Transthyretin Amyloid Cardiomyopathy: JACC State-of-the-Art Review

Transthyretin amyloid cardiomyopathy (ATTR-CM) is an under-recognized cause of heart failure (HF) in older adults, resulting from myocardial deposition of misfolded transthyretin (TTR) or pre-albumin. Characteristic patterns of echocardiography and cardiac magnetic resonance can strongly suggest the...

Full description

Saved in:
Bibliographic Details
Published in:Journal of the American College of Cardiology 2019-06, Vol.73 (22), p.2872-2891
Main Authors: Ruberg, Frederick L, Grogan, Martha, Hanna, Mazen, Kelly, Jeffery W, Maurer, Mathew S
Format: Article
Language:English
Subjects:
Adult
Adults
African Americans
Aged
Aged, 80 and over
Aging
Amino acids
Amyloid
Amyloid Neuropathies, Familial - diagnosis
Amyloid Neuropathies, Familial - genetics
Amyloidosis
Biomarkers
Cardiology
Cardiomyopathies - diagnosis
Cardiomyopathies - genetics
Cardiomyopathy
Carpal tunnel syndrome
Congestive heart failure
Diagnosis
Diagnostic systems
DNA Mutational Analysis
Echocardiography
Female
Genotype
Genotype & phenotype
Heart
Heart Failure - diagnosis
Heart Failure - genetics
Humans
Magnetic resonance
Magnetic Resonance Imaging
Male
Medical prognosis
Middle Aged
Mutation
Older people
Overuse injuries
Peptides
Prealbumin - genetics
Prognosis
Proteins
Radionuclide Imaging
Regulatory approval
Spinal stenosis
Transplantation
Transthyretin
Vitamin A
Online Access:Get full text
Tags: Add Tag
No Tags, Be the first to tag this record!
Description
Summary:Transthyretin amyloid cardiomyopathy (ATTR-CM) is an under-recognized cause of heart failure (HF) in older adults, resulting from myocardial deposition of misfolded transthyretin (TTR) or pre-albumin. Characteristic patterns of echocardiography and cardiac magnetic resonance can strongly suggest the disease but are not diagnostic. The diagnosis can be made with noninvasive nuclear imaging when there is no evidence of a monoclonal protein. Amyloid fibril formation results from a destabilizing mutation in hereditary ATTR amyloidosis (hATTR) or from an aging-linked process in wild-type ATTR amyloidosis (wtATTR). Recent studies have suggested that up to 10% to 15% of older adults with HF may have unrecognized wtATTR. Associated features, including carpal tunnel syndrome and lumbar spinal stenosis, raise suspicion and may afford a means for early diagnosis. Previously treatable only by organ transplantation, pharmaceutical therapy that slows or halts ATTR-CM progression and favorably affects clinical outcomes is now available. Early recognition remains essential to afford the best treatment efficacy.
ISSN:0735-1097
1558-3597
1558-3597
DOI:10.1016/j.jacc.2019.04.003