Apical sparing strain pattern observed in danon disease: insights from a global registry

2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
Q Bui ◽  
G Ma ◽  
M Kraushaar ◽  
V Escobedo ◽  
B Le ◽  
...  

Abstract Background Danon Disease (DD) is a rare X-linked autophagic disorder due to mutations in the Lysosomal Associated Membrane Protein 2 (LAMP-2) gene and causes severe cardiac manifestations. Measurement of longitudinal strain (LS) has been shown to provide diagnostic insights into different etiologies of hypertrophic cardiomyopathies compared to conventional echocardiographic parameters. Purpose The aim of this study was to describe the pattern of global and regional LS in DD. Methods A retrospective, international registry, using medical records provided by patients, was formed to describe the natural history of DD. Complete echocardiogram images were available for review and LS was analyzed globally and regionally (basal, mid, apex). Results A total of eighteen DD patients (male 72%, mean age 17.2±10 years) had sufficient quality echocardiographic images for both traditional and myocardial strain evaluation. Notable traditional echocardiographic parameters included a mean EF of 60±11%, LV mass index 200±159 g/m2, intraventricular septal diameter 17.7±10.3 mm, LV posterior wall diameter 16.1±7.7 mm, LA volume index 21.9±13 mL/m2. Global longitudinal strain was reduced with a mean of −12.1±4.9% with an observed regional strain gradient: apex (−16.6±6.6%), mid (−10.9±4.7%) and basal (−9.2±4.5%). Bull's eye plot patterns reflected an apical sparing pattern that was similar to that described in cardiac amyloidosis. Conclusion In this DD cohort, we describe for the first time a strain pattern characterized by reduction in global longitudinal strain with apical sparing, which was originally pathognomonic for cardiac amyloidosis. This strain pattern in conjunction with a paradoxically normal LA volume may discriminate patients with DD from other hypertrophic conditions. Funding Acknowledgement Type of funding source: None

2020 ◽  
Vol 21 (Supplement_1) ◽  
Author(s):  
P Geenty ◽  
S Sivapathan ◽  
T Deshmukh ◽  
P Brown ◽  
A Boyd ◽  
...  

Abstract Background AL-amyloidosis has a rapid clinical progression, with cardiac involvement associated with a particularly poor prognosis. Cardiac amyloidosis is diagnosed by either invasive biopsy or conventional echocardiographic parameters such as increased wall thickness, in the absence of other causes. More recently, novel parameters including 2D longitudinal strain have demonstrated diagnostic utility in a range of infiltrative cardiomyopathies including cardiac amyloidosis. Aim/Method: We sought to evaluate traditional and novel echocardiographic parameters in their ability to predict adverse outcomes in a cohort of AL-amyloid patients. 80 patients who had transthoracic echocardiograms at a single centre were included. Comprehensive echocardiographic assessment was performed, including left ventricular ejection fraction (LVEF), LV Global Longitudinal Strain (GLS), LV mass (indexed to BSA). The primary endpoint was a composite of of major adverse cardiac events (MACE) and all-cause mortality, that was assessed by interrogation of the medical records on a specified censor date. Results At a mean follow-up (time from echo to censor date) of 5.4 ± 2.6years, 38/80 (47.5%) of patients experienced the primary endpoint of MACE or death, of which 25/80 (31%) were deaths. LVEF (59 ± 5.6%vs56 ± 6.4%, p = 0.04), GLS (17.4 ± 3.9%vs14.8 ± 4.9%, p = 0.01) basal longitudinal strain (12.3 ± 3.2%vs9.6 ± 3.9%, p = 0.002), indexed LV mass (107 ± 36g/m2vs130 ± 34g/m2, p = 0.06) and E/E’ (13.7 ± 4.9vs20.6 ± 9.6, p < 0.001) were all significantly different between patients who experienced the primary endpoint and those that didn’t. The strongest predictors of outcome were E/E’ (AUC 0.74), LV mass (AUC 0.73) and the ratio GLS:LV mass (AUC 0.73). An E/E’ of 15 had a sensitivity of 71% and specificity of 69%, while an indexed LV mass of 108 had a sensitivity and specificity of 74% and 67% respectively. GLS to LV mass as a cutoff of 0.16 had a sensitivity and specificity of 70% and 69% respectively. Conclusion In a cohort of 80 patients with AL-amyloid cardiomyopathy, almost half (47.5%) reached the primary composite endpoint. Diastolic dysfunction as expressed as E/E’, and LV mass were the most powerful predictors of outcome, while global longitudinal strain and LV basal strain were also reduced, and showed superiority over LV ejection fraction in predicting prognosis.


