Transthyretin Cardiac Amyloidosis Evaluation and Management: 2025 ACC Concise Clinical Guidance.

Abstract

Transthyretin amyloid cardiomyopathy has emerged as an increasingly recognized cause of heart failure, particularly in older individuals. There is now greater awareness of transthyretin amyloid cardiomyopathy as an underlying etiology of heart failure, particularly in individuals with musculoskeletal manifestations such as bilateral carpal tunnel syndrome or spinal stenosis. There have also been substantial advances in diagnosis, including the ability to perform accurate noninvasive diagnosis using radionuclide scintigraphy in individuals with a negative monoclonal protein screen. Finally, individuals with transthyretin amyloid cardiomyopathy have benefitted from advances in broadly effective heart failure therapies, namely mineralocorticoid receptor antagonists and sodium glucose-cotransporter 2 inhibitors, as well as specific disease-modifying therapies with transthyretin stabilizers, tafamidis and acoramidis, and the transthyretin silencer vutrisiran. The purpose of this Concise Clinical Guidance is to offer updated strategies to clinicians, reflecting the expanding therapeutic landscape, and reinforcing best practices for the diagnosis and management of transthyretin amyloid cardiomyopathy with a focus on choice of disease-modifying therapies, heart failure therapies, and future directions.