Clinical Importance of Left Atrial Infiltration in Cardiac Transthyretin Amyloidosis

JACC-CARDIOVASCULAR IMAGING

卷:15

Article

Bandera, Francesco^1,2  Martone, Raffaele³  Chacko, Liza⁴  Ganesananthan, Sharmananthan⁴  Gilbertson, Janet A.⁴  Ponticos, Markella⁴  Lane, Thirusha⁴  Martinez-Naharro, Ana⁴  Whelan, Carol⁴  Quarta, Cristina⁴  Rowczenio, Dorota⁴  Patel, Rishi⁴  Razvi, Yousuf⁴  Lachmann, Helen⁴  Wechelakar, Ashutosh⁴  Brown, James⁴  Knight, Daniel⁴  Moon, James⁵  Petrie, Aviva⁶  Cappelli, Francesco³  Guazzi, Marco^1,2  Potena, Luciano⁷  Rapezzi, Claudio^8,9  Leone, Ornella⁷  Hawkins, Philip N.⁴  Gillmore, Julian D.⁴  Fontana, Marianna⁴ 

[1] IRCCS Policlin San Donato, Cardiol Univ Dept, Heart Failure Unit, Milan, Italy

[2] Univ Milan, Dept Biomed Sci Hlth, Milan, Italy

[3] Careggi Univ Hosp AOUC, Tuscan Reg Amyloid Ctr, Florence, Italy

[4] UCL, Natl Amyloidosis Ctr, Royal Free Campus,Rowland Hill St, London NW3 2PF, England

[5] St Bartholomews Hosp, Barts Heart Ctr, London, England

[6] UCL, Eastman Dent Inst, Grays Inn Rd, London, England

[7] Acad Hosp S Orsola Malpighi, Bologna, Italy

[8] Univ Ferrara, Univ Cardiol Ctr, Ferrara, Italy

[9] Maria Cecilia Hosp, GVM Care & Res, Cotignola, RA, Italy

关键词: amyloidosis;    atrial function;    atrial histology;    atrial stiffness;    atrial strain;    echocardiography;   

DOI  :  10.1016/j.jcmg.2021.06.022

来源: Elsevier

【 摘 要 】

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-ATTRCM; 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. (J Am Coll Cardiol Img 2022;15:17-29) (c) 2022 The Authors. Published by Elsevier on behalf of the American College of Cardiology Foundation. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).

【 授权许可】

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