2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
H Kawakami ◽  
S Ramkumar ◽  
F Pathan ◽  
L Wright ◽  
T H Marwick

Abstract Background Although LV hypertrophy and dysfunction are associated with atrial fibrillation (AF), AF often occurs in the absence of LV hypertrophy or reduced ejection fraction. The effect of subclinical LV dysfunction on AF has not been fully studied. Purpose We sought the association between subclinical LV dysfunction (measured with global longitudinal strain, GLS) and new-onset AF. Methods This observational study evaluated 531 consecutive patients (median age, 67 years [interquartile range, 56 to 78]; 56% male), without a history of AF who underwent strain echocardiography after cryptogenic stroke. The CHARGE-AF score was used to calculate the 5-year risk of developing AF. Standard echocardiographic parameters were measured, and speckle-tracking was used to measure LA (reservoir strain, pump strain, and conduit strain) and LV strain (GLS). A strain analysis was conducted using a dedicated software package, using R-R gating. The baseline clinical and echocardiographic parameters of the patients who developed AF and those who did not were compared. Results Over 2.5 years of follow-up, 61 patients (11%) had new-onset AF. Patients who developed AF were older, had a higher CHARGE-AF score, larger LA volume, worse LA strain, and worse GLS than those who did not. Areas under the receiver-operating curve for GLS (0.84) was comparable to CHARGE-AF (0.79), LA pump strain (0.83), and LA reservoir strain (0.85). In the nested Cox models, GLS demonstrated an independent and incremental predictive value over the clinical and LA parameters (Figure). Moreover, adding GLS to the combined clinical and LA parameters model resulted in a significantly improved reclassification (net reclassification improvement, 0.32; p=0.016). Importantly, the predictive value of GLS was confirmed in patients with abnormal LA volumes (LA volume index≥34ml/m2) but not in patients with normal LA volumes. Figure 1 Conclusion GLS is associated with new-onset AF, especially in patients with abnormal LA volumes. This effect is independent of and incremental to the clinical and LA parameters.


Circulation ◽  
2020 ◽  
Vol 142 (Suppl_3) ◽  
Author(s):  
Shaun Khanna ◽  
Aditya Bhat ◽  
Henry H Chen ◽  
Kennith Gu ◽  
Gary Gan ◽  
...  

Introduction: Myocarditis is an inflammatory disease process with growing clinical relevance in the current COVID-19 pandemic. Acute-phase myocarditis is known to result in subclinical changes in left ventricular (LV) function despite normal LV ejection fraction (LVEF), as assessed by myocardial deformation indices. The presence of right ventricular (RV) and left atrial (LA) subclinical dysfunction however has not been well described in current literature. Hypothesis: Myocarditis patients have subclinical impairment of LV, RV and LA function as assessed by global longitudinal strain (GLS) on speckle tracking echocardiography. Methods: Consecutive patients with clinical diagnosis of myocarditis admitted to our institution during 2013-2018 were assessed (n=76). Patients who did not meet appropriate diagnostic criteria (n=14), had impaired LVEF or prior cardiac disease (n=8) or poor transthoracic echocardiogram images (n=14) were excluded from analysis. Clinical and echocardiographic parameters were compared to age- , gender- and risk factor- matched controls. GLS was performed by two independent observers using vendor independent software (TomTec Arena, Germany v4.6). Results: The final cohort consisted 40 patients with myocarditis (age 44.3±16.7, 60% male) and 40 matched controls (44.5±16.6, 60% male). No significant differences in baseline clinical characteristics were observed between groups. No differences in LVEF, indexed LV mass, RV fractional area change, indexed LA volume or TR pressure gradient (p>0.05 for all) were demonstrated between the two groups. Patients with myocarditis had a lower mean LV strain (GLS%: -16.4±2.9 vs -19.7±2.7, p=0.0001), a lower mean RV Free Wall Strain (FWS) (GLS%: -22.1±4.1 vs -26.2±6.9, p=0.03) and a lower mean LA reservoir strain (GLS%: 27.5±4.6 vs. 33.7±6.3, p<0.0001) when compared to controls. Conclusions: Our results demonstrate the presence of significant subclinical global myocardial dysfunction despite normal traditional echocardiographic indices, in patients with acute-phase myocarditis. Routine assessment of GLS may identify such patients for early targeted cardiac therapy.


