The Last Decade in Cardiac Amyloidosis: Advances in Understanding Pathophysiology, Diagnosis and Quantification, Prognosis, Treatment Strategies, and Monitoring Response.

Abstract

Cardiac amyloidosis represents a unique disease process characterized by amyloid fibril deposition within the myocardial extracellular space. Advances in multimodality cardiac imaging enable accurate diagnosis and facilitate prompt initiation of disease-modifying therapies. Furthermore, rapid advances in multimodality imaging have enriched understanding of the underlying pathogenesis, enhanced prognostication, and resulted in the development of imaging-based markers that reflect the amyloid burden, which is of increasing importance when assessing the response to treatment. Whereas conventional therapies have focused on reducing amyloid formation and subsequent stabilization of the cardiac disease process, novel agents are being developed to accelerate the immune-mediated removal of amyloid fibrils from the heart. In this context, the ability to track changes in the amyloid burden over time is of paramount importance. Although advanced imaging techniques have shown efficacy in tracking the treatment response, future research focused on improved precision through use of artificial intelligence may augment the detection of changes earlier in the course of treatment.