Two common echocardiographic variables to diagnose cardiac amyloidosis: the AMYLoidosis Index (AMYLI) score

2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
A Aimo ◽  
V Chubuchny ◽  
G Vergaro ◽  
M Fontana ◽  
M Nicol ◽  
...  
Keyword(s):  
Wall Thickness ◽  
Cardiac Amyloidosis ◽  
Specific Treatment ◽  
Negative Likelihood Ratio ◽  
Left Ventricular ◽  
Blood Dyscrasia ◽  
Inferior Wall ◽  
Derivation Cohort ◽  
Amyloid Light Chain ◽  
Rule Out

Abstract Background Early diagnosis of cardiac amyloidosis (CA) is warranted to initiate specific treatment and improve outcome. The amyloid light chain (AL) and inferior wall thickness (IWT) scores have been proposed to assess patients referred by hematologists or with unexplained left ventricular (LV) hypertrophy, respectively. These scores are composed of 4 or 5 variables, respectively, including strain data, and no decisional cut-offs were introduced. Methods Based on 2 variables common to the AL and IWT scores, we defined a simple score named AMYLoidosis Index (AMYLI) as the product of relative wall thickness (RWT) and E/e' ratio, and assessed its diagnostic performance. Optimal rule-out cut-offs were searched as those with negative likelihood ratio (LR−) <0.1. Results In the derivation cohort (n=251), CA was ultimately diagnosed in 111 patients (44%). The 2.22 score value was selected as rule-out cut-off (LR- 0.0). In the hematology subset, AL CA was finally diagnosed in 32 patients (48%), with 2.36 as rule-out cut-off (LR− 0.0). In the hypertrophy subset, ATTR CA was diagnosed in 79 patients (43%), with 2.22 as best rule-out cut-off (LR− 0.0). In the validation cohort (n=691), where more patients were diagnosed with CA (94% and 68% in the hematology and in the hypertrophy subsets, respectively), the 2.22 rule-out cut-off had a LR− = ∞ (as no patient scoring <2.22 had CA). In the hematology and hypertrophy subsets, the 2.36 and 2.22 cut-offs were effective for ruling-out CA, with both LR− = ∞ (as no patient scoring <2.36 or 2.22, respectively, had CA). Conclusions The AMYLI score (RWT* E/e') is simpler than those proposed and similarly accurate. A 2.22 cut-off value excludes CA diagnosis in patients undergoing a diagnostic screening for CA, while a <2.36 and a <2.22 value may be better considered in the subsets with either blood dyscrasia or unexplained hypertrophy, respectively. Funding Acknowledgement Type of funding source: None

scholarly journals Natural history and disease progression of early cardiac amyloidosis evaluated by echocardiography

2021 ◽  
Vol 22 (Supplement_1) ◽  
Author(s):  
O Itzhaki Ben Zadok ◽  
A Eisen ◽  
Y Shapira ◽  
D Monakier ◽  
Z Iakobishvili ◽  
...  
Keyword(s):  
Disease Progression ◽  
Wall Thickness ◽  
Cardiac Amyloidosis ◽  
Time Course ◽  
Right Ventricular Dysfunction ◽  
Disease Diagnosis ◽  
Left Ventricular ◽  
Funding Sources ◽  
Lv Mass ◽  
Echocardiographic Findings

Abstract Funding Acknowledgements Type of funding sources: None. Background Since the diagnosis of cardiac amyloidosis (CA) is often delayed, echocardiographic findings are frequently indicative of already advanced cardiomyopathy. Aims to describe early echocardiographic features in patients subsequently diagnosed with CA and to delineate disease progression. Methods Pre-amyloid diagnosis echocardiographic studies were screened for structural and functional parameters and stratified according to the pathogenetic amyloid subtype (immunoglobulin light-chain (AL) or amyloid transthyretin (ATTR)). Abnormalities were defined based on published guidelines. Results Our cohort included 75 CA patients of whom 42 (56%) were diagnosed with AL and 33 (44%) with ATTR. Forty-two patients had an earlier echocardiography exam available for review. Patients presented with increased wall thickness (1.3 (IQR 1.0, 1.5)cm) ≥3 years before the diagnosis of CA and relative wall thickness (RWT) was increased (0.47 (IQR 0.41, 0.50)) ≥7 years pre-diagnosis. Between 1 to 3 years before CA diagnosis restrictive left ventricular (LV) filling pattern was present in 19% of patients and LV ejection fraction (LVEF)≤50% was present in 21% of patients. Right ventricular dysfunction was detected concomitantly with disease diagnosis. The echocardiographic phenotype of ATTR versus AL-CA showed increased RWT (0.74 (IQR 0.62, 0.92) vs. 0.62 (IQR 0.54, 0.76), p = 0.004) and LV mass index (144 (IQR 129, 191) vs. 115 (IQR 105, 146)g/m2,p = 0.020) and reduced LVEF (50 (IQR 44, 58) vs. (60 (IQR 53, 60)%, p = 0.009) throughout the time course of CA progression, albeit survival time was similar. Conclusions Increased wall thickness and diastolic dysfunction in CA develop over a time course of several years and can be diagnosed in their earlier stages by standard echocardiography Abstract Figure. Schematic proposed timeline of CA

