scholarly journals P309 The use of echocardiographic parameters to predict clinical outcomes in AL-amyloidosis cardiomyopathy

2020 ◽  
Vol 21 (Supplement_1) ◽  
Author(s):  
P Geenty ◽  
S Sivapathan ◽  
T Deshmukh ◽  
P Brown ◽  
A Boyd ◽  
...  
Keyword(s):  
Ejection Fraction ◽  
Primary Endpoint ◽  
Cardiac Amyloidosis ◽  
Longitudinal Strain ◽  
Global Longitudinal Strain ◽  
Al Amyloidosis ◽  
Predictors Of Outcome ◽  
Lv Mass ◽  
Echocardiographic Parameters ◽  
Al Amyloid

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.

scholarly journals P1543 The assessment of regional myocardial strain in classifying amyloid cardiomyopathy

2020 ◽  
Vol 21 (Supplement_1) ◽  
Author(s):  
P Geenty ◽  
S Shivapathan ◽  
T Deshmukh ◽  
P Brown ◽  
A Boyd ◽  
...  
Keyword(s):  
Ejection Fraction ◽  
Diastolic Dysfunction ◽  
Wall Thickness ◽  
Cardiac Amyloidosis ◽  
Longitudinal Strain ◽  
Treatment Options ◽  
Wild Type ◽  
Lv Mass ◽  
Lv Ejection Fraction ◽  
Al Amyloid

Abstract Background An infiltrative cardiomyopathy is a common manifestation of AL-amyloidosis, with cardiac involvement associated with a poor prognosis. Wild-type transthyretin amyloidosis (wt-TTR), is a distinct clinical entity occurring predominantly in men > 65 yrs, that has gained interest recently due to novel treatment options. Regional strain analysis has been shown to discriminate both forms of cardiac amyloidosis from other causes of concentric left ventricular hypertrophy, with a characteristic pattern of ‘apical sparing’. Due to the significant difference in both the course of the disease and treatment options between groups, a non-invasive echocardiographic method of determining subtype would be valuable. Aim/Method: We sought to compare traditional and novel echocardiographic parameters in a cohort of AL ( n = 80) and wild type (wt-TTR) amyloid ( n = 32) patients. All amyloid patients underwent comprehensive transthoracic echocardiography, including both conventional parameters and LV longitudinal strain. Further novel parameters were computed including the ratio of global longitudinal strain (GLS) to LV ejection fraction (LVEF), as well as GLS to indexed LV mass. Results wt-TTR patients had significantly greater LV mass (176 ± 59g/m2vs118 ± 37g/m2, p < 0.001), and worse diastolic dysfunction as expressed as E/E’ (21.5 ± 11vs17 ± 8, p = 0.04). LVEF was significantly lower in wt-TTR patients however remained in the normal range in both groups (53 ± 6%vs57 ± 6%, p = 0.001), whilst GLS was significantly reduced compared to AL-amyloid patients (11.5 ± 3.4%vs16.2 ± 4.6%, p < 0.001). LVEF:GLS was significantly higher in wt-TTR patients (4.93 ± 1.4vs3.87 ± 1.3, p = 0.001) reflecting a more profound reduction in strain with a relatively preserved ejection fraction. Similarly, the ratio of GLS to LV mass was significantly lower in wt-TTR amyloidosis (0.078 ± 0.05vs0.155 ± 0.07, p < 0.001), reflecting a more significant reduction in strain for a given wall thickness in wt-TTR patients. GLS:LV mass was the strongest discriminator between subtypes (AUC 0.82), with a cutoff of 0.09 giving a sensitivity and specificity of 71% and 80% respectively, for detecting wt-TTR. Conclusion In this cohort, patients with wt-TTR had significantly greater increase in LV wall thickness and diastolic dysfunction, which may in part reflect their increased age (77vs62). However, GLS was also significantly reduced compared to AL-amyloid, even when accounting for LV ejection fraction and LV mass, suggesting these composite parameters may have value in determining the subtype of cardiac amyloidosis.

