Clinical Importance of Left Atrial Infiltration in Cardiac Transthyretin Amyloidosis
Autor: | Luciano Potena, Dorota Rowczenio, Janet A. Gilbertson, Daniel R. Knight, James Brown, Raffaele Martone, Ornella Leone, Ashutosh Wechelakar, Claudio Rapezzi, Carol J. Whelan, James C. Moon, Aviva Petrie, Markella Ponticos, Thirusha Lane, Liza Chacko, Sharmananthan Ganesananthan, Julian D. Gillmore, Rishi K Patel, Ana Martinez-Naharro, Philip N. Hawkins, Helen J. Lachmann, Francesco Bandera, Francesco Cappelli, Cristina Quarta, Marco Guazzi, Yousuf Razvi, Marianna Fontana |
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Jazyk: | angličtina |
Rok vydání: | 2022 |
Předmět: |
Pathology
medicine.medical_specialty ROI region of interest MCF myocardial contraction factor ATTR-CM transthyretin amyloid cardiomyopathy HF heart failure LA left atrium TAPSE tricuspid annular plane systolic excursion NO MAPSE mitral annular plane systolic excursion Muscular Diseases atrial function Predictive Value of Tests Left atrial atrial strain medicine Humans Prealbumin atrial histology echocardiography wtATTR wild-type transthyretin Radiology Nuclear Medicine and imaging Heart Atria Original Research amyloidosis Amyloid Neuropathies Familial biology business.industry Amyloidosis atrial stiffness medicine.disease hATTR hereditary ATTR amyloidosis PASP pulmonary artery systolic pressure Transthyretin Atrial strain LV left ventricle EMB endomyocardial biopsy biology.protein LAS left atrium strain Cardiology and Cardiovascular Medicine Amyloid cardiomyopathy business Infiltration (medical) |
Zdroj: | Jacc. Cardiovascular Imaging |
Popis: | 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. Central Illustration |
Databáze: | OpenAIRE |
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