Cardiac Amyloidosis Without Increased Left Ventricular Wall Thickness

2014 ◽  
Vol 89 (6) ◽  
pp. 781-789 ◽  
Author(s):  
Ga Yeon Lee ◽  
Kihyun Kim ◽  
Jin-Oh Choi ◽  
Seok Jin Kim ◽  
Jung-Sun Kim ◽  
...  
Keyword(s):  
Wall Thickness ◽  
Cardiac Amyloidosis ◽  
Left Ventricular ◽  
Left Ventricular Wall ◽  
Ventricular Wall ◽  
Left Ventricular Wall Thickness ◽  
Ventricular Wall Thickness

Deep learning to diagnose cardiac amyloidosis from CMR findings

2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
A Aimo ◽  
N Martini ◽  
A Barison ◽  
D Della Latta ◽  
A Ripoli ◽  
...  
Keyword(s):  
Deep Learning ◽  
Learning Strategies ◽  
Cardiac Amyloidosis ◽  
Left Ventricular ◽  
Automated Image Analysis ◽  
Blood Dyscrasia ◽  
Gradient Boosting ◽  
Al Amyloidosis ◽  
Test Accuracy ◽  
Imaging Features

Abstract Background Cardiac magnetic resonance (CMR) is an important diagnostic technique for cardiac amyloidosis (CA). A deep learning (DL) approach to define the likelihood of CA based on automated interpretation of CMR images has never been attempted so far. Methods 187 subjects underwent standard 1.5 T CMR examination (GE-Healthcare, Milwaukee, USA) as part of a diagnostic workup for either unexplained left ventricular hypertrophy or blood dyscrasia with suspected light-chain (AL) amyloidosis. Patients were randomly assigned to 3 subgroups, which were used for training (n=121, 65%), internal validation (n=28, 15%), and model testing (n=38, 20%). LGE images in different orientations (short-axis, 2- and 4-chambers) were selected as the most informative CMR features. A deep convolutional neural network was trained to classify CMR examinations as “amyloidosis” (probability ≥50%) or “no amyloidosis” (probability <50%) based on these features. Different learning strategies (data augmentation, batch normalization in convolutional layers, dropout before dense layers) were adopted to prevent model overfitting. Binary cross entropy was used as loss function. For comparison, a machine learning (ML) model based on gradient boosting trees was built for the binary classification of patients (amyloidosis vs no amyloidosis) based on clinical and imaging features extracted from the CMR exam. Results CA was diagnosed in 101 subjects (54%; 45 AL, 56 transthyretin amyloidosis). A model including 2C, 4C and SA LGE images was created. In the test cohort, it allowed to diagnose CA with good diagnostic accuracy (84.2%), and an area under the curve (AUC) of 0.96 (Figure). The precision (positive predictive value), recall score (sensitivity), and F1 score (a measure of test accuracy) were 0.78, 0.94, and 0.86, respectively. An ML algorithm considering all available parameters (LV volumes and function, LGE presence and pattern, early darkening, pericardial and pleural effusion, etc.) displayed a similar diagnostic performance than the DL method (AUC 0.93 vs. 0.96; p=0.45). Conclusions The deep learning technique allowed to create an accurate diagnostic tool for CA based on LGE patterns, which could be easily converted into an online platform for automated image analysis. Funding Acknowledgement Type of funding source: None

scholarly journals Left atrial strain in cardiac amyloidosis

2021 ◽  
Vol 42 (Supplement_1) ◽  
Author(s):  
A Aimo ◽  
I Fabiani ◽  
V Spini ◽  
V Chubuchny ◽  
E M Pasanisi ◽  
...  
Keyword(s):  
Cardiac Amyloidosis ◽  
Longitudinal Strain ◽  
Systolic Dysfunction ◽  
Global Longitudinal Strain ◽  
Left Ventricular ◽  
Left Atrial Strain ◽  
Funding Sources ◽  
Atrial Contraction ◽  
Amyloid Light Chain ◽  
Atrial Strain