Abstract 12952: Global Longitudinal Strain is Simple, Effective & Sensitive Assessment of Cardiac Chamber Function in Patients With Acute-phase Myocarditis

Circulation ◽  
2020 ◽  
Vol 142 (Suppl_3) ◽  
Author(s):  
Shaun Khanna ◽  
Aditya Bhat ◽  
Henry H Chen ◽  
Kennith Gu ◽  
Gary Gan ◽  
...  
Keyword(s):  
Acute Phase ◽  
Longitudinal Strain ◽  
Myocardial Dysfunction ◽  
Global Longitudinal Strain ◽  
Disease Process ◽  
Left Ventricular ◽  
Lv Function ◽  
Lv Mass ◽  
Echocardiographic Parameters ◽  
Cardiac Therapy

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.

scholarly journals 665 Longitudinal strain impairment in organic mitral regurgitation: look beyond the valve

2021 ◽  
Vol 23 (Supplement_G) ◽  
Author(s):  
Giovanni Diana ◽  
Laura Manfredonia ◽  
Monica Filice ◽  
Emanuele Ravenna ◽  
Francesca Graziani ◽  
...  
Keyword(s):  
Mitral Regurgitation ◽  
Ejection Fraction ◽  
Longitudinal Strain ◽  
Global Longitudinal Strain ◽  
Left Ventricular ◽  
Cardiac Damage ◽  
Lv Dysfunction ◽  
Lv Mass ◽  
Significant Difference ◽  
Lv Ejection Fraction

Abstract Aims Global longitudinal strain (GLS) is a hallmark of cardiac damage in mitral regurgitation (MR). GLS &gt; −18% in patients with severe organic MR (OMR) and normal LV ejection fraction (LVEF) is an independent predictor of postoperative LV dysfunction. While it is known that GLS is impaired in less than severe functional ischaemic MR (FMR), the value of GLS in less than severe OMR is not known. We aimed to determine prevalence and determinants of any GLS impairment in OMR, in comparison to FMR. Methods We retrospectively evaluated 51 consecutive patients (33 OMR and 18 FMR) with mild-to-moderate, moderate and moderate-to-severe MR (Table*). Overall, GLS was higher in OMR than FMR (17.9±4.5 vs. 10.3±5.3, P&lt;0.001), with rate of impairment of 45% in OMR and 89% in FMR (P= 0.0024). Results However, no significant difference was found in GLS between mild-to-moderate, moderate and moderate-to-severe MR patients within OMR (17.7±4.7 vs. 16.9±3.9 vs. 22.4±3, respectively, P&gt;0.05), as well as FMR (9.8±6.6 vs. 10.7±5.3 vs. 10.4±5.3, respectively, P&gt;0.05) groups. GLS correlated directly with left ventricular (LV) ejection fraction (EF) in both OMR (r=0.69, P&lt;0.001) and FMR (r=0.90, P&lt;0.001), and inversely with LV mass indexed for body surface area (LVMi) in both OMR (r = −0.50, P=0.005) and FMR (r = −0.48, P=0.042). While correlation with LVEF was better for FMR than OMR (Z − 1.95, P=0.026), correlation with LVMi was similar for OMR and FMR groups (Z − 0.082, P&gt;0.05). Conclusions In patients with OMR, GLS may be reduced, despite normal LVEF, in less than severe MR. Prevalence and degree of GLS impairment in OMR is less than in FMR. In OMR, as well as in FMR, GLS impairment is independent of entity of MR, but rather correlates with LVMi, maybe reflecting impact of myocardial fibrosis derived by increased LVMi on GLS.

Abstract 15734: Left Atrial Reservoir Strain as a Novel Prognostic Marker in Biopsy-proven Light-chain Amyloidosis

Circulation ◽  
2020 ◽  
Vol 142 (Suppl_3) ◽  
Author(s):  
Peter Huntjens ◽  
Kathleen Zhang ◽  
Yuko Soyama ◽  
Maria Karmpalioti ◽  
Daniel Lenihan ◽  
...  
Keyword(s):  
Light Chain ◽  
Left Atrial ◽  
Prognostic Value ◽  
Cardiac Amyloidosis ◽  
Longitudinal Strain ◽  
Global Longitudinal Strain ◽  
Strain Imaging ◽  
Left Ventricular ◽  
Lv Function ◽  
Al Amyloidosis

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.

