• JACC Cardiovasc Imaging · Apr 2020

    Multicenter Study

    Multiparametric Echocardiography Scores for the Diagnosis of Cardiac Amyloidosis.

    • Michele Boldrini, Francesco Cappelli, Liza Chacko, Maria Alejandra Restrepo-Cordoba, Angela Lopez-Sainz, Alberto Giannoni, Alberto Aimo, Andrea Baggiano, Ana Martinez-Naharro, Carol Whelan, Cristina Quarta, Claudio Passino, Vincenzo Castiglione, Vladyslav Chubuchnyi, Valentina Spini, Claudia Taddei, Giuseppe Vergaro, Aviva Petrie, Luis Ruiz-Guerrero, Vanessa Moñivas, Susana Mingo-Santos, Jesus G Mirelis, Fernando Dominguez, Esther Gonzalez-Lopez, Stefano Perlini, Gianluca Pontone, Julian Gillmore, Philip N Hawkins, Pablo Garcia-Pavia, Michele Emdin, and Marianna Fontana.
    • National Amyloidosis Centre, University College London, Royal Free Campus, London, United Kingdom; Emergency Department, Internal Medicine Department, Amyloidosis Research and Treatment Center, Istituto di Ricerca a Carattere Clinico e Scientifico Policlinico San Matteo Foundation, Pavia, Italy.
    • JACC Cardiovasc Imaging. 2020 Apr 1; 13 (4): 909-920.

    ObjectivesThis study aimed to investigate the accuracy of a broad range of echocardiographic variables to develop multiparametric scores to diagnose CA in patients with proven light chain (AL) amyloidosis or those with increased heart wall thickness who had amyloid was suspected. We also aimed to further characterize the structural and functional changes associated with amyloid infiltration.BackgroundCardiac amyloidosis (CA) is a serious but increasingly treatable cause of heart failure. Diagnosis is challenging and frequently unclear at echocardiography, which remains the most often used imaging tool.MethodsWe studied 1,187 consecutive patients evaluated at 3 referral centers for CA and analyzed morphological, functional, and strain-derived echocardiogram parameters with the aim of developing a score-based diagnostic algorithm. Cardiac amyloid burden was quantified by using extracellular volume measurements at cardiac magnetic resonance.ResultsA total of 332 patients were diagnosed with AL amyloidosis and 339 patients with transthyretin CA. Concentric remodeling and strain-derived parameters displayed the best diagnostic performance. A multivariable logistic regression model incorporating relative wall thickness, E wave/e' wave ratio, longitudinal strain, and tricuspid annular plane systolic excursion had the greatest diagnostic performance in AL amyloidosis (area under the curve: 0.90; 95% confidence interval: 0.87 to 0.92), whereas the addition of septal apical-to-base ratio yielded the best diagnostic accuracy in the increased heart wall thickness group (area under the curve: 0.80; 95% confidence interval: 0.85 to 0.90).ConclusionsSpecific functional and structural parameters characterize different burdens of CA deposition with different diagnostic performances and enable the definition of 2 scores that are sensitive and specific tools with which diagnose or exclude CA.Copyright © 2020. Published by Elsevier Inc.

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