Abstract Background Patients with cardiac amyloidosis (CA) display an enlarged and dysfunctional left atrium (LA), because of the effects of left ventricular (LV) diastolic and then systolic dysfunction, as well as the amyloid infiltration of LA wall. A single study reported impaired LA strain in CA, but differences among amyloid light-chain (AL) and transthyretin (ATTR) CA and the correlates of reduced LA strain have not been characterized. Methods We evaluated 426 consecutive patients undergoing a screening for suspected CA in 2 tertiary referral centres. Among them, 262 (61%) were diagnosed with CA (n=117 AL-CA, n=145 ATTR-CA). We measured peak atrial longitudinal strain (PALS) and peak atrial contraction strain (PACS) from 4- and 2-chamber (4C, 2C) views, and correlated them with maximum and minimum LA volumes, E/e' ratio, and LV global longitudinal strain (GLS). Results LA strain was much more severely impaired in patients with ATTR-CA than those without CA, and to a lesser extent than those with AL-CA (Figure). LA volumes were larger in patients with ATTR-CA than those without CA (maximal LA volume, p=0.042; minimal LA volume, p<0.001), and those with AL-CA (both volumes, p<0.001). LA strain values were more closely correlated with minimal than maximal LA volumes, and patients with AL-CA displayed stronger correlations than those with ATTR-CA or without CA; for example, Spearman's rho values for 4C-PALS vs. minimal LA volume were 0.595, 0.481, and 0.462, respectively (all p<0.001). Furthermore, LA strain correlated with E/e' in patients with AL-CA, but not in those with ATTR-CA: 4C-PALS vs. E/e', rho 0.406, p=0.001 (AL-CA), p=0.401 (ATTR-CA), and p=0.097 (no CA). Finally, LA strain correlated most closely with LV GLS in patients with AL-CA: 4C-PALS vs. LV GLS, rho 0.431, p<0.001 (AL-CA), rho 0.401, p<0.001 (ATTR-CA), rho 0.219, p=0.042 (no CA). Conclusions LA volume increase and reduced LA strain is particularly prominent in patients with ATTR-CA. Patients with AL-CA seem to display closer relationships between LA strain, size and haemodynamic load, possibly reflecting the most acute disease course, and lower time for amyloid deposition in the LA wall. FUNDunding Acknowledgement Type of funding sources: None.

scholarly journals 1038 Comparative assessment of phasic left atrial and regional left ventricular strain in patients with cardiac amyloidosis and LV hypertrophy

2020 ◽  
Vol 21 (Supplement_1) ◽  
Author(s):  
A M Brand ◽  
G Baldenhofer ◽  
D Frumkin ◽  
A Huebscher ◽  
K Stangl ◽  
...  
Keyword(s):  
Diagnostic Accuracy ◽  
Sensitivity And Specificity ◽  
Wall Thickness ◽  
Left Atrial ◽  
Cardiac Amyloidosis ◽  
Multivariate Logistic Regression Analysis ◽  
Diagnostic Value ◽  
Left Ventricular ◽  
Volume Index ◽  
Al Amyloidosis

