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Progression of echocardiographic parameters and prognosis in ATTR cardiac amyloidosis

Chacko, Liza; Karia, Nina; Venneri, Lucia; Bandera, Francesco; Dal Passo, Beatrice; Buonamici, Lodovico; Lazari, Jonathan; ... Fontana, Marianna; + view all (2022) Progression of echocardiographic parameters and prognosis in ATTR cardiac amyloidosis. European Journal of Heart Failure , 24 (9) pp. 1700-1712. 10.1002/ejhf.2606. Green open access

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Abstract

AIMS: Transthyretin amyloid cardiomyopathy (ATTR-CM) is an increasingly diagnosed disease. Echocardiography is widely utilized, but studies to confirm the value of echocardiography for tracking changes over time are not available. We sought to describe: (1) changes in multiple echocardiographic parameters; (2) differences in rate of progression of three predominant genotypes; and (3) the ability of changes in echocardiographic parameters to predict prognosis. METHODS AND RESULTS: We prospectively studied 877 ATTR-CM patients attending our centre between 2000 and 2020. Serial echocardiography findings at baseline, 12-months and 24-months were compared with survival. Five-hundred-and-sixty-five patients had wild-type ATTR-CM and 312 hereditary ATTR-CM (201 with V122I; 90 with T60A).There was progressive worsening of structural and functional parameters over time, patients with V122I ATTR-CM showing more rapid worsening of left and right ventricular structural and functional parameters compared to both wild-type and T60A ATTR-CM. Among a wide range of echocardiographic analyses, including deformation-based parameters, only worsening in the degree of mitral and tricuspid regurgitation (MR and TR) at 12-and 24 month assessments was associated with worse prognosis (change at 12-months: MR, hazard ratio 1.43 (1.14-1.80,p=0.002); TR, hazard ratio 1.38 (1.10-1.75,p=0.006). Worsening in MR remained independently associated with poor prognosis after adjusting for known predictors. CONCLUSION: In ATTR-CM, echocardiographic parameters progressively worsen over time. Patients with V122I ATTR-CM demonstrate the most rapid deterioration. Worsening of MR and TR were the only parameters associated with mortality, MR remaining independent after adjusting for known predictors. This article is protected by copyright. All rights reserved.

Type: Article
Title: Progression of echocardiographic parameters and prognosis in ATTR cardiac amyloidosis
Location: England
Open access status: An open access version is available from UCL Discovery
DOI: 10.1002/ejhf.2606
Publisher version: https://doi.org/10.1002/ejhf.2606
Language: English
Additional information: Copyright © 2022 The Authors. European Journal of Heart Failure published by John Wiley & Sons Ltd on behalf of European Society of Cardiology. This is an open access article under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/4.0/), which permits use, distribution and reproduction in any medium, provided the original work is properly cited.
Keywords: Cardiomyopathy, echocardiography, prognosis, progression, restrictive
UCL classification: UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Medical Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Medical Sciences > Div of Medicine > Inflammation
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences
UCL
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Medical Sciences > Div of Medicine
URI: https://discovery.ucl.ac.uk/id/eprint/10151272
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