3078Usefulness of a semi-quantitative and layer-specific assessment of the relative apical sparing pattern of longitudinal strain for the identification of cardiac amyloidosis

2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
M Saito ◽  
M Imai ◽  
D Wake ◽  
R Higaki ◽  
T Sumimoto ◽  
...  

Abstract Background The relative apical sparing pattern (RASP) of left ventricular longitudinal strain (LS) is determined using a strain polar map, while global longitudinal strain is measured using speckle-tracking echocardiography, and it is frequently associated with cardiac amyloidosis (CA). However, the definition of visual RASP is ambiguous, and this leads to insufficient reproducibility, whereas quantitative RASP takes time and leads to difficulty in the clinical application. Generally, amyloid predominantly accumulates in the endo-myocardial layer. As such, layer-specific analysis of RASP may more accurately identify CA. Therefore, the aims of this study were to explore the reproducible and easy definition of RASP for identifying CA and investigate the effect of layer-specific analysis on the assessment. Methods A total of 40 patients with CA diagnosed by biopsy and technetium pyrophosphate scintigraphy were compared with 120 control patients matched for mean left ventricular wall thickness (40 aortic stenosis, 40 hypertrophic cardiomyopathy, and 40 hypertensive heart disease). We compared the discriminative abilities of three definitions of RASP (visual, quantitative, and semi-quantitative). According to a previous paper, visual RASP was defined as visual reduction of LS in the basal and middle LS segments (light red or blue) relative to the apical LS (red). Quantitative RASP was calculated using the following formula: average apical LS/(average basal LS + average mid-ventricle LS), then binarized by the optimal cut-off value for predicting CA. Semi-quantitative RASP was defined as reduction of LS (≥-10%) in five or more segments out of the basal six segments, relative to apical LS (≤-15%). Sample cases are shown in Figure (left). Visual and semi-quantitative RASP were independently assessed by two blinded sonographers. The RASP at the endo-myocardial and all layers was evaluated using customized software. The concordance was assessed using the kappa statistic, whereas the discriminative ability was assessed using receiver operating characteristic curve analysis. Results The concordance of visual RASP was modest but its semi-quantitative RASP was perfect (Table right). The discriminative ability of semi-quantitative RASP at each layer was significantly better than that of visual RASP and close to that of the binary quantitative RASP. Additionally, the discriminative abilities of visual (p=0.10) and semi-quantitative (p=0.11) RASP at the endo-myocardial layer appeared to be better than those at all layers. Conclusions The assessment method of semi-quantitative RASP is easy and highly reproducible. Furthermore, it accurately discriminates CA. In addition, assessment at the endo-myocardial layer potentially improves the discriminative ability.

Open Heart ◽  
2020 ◽  
Vol 7 (2) ◽  
pp. e001346
Author(s):  
Aénora Roger-Rollé ◽  
Eve Cariou ◽  
Khailène Rguez ◽  
Pauline Fournier ◽  
Yoan Lavie-Badie ◽  
...  

BackgroundCardiac amyloidosis (CA) is a life-threatening restrictive cardiomyopathy. Identifying patients with a poor prognosis is essential to ensure appropriate care. The aim of this study was to compare myocardial work (MW) indices with standard echocardiographic parameters in predicting mortality among patients with CA.MethodsClinical, biological and transthoracic echocardiographic parameters were retrospectively compared among 118 patients with CA. Global work index (GWI) was calculated as the area of left ventricular pressure–strain loop. Global work efficiency (GWE) was defined as percentage ratio of constructive work to sum of constructive and wasted works. Sixty-one (52%) patients performed a cardiopulmonary exercise.ResultsGWI, GWE, global longitudinal strain (GLS), left ventricular ejection fraction (LVEF) and myocardial contraction fraction (MCF) were correlated with N-terminal prohormone brain natriuretic peptide (R=−0.518, R=−0.383, R=−0.553, R=−0.382 and R=−0.336, respectively; p<0.001). GWI and GLS were correlated with peak oxygen consumption (R=0.359 and R=0.313, respectively; p<0.05). Twenty-eight (24%) patients died during a median follow-up of 11 (4–19) months. The best cut-off values to predict all-cause mortality for GWI, GWE, GLS, LVEF and MCF were 937 mm Hg/%, 89%, 10%, 52% and 15%, respectively. The area under the receiver operator characteristic curve of GWE, GLS, GWI, LVEF and MCF were 0.689, 0.631, 0.626, 0.511 and 0.504, respectively.ConclusionIn CA population, MW indices are well correlated with known prognosis markers and are better than LVEF and MCF in predicting mortality. However, MW does not perform better than GLS.