Abstract Background Echocardiographic hallmarks of cardiac amyloidosis (CA), such as increased wall thickness of the LV and sparkling appearance of the myocardium, are limited by a reduced diagnostic accuracy. Purpose We sought to evaluate the diagnostic value of phasic left atrial strain alterations and of regional global longitudinal systolic LV strain (LVGLS) reductions in patients with CA and with other forms of LV hypertrophy. Methods Standard apical 4-chamber views were stored for offline analysis (Vivid E9, GE, Vingmed, Horton) in 54 patients who underwent endomyocardial biopsy for unclear LV hypertrophy. We then analyzed LVGLS as well as LA reservoir, conduit, and contraction strain using 2D speckle tracking echocardiography (2DSTE; EchoPAC software, GE). To assess regional LVGLS, the average of apical strain values / (average of mid + basal LV strain values) was calculated (relative apical sparing; RELAPS). Receiver operating characteristic (ROC) curve analyses and a multivariate logistic regression analysis were performed to investigate the diagnostic value of the respective LA and LV deformation analysis. Results CA was bioptically confirmed in 34 patients (13 TTR, 1 AA, 20 AL amyloidosis). In 18 patients, myocardial biopsy revealed other forms of LV hypertrophy, such as hypertensive heart disease (n = 2), hypertrophic cardiomyopathy (n = 12), and inflammatory myocardial diseases (n = 4). Mean septal wall thickness (17.7 ± 2.9 mm and 17.9 ± 4.3 mm) and left atrial volume index (43.8 ± 12.2 and 44.1 ± 17.2) were not different between groups. RELAPS was significantly higher in patients with CA (1.37 ± 0.94 vs. 0.86 ± 0.29, p<.007). Phasic atrial mechanics were significantly worse in CA (LA reservoir, conduit, and contraction strain 10.0 ± 5.2%, -6.5 ± 3.5%, and -5.0 ± 4.1%, respectively, in CA; and 22.7 ± 7.8%, -13.9 ± 5.2%, and -13.0 ± 5.5%, in LVH, respectively; p<.001). With an area under the curve (AUC) of 0.91, and a sensitivity and specificity of 91.2 and 84.2% for a cut-off value of <15.8%, LA reservoir strain showed a higher diagnostic accuracy in discriminating CA from LVH than the parameter RELPAS (AUC 0.74, sensitivity and specificity 60% and 71% for a cut-off of >1.0; p<.05). LA conduit and contraction strain performed significantly better than RELAPS as well (AUC 0.87 for conduit, and AUC 0.86 for contraction function; p<.001 each). Of all echocardiographic parameters, LA reservoir strain remained significantly associated with CA in a multivariate regression model. Conclusions LA strain during all three phases of the atrial cycle was significantly reduced in patients with CA compared to other forms of LVH, and showed a markedly higher diagnostic accuracy than regional LV strain analysis, with LA reservoir strain showing highest discriminative value. The assessment of LA strain, as part of a comprehensive echocardiographic assessment, may be useful to rule-in the possible diagnosis of CA in patients with unclear LV hypertrophy.

scholarly journals Incremental benefits of echocardiographic indices over clinical parameters for screening cardiac amyloidosis in patients with left ventricular hypertrophy

2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
M Saito ◽  
Y Nakao ◽  
R Higaki ◽  
Y Yokomoto ◽  
A Ogimoto ◽  
...  
Keyword(s):  
Ejection Fraction ◽  
Diagnostic Accuracy ◽  
Wall Thickness ◽  
Left Atrial ◽  
Cardiac Amyloidosis ◽  
Global Longitudinal Strain ◽  
Left Ventricular ◽  
Volume Index ◽  
Clinical Parameters ◽  
Clinical Model

Abstract Background Cardiac amyloidosis (CA), characterized by amyloid protein deposition in the heart, is a treatable disease. Although left ventricular (LV) wall thickness is the most established imaging predictor for CA, several echocardiographic indices including deformation parameters also contribute to the screening of CA. However, it is unclear whether additive values of echocardiographic indices have greater benefit over the conventional clinical predictors for the screening of CA. Therefore, we sought to compare the incremental benefits of echocardiographic indices over the clinical parameters for the screening of CA and externally validate their incremental benefits. Methods We retrospectively studied 295 consecutive patients (median age, 67 years; male, 65%; mean LV wall thickness (MWT), 12 mm) with LV hypertrophy who underwent echocardiography as well as the detailed work-up for myocardium (Biopsy, technetium pyrophosphate scintigraphy (99mTc-PYP) or cardiac magnetic resonance imaging). CA was diagnosed through biopsy or 99mTc-PYP. The clinical model considers patients' age and the low-voltage in electrocardiography in reference to previous studies. Continuous echocardiographic variables were represented in binary through generally accepted external cutoff points. The incremental benefits of the echocardiography findings over the clinical model were assessed using with the help of both receiver-operated characteristic curve analysis and comparison of area under the curves. Furthermore, these incremental benefits were validated in the external validation sample (median age, 70 years; male, 69%; MWT, 12 mm). Results Among the enrolled patients, CA was observed in 18% of cases. Table presents the results of this study. Of the echocardiographic parameters, relative apical sparing pattern (RASP) was the greatest contributor for improvement of diagnostic accuracy of the clinical model. The next greatest contributor was LV wall thickness, followed by left atrial reservoir strain (LAS), E/e', left atrial volume index, ejection fraction strain ratio, and pericardial effusion, respectively. Similarly, RASP, LV wall thickness, global longitudinal strain, ejection fraction, LAS, and granular sparkling showed significant incremental benefit in the validation cohort. Only mean wall thickness, LV wall thickness, LAS, E/e' and RASP consistently improved the diagnostic accuracy of the clinical model. Conclusion During the screening process, adding LV wall thickness, LAS, and RASP to the clinical parameters may be useful for the accurate diagnosis of CA in patients with LV hypertrophy. Figure 1 Funding Acknowledgement Type of funding source: None