Abstract 17994: Echocardiographic parameters of Left Ventricular Size and Function as Predictors of Symptomatic Heart Failure in Patients with Low Normal Ejection Fraction Treated with Anthracyclines

Circulation ◽  
2014 ◽  
Vol 130 (suppl_2) ◽  
Author(s):  
Negareh Mousavi ◽  
Timothy Tan ◽  
Mohammad Ali ◽  
Elkan F. Halpern ◽  
Lin Wang ◽  
...  
Keyword(s):  
Heart Failure ◽  
Ejection Fraction ◽  
Longitudinal Strain ◽  
Global Longitudinal Strain ◽  
Left Ventricular ◽  
Left Ventricular Ejection ◽  
Ventricular Ejection Fraction ◽  
Symptomatic Heart Failure ◽  
Echocardiographic Parameters ◽  
And Function

Objectives: The aim of this study was to assess whether baseline echocardiographic measures of left ventricular (LV) size and function predict the development of symptomatic heart failure or cardiac death (major adverse cardiac events, MACE) in patients treated with anthracyclines who have a pre-chemotherapy left ventricular ejection fraction (LVEF) in the low normal range (between 50-59%). Background: Anthracycline-induced symptomatic heart failure and impaired LVEF are late and often irreversible manifestations of anthracycline-induced cardiotoxicity. The value of echocardiographic parameters of myocardial size and function before chemotherapy to identify patients at high-risk for development of symptomatic heart failure in patients with low normal LVEF was studied. Methods: Patients with a LVEF between 50 and 59% before anthracyclines were selected. In these patients, LV volumes, LVEF and peak longitudinal strain (GLS) were measured. Individuals were followed for MACE and all-cause mortality over a median of 659 days (range; 3-3704 days). Results: Of 2234 patients undergoing echocardiography for pre-anthracycline assessment, 158 (7%) had a resting ejection fraction of 50-59%. Their average LV end-diastolic volume (LVEDV) was 101±22ml, LVEF was 54 ±3% and global longitudinal strain (GLS) was -17.7±2.6%. Twelve patients experienced a MACE (congestive heart failure) at a median of 173 days (range; 15-530). Age, diabetes, previous coronary artery disease, LVEDV, LVESV and GLS were all-predictive of MACE (P= 0.015, 0.0043 and 0.0065 for LVEDV, LVESV, and GLS respectively). LVEDV and GLS remained predictive of MACE when adjusted for age. Age and GLS were also predictive of overall mortality (p<0.0001 and 0.0105 respectively). Conclusions: In patients treated with anthracyclines with an LVEF of 50-59%, both baseline EDV and GLS predict the occurrence of MACE. These parameters may help target patients who could bene[[Unable to Display Character: &#64257;]]t from closer cardiac surveillance and earlier initiation of cardioprotective medical therapy.

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 ◽  
...  
Keyword(s):  
Cardiac Amyloidosis ◽  
Longitudinal Strain ◽  
Global Longitudinal Strain ◽  
Myocardial Strain ◽  
Posterior Wall ◽  
Volume Index ◽  
Danon Disease ◽  
Strain Pattern ◽  
Echocardiographic Parameters ◽  
Echocardiographic Images

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

scholarly journals Independent Value of Cardiac Troponin T and Left Ventricular Global Longitudinal Strain in Predicting All-Cause Mortality among Stable Hemodialysis Patients with Preserved Left Ventricular Ejection Fraction

