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Clinical Importance of Left Atrial Infiltration in Cardiac Transthyretin Amyloidosis

Bandera, Francesco; Martone, Raffaele; Chacko, Liza; Ganesananthan, Sharmananthan; Gilbertson, Janet A; Ponticos, Markella; Lane, Thirusha; ... Fontana, Marianna; + view all (2022) Clinical Importance of Left Atrial Infiltration in Cardiac Transthyretin Amyloidosis. JACC: Cardiovascular Imaging , 15 (1) pp. 17-29. 10.1016/j.jcmg.2021.06.022. Green open access

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Abstract

Objectives: The aim of this study was to characterize left atrial (LA) pathology in explanted hearts with transthyretin amyloid cardiomyopathy (ATTR-CM); LA mechanics using echocardiographic speckle-tracking in a large cohort of patients with ATTR-CM; and to study the association with mortality. Background: The clinical significance of LA involvement in ATTR-CM is of great clinical interest. Methods: Congo red staining and immunohistochemistry was performed to assess the presence, type, and extent of amyloid and associated changes in 5 explanted ATTR-CM atria. Echo speckle tracking was used to assess LA reservoir, conduit, contractile function, and stiffness in 906 patients with ATTR-CM (551 wild-type (wt)-ATTR-CM; 93 T60A-ATTR-CM; 241 V122I-ATTR-CM; 21 other). Results: There was extensive ATTR amyloid infiltration in the 5 atria, with loss of normal architecture, vessels remodeling, capillary disruption, and subendocardial fibrosis. Echo speckle tracking in 906 patients with ATTR-CM demonstrated increased atrial stiffness (median [25th-75th quartile] 1.83 [1.15-2.92]) that remained independently associated with prognosis after adjusting for known predictors (lnLA stiff: HR: 1.23; 95% CI: 1.03-1.49; P = 0.029). There was substantial impairment of the 3 phasic functional atrial components (reservoir 8.86% [5.94%-12.97%]; conduit 6.5% [4.53%-9.28%]; contraction function 4.0% [2.29%-6.56%]). Atrial contraction was absent in 22.1% of patients whose electrocardiograms showed sinus rhythm (SR) “atrial electromechanical dissociation” (AEMD). AEMD was associated with poorer prognosis compared with patients with SR and effective mechanical contraction (P = 0.0018). AEMD conferred a similar prognosis to patients in atrial fibrillation. Conclusions: The phenotype of ATTR-CM includes significant infiltration of the atrial walls, with progressive loss of atrial function and increased stiffness, which is a strong independent predictor of mortality. AEMD emerged as a distinctive phenotype identifying patients in SR with poor prognosis.

Type: Article
Title: Clinical Importance of Left Atrial Infiltration in Cardiac Transthyretin Amyloidosis
Location: United States
Open access status: An open access version is available from UCL Discovery
DOI: 10.1016/j.jcmg.2021.06.022
Publisher version: https://doi.org/10.1016/j.jcmg.2021.06.022
Language: English
Additional information: THI S I S AN OP EN AC C E S S ART I C L E UNDE R T HE CC BY -NC-ND L I C ENS E ( h t t p : / / c r e a t i v e commo n s . o r g / l i c e n s e s / b y - n c - n d / 4 .0/ ) .
Keywords: Science & Technology, Life Sciences & Biomedicine, Cardiac & Cardiovascular Systems, Radiology, Nuclear Medicine & Medical Imaging, Cardiovascular System & Cardiology, amyloidosis, atrial function, atrial histology, atrial stiffness, atrial strain, echocardiography, CARDIOVASCULAR MAGNETIC-RESONANCE, PROGNOSTIC VALUE, SINUS RHYTHM, THROMBI, DIAGNOSIS, FAILURE
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
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Medical Sciences > Eastman Dental Institute
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Population Health Sciences > Institute of Cardiovascular Science > Clinical Science
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Population Health Sciences > Institute of Cardiovascular Science
URI: https://discovery.ucl.ac.uk/id/eprint/10143589
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