Clinical significance of the relative apical sparing pattern of longitudinal strain in patients with cardiac amyloidosis

2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
M Saito ◽  
Y Nakao ◽  
R Higaki ◽  
Y Kawachi ◽  
Y Yokomoto ◽  
...  
Keyword(s):  
New York ◽  
Clinical Significance ◽  
Wall Thickness ◽  
Cardiac Amyloidosis ◽  
Longitudinal Strain ◽  
Heart Association ◽  
Left Ventricular ◽  
Association Class ◽  
New York Heart Association ◽  
Prognostic Prediction

Abstract Background The relative apical sparing pattern (RASP) of left ventricular (LV) longitudinal strain (LS) is frequently associated with cardiac amyloidosis (CA). However, some patients with CA do not show the RASP, and their clinical characteristics have not been fully clarified. We sought to investigate the clinical significance of RASP in patients with CA. Methods One hundred consecutive CA patients who were diagnosed by biopsy or myocardial pyrophosphate scintigraphy and evaluated for RASP (mean age: 76 years, male: 77%, LV mean wall thickness: 13.5 mm, light-chain [AL] type: 33 cases, transthyretin [TTR] type: 67 cases) were retrospectively enrolled. The RASP was semi-quantitatively and quantitatively assessed. Semi-quantitative RASP was defined as reduction of LS (≥−10%) in ≥5 (of 6) basal segments relative to preserved LS (<−15%) in ≥1 apical segment. Quantitative RASP was calculated according to the following formula: Quantitative RASP = [Average apical LS] / [Average basal LS + Average mid LS]. We adapted three validated thresholds (>1.00, >0.90, and >0.87) according to the literature. Results Semi-quantitative and binalized quantitative RASP (>1.00, >0.90, and >0.87) were observed in 55, 55, 63, and 65 patients, respectively. RASP in each definition was more prevalent in the TTR group than in the AL group. Additionally, RASP was significantly associated with higher LV wall thickness even after adjustment for the CA subtypes (all, p<0.05, Figure). After the RASP assessment, 35 all-cause deaths and 26 cardiac deaths were observed during the follow-up period (median, 1.1 years). Although these events were significantly associated with poor nutrition, lower blood pressure, higher New York Heart Association class, and the AL group, no association was found with RASP and LV wall thickness. Conclusions The incidence of RASP is low in the case of thin LV wall thickness in CA patients, which may indicate the difficulty of early diagnosis of CA using RASP in patients with mild LV hypertrophy. The prognostic prediction using RASP may be challenging in this cohort. Figure 1 Funding Acknowledgement Type of funding source: None

scholarly journals A simple tool to help ruling-out Covid-19 in the emergency department: derivation and validation of the LDH-CRP-Lymphocyte (LCL) score

2020 ◽  
Vol 16 (3) ◽  
Author(s):  
Jacopo Davide Giamello ◽  
Giulia Paglietta ◽  
Giulia Cavalot ◽  
Attilio Allione ◽  
Sara Abram ◽  
...  
Keyword(s):  
Validation Cohort ◽  
Negative Likelihood Ratio ◽  
C Reactive Protein ◽  
Derivation Cohort ◽  
Reactive Protein ◽  
Simple Tool ◽  
Lactate Dehydrogenase C ◽  
The Difference ◽  
To Receive ◽  
Rule Out

