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Echocardiography parameters for Differential Diagnosis in patients with suspected Cardiac Amyloidosis
Session:
Painel 9 - Doença Valvular 5
Speaker:
José Lopes De Almeida
Congress:
CPC 2020
Topic:
F. Valvular, Myocardial, Pericardial, Pulmonary, Congenital Heart Disease
Theme:
17. Myocardial Disease
Subtheme:
17.3 Myocardial Disease – Diagnostic Methods
Session Type:
Posters
FP Number:
---
Authors:
José Lopes De Almeida; Maria João Ferreira; Valdirene Gonçalves; Sofia S. Martinho; Maria João Cunha; Gracinda Costa; André Azul Freitas; João André Ferreira; Lino Gonçalves
Abstract
<p><strong>Background</strong></p> <p>Although a lot of studies validating echo parameters to distinguishing cardiac amyloidosis (CA) from other causes of myocardial thickening have been published, most of these were extrapolated by comparing CA patients data with reference values. Recently a study was carried out using a head-to-head comparison between CA and other causes of cardiac hypertrophy but it followed case-control design. This study aimed at comparing the diagnostic accuracy of various echo parameters in differentiating CA from other diagnosis, in a cohort of patients with a very high clinical suspicion of CA.</p> <p><strong>Methods </strong></p> <p>We retrospectively analyzed 48 patients referred between 2018 and 2019 to perform 99mTc-DPD scintigraphy due to a clinical suspicion of CA.</p> <p>We compared quantifiable conventional morphological and functional echocardiographic parameters along with deformation (strain) indices, previously suggested to diagnose CA, of patients with a positive CA diagnosis (by either DPD scan, a positive monoclonal component on serum immunofixation, urine immunofixation or serum free light chain assay or biopsy) (n=28) with the rest of the population (n=20). We used receiver operating characteristic curves (ROC) analysis for different echocardiographic parameters for prediction of the correct diagnosis.</p> <p><strong>Results</strong></p> <p>The studied population was predominantly old (77 +/- 12 years) and male (54%). Echocardiographic features were typical of a restrictive cardiomyopathy phenotype with dilated atria (mean Left Atrial Volume Index (mL/m2) = 54.66 +/- 25.99, mean Left Atrial Volume Index (mL/m2) = 37.82 +/- 18.77) and thickened ventricular walls (Interventricular septum thickness at end-diastole (IVSd) (mm) = 14.73 +/- 3.64, posterior wall thickness at end-diastole (PWTd) (mm) = 12.08 +/- 3.10).</p> <p>Of the 28 patients with a diagnosis of amyloidosis, 17 had senile (ATTR wild type) amyloidosis, 7 primary (AL) amyloidosis and 4 had familial amyloid neuropathies.</p> <p>Among traditional echo indices, Left Ventricular Mass Index (LVMI) show the best discriminatory power with the highest area under the curve (AUC = 0.797, p=003, 95% CI 0.644 to 0.950) [Figure 1] followed by IVSd (AUC= 0.733, p=.011, 95%IC 0.582 to 0.884) and PWTd (AUC=0.703, p=0.028, 95% IC 0.542 to 0.865). Strain parameters, including right and left global longitundinal strain and atrial strain, and diastolic parameters, including E/E’ and mitral deceleration time time were not statistically different between groups.</p> <p><strong>Conclusions</strong></p> <p>In a population of patients with a high suspicion of amyloidosis and echocardiographic characteristics suggestive of a restrictive cardiomyopathy, LVMI, IVST and PWT have the best discrimination power for an accurate diagnosis of CA.</p>
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