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Cited by 345

The DOI matches its Crossref record; 24 of the 24 references it cites were found in a registry or an index.

Left Ventricular Structure and Function in Transthyretin-Related Versus Light-Chain Cardiac Amyloidosis

Open access
Quarta, Candida Cristina, Solomon, Scott D., Uraizee, Imran, Kruger, Jenna, Longhi, Simone, Ferlito, Marinella, Gagliardi, Christian, Milandri, Agnese, Rapezzi, Claudio, Falk, Rodney H.·2014·Circulation

Abstract

Background—: Immunoglobulin amyloid light-chain (AL)-related cardiac amyloidosis (CA) has a worse prognosis than either wild-type (ATTRwt) or mutant (ATTRm) transthyretin (TTR) CA. Detailed echocardiographic studies have been performed in AL amyloidosis but not in TTR amyloidosis and might give insight into this difference. We assessed cardiac structure and function and outcome in a large population of patients with CA and compared findings in TTR and AL-related disease. Methods and Results—: We analyzed 172 patients with CA (AL amyloidosis, n=80; ATTRm, n=36; ATTRwt, n=56) by standard echocardiography and 2-dimensional speckle-tracking imaging-derived left ventricular (LV) longitudinal (LS), radial, and circumferential strains. Despite a preserved LV ejection fraction (55±12%), LS was severely impaired in CA. Standard measures of LV function and speckle-tracking imaging worsened as wall thickness increased, whereas apical LS was preserved regardless of the pathogenesis of CA and the degree of wall thickening. Compared with ATTRm and AL amyloidosis, ATTRwt was characterized by greater LV wall thickness and lower ejection fraction. LS was more depressed in both ATTRwt and AL amyloidosis (−11±3% and −12±4%, respectively, P =0.54) than in ATTRm (−15±4%, P <0.01 versus AL amyloidosis and ATTRwt). TTR-related causes were favorable predictors of survival, whereas LS and advanced New York Heart Association class were negative predictors. Conclusions—: In patients with CA, worsening LV function correlated with increasing wall thickness regardless of pathogenesis. Patients with ATTRwt had a statistically greater wall thickness but lesser mortality than those with AL amyloidosis, despite very similar degrees of LS impairment. This paradox suggests an additional mechanism for LV dysfunction in AL amyloidosis, such as previously demonstrated light-chain toxicity.

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Crossref · 10.1161/circulationaha.113.006242

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Metadata

Authors
Quarta, C. C., Solomon, S. D., Uraizee, I., Kruger, J., Longhi, S., Ferlito, M., Gagliardi, C., Milandri, A., Rapezzi, C., & Falk, R. H.
Venue
Circulation
Published
2014-05-06
Volume
129
Issue
18
Pages
1840-1849
ISSN
  • 0009-7322 (ISSN-L)
  • 1524-4539
OpenAlex
W1987419031
Cited by
345
References
24
FWCI
10.71
Type
journal article
Language
en
Open access
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CoChat. (2026). Citation check: Left Ventricular Structure and Function in Transthyretin-Related Versus Light-Chain Cardiac Amyloidosis. https://app.cochat.ai/papers/10.1161/circulationaha.113.006242
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Quarta, C. C., Solomon, S. D., Uraizee, I., Kruger, J., Longhi, S., Ferlito, M., Gagliardi, C., Milandri, A., Rapezzi, C., & Falk, R. H. (2014). Left Ventricular Structure and Function in Transthyretin-Related Versus Light-Chain Cardiac Amyloidosis. Circulation. https://doi.org/10.1161/circulationaha.113.006242

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