After the outbreak of the Covid-19 pandemic, cases of SARSCoV- 2 infections may gradually decrease in the next months. Given the reduced prevalence of the disease, Emergency Departments (ED) are starting to receive more and more non- Covid19 patients. Thus, a way to quickly discriminate ED patients with potential Covid-19 infection from non-Covid19 patients is needed in order to keep potentially contagious patients isolated while awaiting second-level testing. In this paper, we present the derivation and validation of a simple, practical, and cheap score that could be helpful to rule out Covid-19 among ED patients with suspicious symptoms (fever and/or dyspnoea). The LCL score was derived from a cohort of 335 patients coming to the ED of our hospital from March 16th to April 1st, 2020. It was then retrospectively validated in a similar cohort of 173 patients admitted to our ED during April. The score is based on blood values of lactate dehydrogenase, C-reactive protein, and lymphocyte count. The LCL score performed well both in the derivation and in the validation cohort, with an AUC respectively of 0.81 (95% CI: 0.77 – 0.86) and of 0.71 (95% CI: 0.63 – 0.78), given the difference in Covid- 19 prevalence between the two cohorts (57% vs 41% respectively). An LCL score equal to 0 had a negative predictive value of 0.92 in the derivation cohort and of 0.81 in the validation cohort, with a negative likelihood ratio respectively of 0.08 and 0.36 for Covid- 19 exclusion. This score could, therefore, constitute a useful tool to help physicians manage patients in the ED.

P2458Restricted left atrial motion as a result of atrial stiffening in patients with cardiac amyloidosis

2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
H Higashi ◽  
K Inoue ◽  
M Saito ◽  
M Kinoshita ◽  
J Aono ◽  
...  
Keyword(s):  
Ejection Fraction ◽  
Left Atrial ◽  
Cardiac Amyloidosis ◽  
Validation Cohort ◽  
Left Ventricular ◽  
Volume Index ◽  
Stiffness Index ◽  
Derivation Cohort ◽  
Independent Validation ◽  
Lv Ejection Fraction

Abstract Background Left atrial (LA) involvement of abnormal amyloid fibrils could induce LA dysfunction and stiffening in patients with cardiac amyloidosis (CA). Thus, the assessments of LA function and stiffness might be a potential approach to diagnose CA phenotype among patients with hypertrophied hearts. Purpose We sought to determine whether LA reservoir strain with speckle tracking echocardiography could be used a marker of LA stiffness in a derivation cohort. Furthermore, we tested to our hypothesis that LA reservoir strain could differentiate CA patients from hypertrophic cardiomyopathy (HCM) in an independent validation cohort. Methods In the derivation cohort, echocardiography was performed simultaneously with measurements of pulmonary capillary wedge pressure (PCWP) in 50 patients with suspected or established heart failure and relatively preserved left ventricular (LV) ejection fraction (56±10%). LA maximum and minimum volume index, and reservoir strain were measured from apical four-chamber view. LA stiffness index was computed as a pressure rise from x-trough to v-wave divided by an increase from minimum to maximum indexed LA volume (Figure A). In an independent validation group, we studied a total of 33 biopsy-proved CA patients and 127 HCM patients (LV ejection fraction: 57±11% vs. 66±10%, P<0.01) in sinus rhythm on the date of comprehensive echocardiographic study. Among them, cardiac magnetic resonance imaging (CMR) could be evaluated in 17 CA patients and 98 HCM patients. Furthermore, right heart catheterization was performed with 12 CA patients and 12 HCM patients in the CMR group. Results The derivation cohort study found that there was a significant curvilinear correlation of LA reservoir strain to LA stiffness index (Figure B). In the validation cohort, LA reservoir strain was reduced in patients with CA compared with HCM in all participants (11.6±5.6% vs. 18.5±6.9%, P<0.01), although there was no significant difference of LA maximal volume index between 2 groups (37±16 ml/m2 vs. 37±12 ml/m2, p=0.89). In the CMR group, the late gadolinium enhancement was observed in the LA wall in 16 patients with CA (94.1%) as shown in Figure C. In contrast, the LA enhancement revealed only in 1 patient with HCM (1.0%). Among patients with invasive measures, LA stiffness index [median (interquartile range)] was higher in patients with CA than that in patients with HCM [1.1 (0.4–2.8) vs. 0.2 (0.1–0.6), P=0.01]. Conclusions LA reservoir function was fairly limited in patients with CA compared with HCM. Restricted LA motion might be related to atrial amyloid deposits or fibrosis, which potentially provokes atrial chamber stiffening.

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