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
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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