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.


ESC CardioMed ◽  
2018 ◽  
pp. 1283-1286
Author(s):  
John D. Horowitz ◽  
Thanh Ha Nguyen ◽  
Sven Y. Surikow ◽  
Gao Jing Ong ◽  
Cher-Rin Chong ◽  
...  

Takotsubo syndrome reflects an ‘aberrant’ response to acute catecholamine stimulation, largely but not entirely as a manifestation of increased sensitivity to β‎2-adrenoceptor stimulation and signal transduction via Gi proteins, especially in ageing female hearts. It is now apparent that prolonged impairment of quality of life and slow recovery of echocardiographic parameters such as global longitudinal strain after episodes of takotsubo syndrome reflect ongoing myocardial inflammation and associated oedema, together with impairment of cardiac energetics on phosphorus magnetic resonance spectroscopy.


2020 ◽  
Vol 21 (Supplement_1) ◽  
Author(s):  
J Sabatino ◽  
I Leo ◽  
D De Rosa ◽  
A Polimeni ◽  
S Sorrentino ◽  
...  

Abstract Funding Acknowledgements ESC Training Grant 2019 Background Non-invasive prediction of critical coronary artery stenosis (CCS) is challenging. CCS may cause persistent impaired longitudinal function at rest, which could be detected by two-dimensional speckle tracking echocardiography (2D-STE). However, strain parameters are load dependent, hence they might not reflect systolic function accurately. A novel non-invasive method to calculate MW recently showed a strong correlation with invasive work measurements. Objectives To investigate the ability of non-invasive Myocardial Work (MW) indices: 1) to identify the ischaemic risk area during acute coronary occlusion (ACO); 2) to predict the ischaemic risk area underlying a CCS before coronary angiography. Methods The study population comprises 80 individuals: fifty consecutive patients (pts) referred for coronary angiography in a single tertiary coronary care centre and thirty controls (CTRL). Echocardiography recordings (GE-Healthcare) with systolic and diastolic non-invasive pressures, were obtained immediately before coronary angiography, during ACO, and immediately after revascularization to measure longitudinal strain along with myocardial work indices: Myocardial Work Index (MWI), Myocardial Constructive Work (MCW), Myocardial Wasted work (MWW), Myocardial work efficiency (MWE). Results Compared to baseline, we found a significant reduction of global longitudinal strain (GLS) (p = 0.005), MWI, MCW and MWE (p &lt; 0.001) during ACO. After the procedure, we observed a significant improvement in GLS, MWI, MCW and MWE (p &lt; 0.001) compared to the findings obtained during CO. Interestingly, baseline global MWI, MCW and MWE were significantly reduced in pts with CCS at angiography compared to controls (p &lt; 0.05). Moreover, regional MWE, but not longitudinal strain, measured within the myocardial segments underlying the CCS, was significantly reduced compared to non-target segments (p &lt; 0.001). Conclusions Our results demonstrate that non-invasive estimation of regional and global MW parameters are able to identify myocardial ischemia upon ACO. Moreover, myocardial work indices measured at rest were able to predict the presence of CCS before invasive angiography, whereas MWE was more sensitive than myocardial strain to document regional impairment of myocardial function. Abstract 157 Figure.


2020 ◽  
Vol 9 (12) ◽  
pp. 3882
Author(s):  
Thomas Stiermaier ◽  
Kira Busch ◽  
Torben Lange ◽  
Toni Pätz ◽  
Moritz Meusel ◽  
...  

