Pathophysiology and Therapeutic Approaches to Cardiac Amyloidosis

Often considered a rare disease, cardiac amyloidosis is increasingly recognized by practicing clinicians. The increased rate of diagnosis is in part due the aging of the population and increasing incidence and prevalence of cardiac amyloidosis with advancing age, as well as the advent of noninvasive...

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Bibliographic Details
Published in:Circulation research 2021-05, Vol.128 (10), p.1554-1575
Main Authors: Griffin, Jan M., Rosenblum, Hannah, Maurer, Mathew S.
Format: Article
Language:English
Subjects:
Aging
Alkylating Agents - therapeutic use
Amyloid - chemistry
Amyloid - metabolism
Amyloid Neuropathies, Familial - diagnosis
Amyloidosis - diagnosis
Amyloidosis - etiology
Amyloidosis - physiopathology
Amyloidosis - therapy
Antibodies, Monoclonal - therapeutic use
Benzoates - therapeutic use
Benzoxazoles - therapeutic use
Bridged Bicyclo Compounds, Heterocyclic - therapeutic use
Cardiomyopathies - diagnosis
Cardiomyopathies - etiology
Cardiomyopathies - physiopathology
Cardiomyopathies - therapy
Catechol O-Methyltransferase Inhibitors - therapeutic use
Heart Transplantation
Humans
Immunomodulating Agents - therapeutic use
Oligonucleotides - therapeutic use
Proteasome Inhibitors - therapeutic use
Protein Folding
Pyrazoles - therapeutic use
RNA, Small Interfering - therapeutic use
Stem Cell Transplantation
Sulfonamides - therapeutic use
Tolcapone - therapeutic use
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Summary:Often considered a rare disease, cardiac amyloidosis is increasingly recognized by practicing clinicians. The increased rate of diagnosis is in part due the aging of the population and increasing incidence and prevalence of cardiac amyloidosis with advancing age, as well as the advent of noninvasive methods using nuclear scintigraphy to diagnose transthyretin cardiac amyloidosis due to either variant or wild type transthyretin without a biopsy. Perhaps the most important driver of the increased awareness is the elucidation of the biologic mechanisms underlying the pathogenesis of cardiac amyloidosis which have led to the development of several effective therapies with differing mechanisms of actions. In this review, the mechanisms underlying the pathogenesis of cardiac amyloidosis due to light chain (AL) or transthyretin (ATTR) amyloidosis are delineated as well as the rapidly evolving therapeutic landscape that has emerged from a better pathophysiologic understanding of disease development.
ISSN:0009-7330
1524-4571
DOI:10.1161/CIRCRESAHA.121.318187