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Bandera, Francesco; Martone, Raffaele; Chacko, Liza; Ganesananthan, Sharmananthan; Gilbertson, Janet A.; Ponticos, Markella; Lane, Thirusha; Martinez-Naharro, Ana; Whelan, Carol; Quarta, Cristina; Rowczenio, Dorota; Patel, Rishi; Razvi, Yousuf; Lachmann, Helen; Wechelakar, Ashutosh; Brown, James; Knight, Daniel; Moon, James; Petrie, Aviva; Cappelli, Francesco; Guazzi, Marco; Potena, Luciano; Rapezzi, Claudio; Leone, Ornella; Hawkins, Philip N.; Gillmore, Julian D.; Fontana, Marianna
JACC. Cardiovascular imaging, January 2022, 2022-01-00, 20220101, Letnik: 15, Številka: 1Journal Article
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. The clinical significance of LA involvement in ATTR-CM is of great clinical interest. 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). 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. 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. Display omitted
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JCR | SNIP | JCR | SNIP | JCR | SNIP | JCR | SNIP |
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in: SICRIS
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