Pathophysiology and Therapeutic Approaches to Cardiac Amyloidosis.

Published on May 14, 2021in Circulation Research14.467
· DOI :10.1161/CIRCRESAHA.121.318187
Jan M. Griffin4
Estimated H-index: 4
(Columbia University),
Hannah Rosenblum5
Estimated H-index: 5
(Columbia University),
Mathew S. Maurer65
Estimated H-index: 65
(Columbia University)
Sources
Abstract
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.
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8 CitationsSource
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4 CitationsSource
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17 CitationsSource
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Background: In patients with transthyretin amyloid cardiomyopathy (ATTR-CM), tafamidis reduces all-cause mortality and cardiovascular hospitalizations, and slows decline in quality-oflife compared ...
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#1Haruki Koike (Nagoya University)H-Index: 44
#2Masahisa Katsuno (Nagoya University)H-Index: 66
Amyloidosis is a group of diseases that includes Alzheimer’s disease, prion diseases, transthyretin (ATTR) amyloidosis, and immunoglobulin light chain (AL) amyloidosis. The mechanism of organ dysfunction resulting from amyloidosis has been a topic of debate. This review focuses on the ultrastructure of tissue damage resulting from amyloid deposition and therapeutic insights based on the pathophysiology of amyloidosis. Studies of nerve biopsy or cardiac autopsy specimens from patients with ATTR a...
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#1Douglas L. Mann (WashU: Washington University in St. Louis)H-Index: 114
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