Cardiac Amyloidosis: Multimodal Imaging of Disease Activity and Response to Treatment. (16th June 2021)

• Record Type: Journal Article
• Authors: Patel, Rishi K.; Fontana, Marianna; Ruberg, Frederick L.
• Is Part Of: Circulation. Volume 14:Number 6(2021)
• Page Start: e009025
• Publication Date: 2021-06-16
• Subjects: amyloidosis -- echocardiography -- morbidity -- multimodal imaging -- prognosis; Cardiovascular system -- Imaging -- Periodicals; Heart -- Imaging -- Periodicals; 616.1075405
• DOI: 10.1161/CIRCIMAGING.121.009025
• Languages: English
• ISSNs: 1941-9651

Abstract

Abstract : Cardiac amyloidosis (CA) is a disease characterized by the deposition of misfolded protein deposits in the myocardial interstitium. Although advanced CA confers significant morbidity and mortality, the magnitude of deposition and ensuing clinical manifestations vary greatly. Thus, an improved understanding of disease pathogenesis at both cellular and functional levels would afford critical insights that may improve outcomes. This review will summarize contemporary therapies for the 2 major types of CA, transthyretin and light chain amyloidosis, and outline the capacity of imaging modalities to both diagnose CA, inform prognosis, and follow response to available therapies. We explore the current landscape of echocardiography, cardiac magnetic resonance, and bone scintigraphy in the assessment of functional and cellular parameters of dysfunction in CA throughout disease pathogenesis. Finally, we examine the impact of concurrent advances in both therapeutics and imaging on future research questions that improve our understanding of underlying disease mechanisms. Multimodal imaging in CA affords an indispensable tool to offer individualized treatment plans and improve outcomes in patients with CA.