2019
DOI: 10.1002/ehf2.12518
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Transthyretin cardiac amyloidosis: an update on diagnosis and treatment

Abstract: Transthyretin cardiac amyloidosis (ATTR-CA) demonstrates progressive, potentially fatal, and infiltrative cardiomyopathy caused by extracellular deposition of transthyretin-derived insoluble amyloid fibrils in the myocardium. Two distinct types of transthyretin (wild type or variant) become unstable, and misfolding forms aggregate, resulting in amyloid fibrils. ATTR-CA, which has previously been underrecognized and considered to be rare, has been increasingly recognized as a cause of heart failure with preserv… Show more

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Cited by 148 publications
(189 citation statements)
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References 99 publications
(160 reference statements)
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“…The lowest proportion of women (9%) was observed in studies of wild-type ATTR-CM, whereas the highest (29%) was observed in studies of hereditary ATTR-CM. These findings are consistent with previous reports that ATTR-CM is a condition predominantly identified in men [6,39], especially for the wild-type versus the hereditary subtype [6][7][8].…”
Section: Discussionsupporting
confidence: 93%
See 1 more Smart Citation
“…The lowest proportion of women (9%) was observed in studies of wild-type ATTR-CM, whereas the highest (29%) was observed in studies of hereditary ATTR-CM. These findings are consistent with previous reports that ATTR-CM is a condition predominantly identified in men [6,39], especially for the wild-type versus the hereditary subtype [6][7][8].…”
Section: Discussionsupporting
confidence: 93%
“…Among hospitalized patients with heart failure and preserved ejection fraction, older than 60 years of age, with left ventricular wall thickness of 12 mm or greater on scintigraphy, the prevalence of wild-type ATTR-CM was 13% [5]. The frequency of ATTR-CM, particularly the wild-type form, is reportedly lower in women than men [6][7][8][9][10][11], and the female sex has been Electronic supplementary material The online version of this article (https://doi.org/10.1007/s10741-020-10010-8) contains supplementary material, which is available to authorized users.…”
Section: Introductionmentioning
confidence: 99%
“…Except for myocardial thickening with granular sparkling by transthoracic echocardiography, the patient did not show any signs or symptoms suggesting TTR amyloidosis like a history of heart failure, arrhythmias, cardiac conduction system disease, carpal tunnel syndrome, lumbar spinal stenosis, biceps tendon rupture, or peripheral or autonomic neuropathy. 5,6 Granular sparkling is an echocardiographic finding that is not specific for amyloidosis but has also been reported in end-stage renal disease, hypertrophic cardiomyopathy, and Friedreich cardiomyopathy. 4,14,15 Myocardial thickening, furthermore, may be due to many different conditions and diseases.…”
Section: Discussionmentioning
confidence: 99%
“…At present, it is unknown how often cardiac TTR amyloidosis is erroneously diagnosed because physicians rely exclusively on echocardiographic and scintigraphic findings. Myocardial oedema with cardiac uptake of bone-avid radiotracers due to reperfused myocardial infarction is missing in current consensus papers, diagnostic algorithms, and stateof-the art reviews 1,3,5,6 and should be acknowledged as a differential diagnosis for cardiac TTR amyloidosis. In addition, a recent report demonstrated diffuse myocardial uptake of technetium-99m pyrophosphate in a patient with hydroxychloroquine-induced restrictive cardiomyopathy.…”
Section: Discussionmentioning
confidence: 99%
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