P895Apical sparing in patients without cardiac amyloidosis

2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
C B Binder ◽  
F Duca ◽  
S Aschauer ◽  
R Rettl ◽  
L Camuz Ligios ◽  
...  

Abstract Background Apical sparing describes a reduced longitudinal strain in the basal segments and preserved or supranormal longitudinal strain in the apical segments of the left ventricular (LV) myocardium. This pattern has been described as a typical finding in patients with cardiac amyloidosis (CA) and restrictive cardiomyopathy. However, apical sparing is not a quantitative parameter and is fairly subjective to the echocardiographer's judgement. It is not known, if a certain degree of apical sparing is also present in patients with only mild LV hypertrophy and diastolic dysfunction such as it is present in heart failure with preserved ejection fraction (HFpEF). Methods Patients with cardiac transthyretin and light chain amyloidosis and patients with HFpEF were included in a clinical registry at our outpatient clinic. CA was diagnosed according to current guidelines. All patients underwent a comprehensive transthoracic echocardiography (TTE) exam at the time of study inclusion. The TTE protocol included standard and speckle-tracking imaging to assess the presence of apical sparing as well as the basal to apical strain gradient. Patients with known coronary artery disease were excluded. Results In total 115 patients were included in this study. Of these, 87 (75.7%) were diagnosed with CA and 28 (24.3%) with HFpEF. Not surprisingly, apical sparing was found in a majority (86.2%) of patients with CA, however mild forms of this phenomenon were also present in 67.9% of patients with HFpEF (p=0.029, Figure 1). Median basal longitudinal strain was significantly more impaired in patients with CA (p<0.001) but there was no difference between longitudinal strain in the apical segments when comparing CA to HFpEF (p=0.443). This resulted in a higher median apical to basal strain gradient in patients with CA (2.3 (IQR 1.7–3.83) versus 1.13 (IQR 1.5–1.8), p<0.001). Figure 1 Conclusion Mild forms of apical sparing can be found in patients without CA. Gradual reduction in strain from base to apex could be an unspecific pathophysiologic mechanism which is remarkably pronounced in patients with CA.

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


PLoS ONE ◽  
2021 ◽  
Vol 16 (8) ◽  
pp. e0255487
Author(s):  
Osnat Itzhaki Ben Zadok ◽  
Mordehay Vaturi ◽  
Iuliana Vaxman ◽  
Zaza Iakobishvili ◽  
Noa Rhurman-Shahar ◽  
...  

Aims To compare the baseline cardiovascular characteristics of immunoglobulin light-chain (AL) and amyloid transthyretin (ATTR) cardiac amyloidosis (CA) and to investigate patients’ contemporary cardiac outcomes. Methods Single-center analysis of clinical, laboratory, echocardiographic and cardiac magnetic resonance imaging (CMRi) characteristics of AL and ATTR-CA patients’ cohort (years 2013–2020). Results Included were 67 CA patients of whom 31 (46%) had AL-CA and 36 (54%) had ATTR-CA. Patients with ATTR-CA versus AL-CA were older (80 (IQR 70, 85) years versus 65 (IQR 60, 71) years, respectively, p<0.001) with male predominance (p = 0.038). Co-morbidities in ATTR-CA patients more frequently included diabetes mellitus (19% versus 3.0%, respectively, p = 0.060) and coronary artery disease (39% versus 10%, respectively, p = 0.010). By echocardiography, patients with ATTR-CA versus AL-CA had a trend to worse left ventricular (LV) ejection function (50 (IQR 40, 55)% versus 60 (IQR 45, 60)%, respectively, p = 0.051), yet comparable LV diastolic function. By CMRi, left atrial area (31 (IQR 27, 36)cm2 vs. 27 (IQR 23, 30)cm2, respectively, p = 0.015) and LV mass index (109 (IQR 96, 130)grams/m2 vs. 82 (IQR 72, 98)grams/m2, respectively, p = 0.011) were increased in patients with ATTR-CA versus AL-CA. Nevertheless, during follow-up (median 20 (IQR 10, 38) months), patients with AL-CA were more frequently admitted with heart failure exacerbations (HR 2.87 (95% CI 1.42, 5.81), p = 0.003) and demonstrated increased mortality (HR 2.51 (95%CI 1.19, 5.28), p = 0.015). Conclusion Despite the various similarities of AL-CA and ATTR-CA, these diseases have distinct baseline cardiovascular profiles and different heart failure course, thus merit tailored-cardiac management.


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.


2021 ◽  
Vol 42 (Supplement_1) ◽  
Author(s):  
K Inoue ◽  
Y Nakao ◽  
M Saito ◽  
M Kinoshita ◽  
R Higaki ◽  
...  

