-development ATTR therapies and discuss current knowledge gaps regarding the use of ATTR therapies.

Speakers / Panelists