Circulation ◽  
2020 ◽  
Vol 142 (Suppl_3) ◽  
Author(s):  
Julia M Simkowski ◽  
Michael Jiang ◽  
NADIA El HANGOUCHE ◽  
Jeesoo Lee ◽  
Milica Marion ◽  
...  

Introduction: Relative apical longitudinal strain (RALS) is defined as (average apical LS/(average basal & mid-ventricular LS)). A threshold of 2 has been found to have high sensitivity and specificity for differentiating cardiac amyloidosis (CA) from other causes of left ventricular hypertrophy (LVH). This threshold was developed using General Electric (GE) software, and its reproducibility among different software vendors is unknown. Hypothesis: In patients with CA, regional segmental LS patterns and relative apical longitudinal strain will vary among software vendors. Methods: Speckle-tracking echocardiography was retroactively performed by an experienced technician on two patient cohorts, CA (n=52) and LVH (n=52), using software from two independent vendors: EchoPAC (GE Medical Systems) and TomTEC (TOMTEC Imaging Systems GMBH). For each vendor and patient, strain values for the basal, mid, and apical segments were averaged to obtain three regional LS values which were then used to calculate global longitudinal strain (GLS) and RALS. Results: EchoPAC demonstrated greater average apical LS (-16.5±5.7 vs -13.1±6.6, p<0.001) and RALS (2.1±0.9 vs 1.7±0.7, p<0.001) compared to TomTEC. Bland-Altman analysis yielded a mean bias of -0.4 with limit of agreement 2.2 (p<0.001) in RALS between the two vendors. ROC curve analysis using a RALS cutoff of 2 to differentiate CA from the overall control group showed similarly high specificity (EchoPAC 85%, TomTEC 83%) between vendors but lower sensitivity for TomTEC (23% vs 45%) (Figure 1). LVH subgroup analysis showed similar comparisons. Overall difference in area-under-curve (AUC) was significant (AUC = 0.78 EchoPAC vs AUC = 0.52 TomTEC, p < 0.001). Conclusions: Software measurements of regional LS and thus RALS vary between vendors. Further efforts are needed for intervendor regional strain fidelity. For now, different RALS thresholds to diagnose CA may be needed for various vendors.


2020 ◽  
Vol 21 (Supplement_1) ◽  
Author(s):  
R Ramos Polo ◽  
S Moral Torres ◽  
C Tiron De Llano ◽  
M Morales Fornos ◽  
J M Frigola Marcet ◽  
...  

Abstract INTRODUCTION Differential diagnosis by echocardiography between cardiac amyloidosis (CA) and hypertrophic cardiomyopathy (HCM) is based on the evaluation of left ventricular ejection fraction (LVEF) and global longitudinal strain (GLS) of the entire myocardial wall. Nevertheless, histopathological studies describe a higher involvement of subendocardial tissue in CA. The aim of our study was to evaluate whether the subanalysis of the GLS by layers (subendocardial and subepicardial) and segments (apical and basal) can provide further information. METHODS Retrospective study including 33 consecutive patients diagnosed with CA (with histological confirmation and imaging tests) or HCM by established criteria. Advanced myocardial deformation analysis software was used for both subendocardial and subepicardial evaluation of the left ventricle wall by transthoracic echocardiography. RESULTS Seventeen patients (52%) had CA and sixteen (48%) had HCM. Differences were observed in LVEF (52.9 ± 10.9% vs 62.4 ±5.0%; p = 0.004), but not in the analysis of the entire wall GLS (-12.3 ± 4.9 vs -13.4 ± 2.8; p = 0.457) nor in the LVEF/GLS ratio (4.7 ± 1.4 vs 4.8 ± 1.1; p = 0.718). In the layered analysis there was no difference in subendocardial GLS (-16.2 ± 5.0 vs -16.4 ± 3.2%; p = 0.916) or subepicardial GLS (-11.7 ± 4.1 vs -11.6 ±2.7%; p = 0.945); however, the increase in GLS from base to apex was greater for CA than for HCM both at subepicardial level (increase: 101% vs 16%; p = 0.006) and subendocardial level (increase: 242% vs 114%; p = 0.006), with inversion of the greatest values for each group (Fig. 1).The ratio (apical GLS/basal GLS) was diagnostic predictor of CA (area under the curve = 86%; p = 0.002): a value &gt;2 presented a sensitivity of 84% and a specificity of 85% for the diagnosis of CA. CONCLUSIONS CA presents an impairment of both subendocardial and subepicardial deformation in transthoracic echocardiography. These patterns provide additional information on differential diagnosis with HCM. Abstract P940 Figure. Subendo vs subepicardial mean values