Abstract Objective To investigate a mechanistic determinant of left atrial (LA) reservoir function in patients presenting left ventricular (LV) hypertrophy, and clarify diagnostic and prognostic values of LA reservoir strain in patients with cardiac amyloidosis (CA). Methods Three-hundred sixty patients (median age: 68 years, male gender: 65%) with left ventricular hypertrophy (LVH) assessed by echocardiography were retrospectively included. The LVH etiologies were diagnosed by any of biopsy, cardiac magnetic resonance imaging or 99mTc-PYP scintigraphy. LV segmental longitudinal strain was estimated from apical three views, and LA reservoir strain was measured from an apical 4-chamber view. Results The LVH etiologies were confirmed with CA in 81 patients, hypertensive heart disease in 87 patients, hypertrophic cardiomyopathy in 143 patients, and miscellaneous disorders in 49 patients. The median (25th, 75th percentile) value of LV ejection fraction was 59% (48–67). LV basal longitudinal strain and LA reservoir strain were significantly reduced in patients with CA compared with those with other etiologies; LV basal strain: 5.4% (3.9–8.7) vs. 11.9% (9.3–14.6), LA reservoir strain: 9.2% (6.3–12.3) vs. 17.5% (11.3–24.1), p&lt;0.01 respectively. LV basal strain was significantly correlated with LA reservoir strain in patients with CA (r=0.57, p&lt;0.01) and in those with other etiologies (r=0.45, p&lt;0.01). The area under the receiver-operating characteristic curves of LA reservoir strain and E/e' (0.78 and 0.74) to identify CA etiology were significantly larger than that of LA volume index (0.62) (p&lt;0.01). During the follow-up period (median 2.9 years), 53 patients experienced heart failure hospitalization. The Cox regression model including age, gender, LV ejection fraction, E/e' and LA reservoir strain showed that male gender (hazard ratio: 0.46, p=0.03), E/e' (hazard ratio: 1.04, p&lt;0.01) and LA reservoir strain (hazard ratio: 0.94, p&lt;0.01) independently predicted heart failure hospitalization. Conclusions The decrease of LV longitudinal shortening at cardiac base could worsen LA reservoir function especially in patients with CA. LA reservoir strain might be an alternative measure to identify CA etiology and have a predictive value of heart failure hospitalization in patients with LV hypertrophy. FUNDunding Acknowledgement Type of funding sources: None.


Medicina ◽  
2021 ◽  
Vol 57 (12) ◽  
pp. 1372
Author(s):  
Gheorghe Stoichescu-Hogea ◽  
Florina Nicoleta Buleu ◽  
Ruxandra Christodorescu ◽  
Raluca Sosdean ◽  
Anca Tudor ◽  
...  

Background: Contribution of global and regional longitudinal strain (GLS) for clinical assessment of patients with heart failure with preserved ejection fraction (HFpEF) is not well established. We sought to evaluate subclinical left ventricular dysfunction secondary to coronary artery disease (CAD) in HFpEF patients compared with hypertensive patients and age-matched healthy subjects. Material and methods: This was a retrospective study that included 148 patients (group 1 = 62 patients with HFpEF, group 2 = 46 hypertensive patients, and group 3 = 40 age-matched control subjects). Peak systolic segmental, regional (basal, mid, and apical), and global longitudinal strain were assessed for each study group using two-dimensional speckle-tracking echocardiography (2D-STE). Results: GLS values presented statistically significant differences between the three groups (p < 0.001); markedly increased values (more negative) were observed in the control group (−20.2 ± 1.4%) compared with HTN group values (−18.4 ± 3.0%, p = 0.031) and with HFpEF group values (−17.6 ± 2.3%, p < 0.001). The correlation between GLS values and HTN stages was significant, direct, and average (Spearman coefficient rho = 0.423, p < 0.001). GLS had the greatest ability to detect patients with HFpEF when HFpEF + CAD + HTN diastolic dysfunction (n = 30) + CON diastolic dysfunction (n = 2) from HFpEF + CAD + HTN + CON was analyzed. (optimal GLS limit of −19.35%, area under curve = 0.833, p < 0.001). Conclusions: Global longitudinal strain can be used for clinical assessment in differentiating coronary and hypertensive patients at higher risk for development of systolic dysfunction.


Classification 418Dilated cardiomyopathy 420Dilated cardiomyopathy: treatment 422Hypertrophic cardiomyopathy 424Hypertrophic cardiomyopathy: investigations 428Hypertrophic cardiomyopathy: treatment 430Restrictive cardiomyopathy 432Cardiac amyloidosis 434Cardiac amyloidosis: treatment 436Fabry disease 438Arrhythmogenic right ventricular cardiomyopathy (ARVC) 440ARVC: management 442Left ventricular non-compaction ...


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