Cardiac magnetic resonance (CMR)-derived left ventricular (LV) global longitudinal strain (GLS) provides incremental prognostic information on various cardiovascular diseases but has not yet been investigated comprehensively in patients with Takotsubo syndrome (TS). This study evaluated the prognostic value of feature tracking (FT) GLS, tissue tracking (TT) GLS, and fast manual long axis strain (LAS) in 147 patients with TS, who underwent CMR at a median of 2 days after admission. Long-term mortality was assessed 3 years after the acute event. In contrast to LV ejection fraction and tissue characteristics, impaired FT-GLS, TT-GLS and fast manual LAS were associated with adverse outcome. The best cutoff points for the prediction of long-term mortality were similar with all three approaches: FT-GLS −11.28%, TT-GLS −11.45%, and fast manual LAS −10.86%. Long-term mortality rates were significantly higher in patients with FT-GLS > −11.28% (25.0% versus 9.8%; p = 0.029), TT-GLS > −11.45% (20.0% versus 5.4%; p = 0.016), and LAS > −10.86% (23.3% versus 6.6%; p = 0.014). However, in multivariable analysis, diabetes mellitus (p = 0.001), atrial fibrillation (p = 0.001), malignancy (p = 0.006), and physical triggers (p = 0.006) outperformed measures of myocardial strain and emerged as the strongest, independent predictors of long-term mortality in TS. In conclusion, CMR-based longitudinal strain provides valuable prognostic information in patients with TS, regardless of the utilized technique of assessment. Long-term mortality, however, is mainly determined by comorbidities.


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


2020 ◽  
Vol 21 (Supplement_1) ◽  
Author(s):  
Y Dimitroglou ◽  
C Aggeli ◽  
A Alexopoulou ◽  
T Alexopoulos ◽  
A Nitsa ◽  
...  

Abstract Introduction Non-alcoholic steatohepatitis (NASH) in patients with metabolic syndrome is a common cause of cirrhosis and has been associated with increased cardiovascular mortality. In patients with liver cirrhosis systolic or diastolic dysfunction can be observed and is independent of the cirrhosis etiology. Only few studies using newer echocardiography indices such as Global Longitudinal Strain (GLS) have been published in cirrhotic patients. Purpose To evaluate GLS in patients with NASH cirrhosis when compared to other etiologies. Methods A total of consecutive 36 cirrhotic patients aged 18-70 were included in our study. Standard speckle-tracking software was used for offline analysis of standard apical views and GLS was calculated. Stroke Volume Index (SVI) was calculated with the Simpson method and a standard 2D, Doppler and Tissue Doppler examination was performed in all patients. Results Median age of the study population was 58 (IQR 50-64) years, 78% were male and 17% had ascites. Cirrhosis was considered decompensated in 21 (58%) of patients. The 28%, 42% and 19% had NASH-associated, alcoholic and viral etiology of cirrhosis, respectively. Median ejection fraction (EF) was 60% (IQR: 57%; 65%) and GLS was -21.1% (-19.7%; -23.1%) in the total population. Absolute value of GLS was lower in patients with NASH cirrhosis compared to other etiologies (p = 0.009) (figure 1). EF, SVI, left atrial volume index (LAVI), E/e’ ratio and mitral annular velocity (e’) did not differ significantly between those with NASH associated cirrhosis and the rest. GLS values were significantly correlated with EF (r=-0.588, p = 0.002), SVI (r=-0.469, p = 0.016) and BNP levels (r=-0.571, p = 0.007), but not with age, left ventricular end diastolic volume, left atrial volume index, E/e’, mitral annular velocity and blood pressure. According to a multivariable linear regression model, NASH etiology [B = 2.1 (0.6; 3.7), p = 0.008)] and EF (per 10% increase) [B=-1.7 (-3.3; -0.2), p = 0.03)] were the only independent factors associated with GLS values in cirrhotic patients. Conclusions GLS values are within normal limits in cirrhotic patients but seem to be affected in patients with NASH associated cirrhosis. Further studies are needed to assess the prognostic implications of this finding. Abstract P1768 Figure 1


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