2021 ◽  
Vol 42 (Supplement_1) ◽  
Author(s):  
A Aimo ◽  
I Fabiani ◽  
V Spini ◽  
V Chubuchny ◽  
E M Pasanisi ◽  
...  

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&lt;0.001), and those with AL-CA (both volumes, p&lt;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&lt;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&lt;0.001 (AL-CA), rho 0.401, p&lt;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.


Circulation ◽  
2020 ◽  
Vol 142 (Suppl_3) ◽  
Author(s):  
Peter Huntjens ◽  
Kathleen Zhang ◽  
Yuko Soyama ◽  
Maria Karmpalioti ◽  
Daniel Lenihan ◽  
...  

Introduction: Light chain cardiac amyloidosis (AL) has a variable but usually poor prognosis. Left ventricular (LV) function measures including LV strain imaging for global longitudinal strain (GLS) have shown clinically prognostic value in AL. However, the utility of novel left atrial (LA) strain imaging and its associations with LV disease remains unclear. Hypothesis: LA strain is of additive prognostic value to GLS in AL. Methods: We included 99 consecutive patients with AL. Cardiac amyloidosis either confirmed by endocardial biopsy (25%) or by non-cardiac tissue biopsy and imaging data supportive of cardiac amyloidosis. Peak LA reservoir strain was calculated as an average of peak longitudinal strain from apical 2- and 4-chamber views. GLS and apical sparing ratio were assessed using the 3 standard apical views. All-cause mortality was tracked over a median of 5 years. Results: Echocardiographic GLS and peak longitudinal LA strain were feasible in 96 (97%) and 86 (87%) of patients, respectively. There were 48 AL patients who died during follow-up. Patients with low GLS (GLS < median; 10.3% absolute values) had worse prognosis than patients with high GLS group (p<0.001). Although peak longitudinal LA strain was correlated with GLS (R=0.65 p<0.001), peak longitudinal LA strain had additive prognostic value. AL patients with low GLS and low Peak LA strain (<13.4%) had a 8.3-fold increase in mortality risk in comparison to patients with high GLS (95% confidence interval: 3.84-18.03; p<0.001). Multivariable analysis showed peak longitudinal LA strain was significantly and independently associated with survival after adjusting for clinical and echocardiographic covariates (p<0.01). Conclusions: Peak longitudinal LA strain was additive to LV GLS in predicting prognosis in patients with biopsy confirmed AL amyloidosis. LA strain imaging has potential clinical utility in patients with AL cardiac amyloidosis.


2020 ◽  
Vol 26 (10) ◽  
pp. S33-S34
Author(s):  
Ahmad Yehia Alazawie ◽  
Ali S. Ali Al-Shammari ◽  
Reham M. Ibrahim ◽  
Mohammed T. Mutar ◽  
Hilal Al-Saffar ◽  
...  

Circulation ◽  
2014 ◽  
Vol 130 (suppl_2) ◽  
Author(s):  
Taichi Hayashi ◽  
Satoshi Yamada ◽  
Masahiro Nakabachi ◽  
Hiroyuki Iwano ◽  
Mamoru Sakakibara ◽  
...  

Background: We conducted a multicenter study in Japan to compare tissue Doppler echocardiography-derived and speckle tracking echocardiography (STE)-derived parameters of left ventricular (LV) diastolic function for the estimation of LV filling pressure. Methods: Seventy-eight patients (age 59±14 years, 59 male) with structural heart diseases including angina pectoris (n=34), old myocardial infarction (n=15), dilated cardiomyopathy (n=13), hypertrophic cardiomyopathy (n=8), and hypertensive heart disease (n=8) were studied according to the same protocol in 5 university hospitals in Japan. LV mean diastolic pressure (MDP) was measured by using a micromanometer-tipped catheter. Early-diastolic mitral annular velocity (e′) was measured at septal and lateral side of the annulus and averaged, and the ratio of early-diastolic transmitral velocity to e′ (E/e′) was calculated. Global longitudinal strain was measured using STE in 3 apical views and averaged on a frame-by-frame basis to construct a time-strain curve as well as a time-strain rate (SR) curve. Then, the absolute value of peak longitudinal strain (LS), early-diastolic peak SR (LSR E ), and that during isovolumic relaxation (IVR) period (LSR IVR ) were measured. Similarly, peak circumferential strain (CS), early-diastolic peak circumferential SR (CSR E ), and that during IVR (CSR IVR ) were measured from 3 short-axis views. Results: E/e′ weakly correlated with MDP (r=0.50, p<0.001). E/LSR IVR and E/CSR IVR did not correlate with MDP, whereas there was a significant but weak correlation between E/LSR E and MDP (r=0.50, p<0.001) or between E/CSR E and MDP (r=0.41, p<0.001). In contrast, there was a good correlation between E/LS and MDP (r=0.70, p<0.001) or between E/CS and MDP (r=0.62, p<0.001). Especially, the correlation between E/LS and MDP was statistically better than that between E/e′ and MDP (p<0.01). Conclusions: STE-derived longitudinal parameters better correlated with MDP than circumferential parameters. Especially, E/LS was better than E/e′ for estimating LV filling pressure.


2021 ◽  
Vol 42 (Supplement_1) ◽  
Author(s):  
K Uneno

Abstract Background Type 2 diabetes mellitus (T2DM) impairs cardiac function and is one of the risk factors for heart failure. However, the mechanism of cardiac impairment is not elucidated. Despa et al. reported that amylin has aggregation properties similar to amyloidogenic proteins and impairs cardiac function in T2DM patients with hyperamylinemia. In this hypothesis, T2DM-induced myocardial impairment is thought of as amylin-induced cardiac amyloidosis. The purpose of this study is to clarify whether a relative apical sparing pattern (RASP), which is well known as a typical pattern of cardiac amyloidosis, is common in T2DM. Methods We studied patients aged 75 years or over who underwent echocardiography from January 2018 to December 2020 in our clinic. We calculated the quantitative relative apical sparing (qRASP) as average apical-longitudinal strain (LS)/(average basal-LS + average mid-LS) in each patient. According to the validated threshold, a qRASP ≥1.0 was defined as an obvious RASP (oRASP). We compared the ratio of oRASP between patients with and without T2DM. Results We researched 506 patients, mean 81.8 years, 290 females, 133 with T2DM. The average age, atrial fibrillation rate, heart failure rate, and hypertension rate were similar in both groups. The ratio of ischemic heart disease was higher in the T2DM group. Echocardiography showed that the left atrial dimension (LAD), E/e', and left ventricular hypertrophy (LVH) ratio was higher in the T2DM group. Speckle tracking echocardiography revealed that global longitudinal strain (GLS) and qRASP of the T2DM group were higher than the non-DM group (GLS;-18.0% vs. −19.2%, p&lt;0.001. qRASP; 0.809 vs. 0.699, P&lt;0.001). Furthermore, the ratio of oRASP was significantly higher in the T2DM group (19.0% vs. 1.34%, p&lt;0.001). Multivariable logistic regression analysis showed T2DM was an independent predictor for oRASP. Conclusion This study revealed that the qRASP and the ratio of oRASP in the T2DM group were higher than non-DM group. This finding supports the hypothesis that T2DM related cardiomyopathy is a kind of cardiac amyloidosis caused by amylin. FUNDunding Acknowledgement Type of funding sources: None.


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