2014 ◽  
Vol 2014 ◽  
pp. 1-12 ◽  
Author(s):  
Junne-Ming Sung ◽  
Chi-Ting Su ◽  
Yu-Tzu Chang ◽  
Yu-Ru Su ◽  
Wei-Chuan Tsai ◽  
...  
Keyword(s):  
Ejection Fraction ◽  
Cardiac Troponin ◽  
Longitudinal Strain ◽  
Troponin T ◽  
Global Longitudinal Strain ◽  
Hemodialysis Patients ◽  
Left Ventricular ◽  
Cardiac Troponin T ◽  
Clinical Parameters ◽  
Echocardiographic Parameters

Using a speckle-tracking echocardiography (STE), we recently demonstrated that a left ventricular (LV) global longitudinal strain (GLS) ≥ −15% and the serum cardiac troponin T (cTnT) concentration are associated with mortality in stable hemodialysis patients with preserved LV ejection fraction (LVEF). In this study, we explored the relationship between cTnT and echocardiographic parameters and evaluated whether the prognostic value provided by cTnT is independent of a GLS ≥ −15% and vice versa. Eighty-eight stable hemodialysis patients with preserved LVEF were followed for 31 months. STE studies and measurements of cTnT were performed at baseline. CTnT concentration had a modest correlation with GLS (rs=0.44;P<0.001) but had a weak or nonsignificant correlation with other echocardiographic parameters. Adjusting for clinical parameters, hazard ratios for each increase of 0.01 ng/mL in cTnT, and a GLS ≥ −15% on mortality were 1.13 (P=0.009) and 3.09 (P=0.03) without significant interaction between cTnT and GLS ≥ −15%. In addition, an increased cTnT concentration, a GLS ≥ −15%, or their combination showed significant additional predictive value for mortality when included in models consisting of clinical parameters. Therefore, both cTnT and a GLS ≥ −15% are independent predictors of mortality and are useful for risk stratification.

scholarly journals Improvement in Global Longitudinal Strain (GLS) Correlates with NT-Probnp Response in Patients with Cardiac Amyloidosis Treated on a Phase 1b Study of Anti-Amyloid Mab Cael-101

Blood ◽  
2018 ◽  
Vol 132 (Supplement 1) ◽  
pp. 958-958 ◽  
Author(s):  
Divaya Bhutani ◽  
Siyang Leng ◽  
Andrew Eisenberger ◽  
Mathew S. Maurer ◽  
Sofia Shames ◽  
...  
Keyword(s):  
Research Funding ◽  
Cardiac Amyloidosis ◽  
Longitudinal Strain ◽  
Cardiac Involvement ◽  
Pearson Correlation ◽  
Global Longitudinal Strain ◽  
Cardiac Response ◽  
Al Amyloidosis ◽  
Amyloid Deposits ◽  
Phase 1B

Abstract Background: Cardiac AL amyloidosis continues to have poor prognosis with median survival of less than a year from diagnosis and only 3 months in patients with cardiac stage 3b disease (1). Targeting the underlying plasma cell clone can clear circulating light chains but cannot remove deposited protein in the organs. CAEL-101 (11-1F4 mAb) is a monoclonal IgG1 antibody that directly binds to a conformational epitope present on both human kappa and lambda light-chain amyloid fibrils. In preclinical studies the antibody was able to localize to the amyloid tissue and led to decrease in size as well as elimination of the amyloid protein (2). An open-label phase 1b clinical trial of the CAEL-101 showed a promising organ response rate of 63% (3). Global Longitudinal Strain (GLS) is a sensitive measure of functional impairment in cardiac AL Amyloidosis, and may predict survival over and above that of cardiac biomarkers (4). Here, we evaluated the effects of CAEL-101 on the myocardial function using GLS in correlation with NT-proBNP in patients with cardiac amyloidosis treated with CAEL-101. Methods: Patients with relapsed/refractory AL amyloidosis were enrolled and treated in a phase 1b study (N=19) using the anti-amyloid mAb CAEL-101. CAEL-101 was administered weekly for 4 weeks with sequential doses of 0.5, 5, 10, 50, 100, 250 and 500 mg/m2 in a dose-escalation design. All patients underwent transthoracic echocardiograms at screening and 12 weeks. GLS was measured using speckle-tracking (TomTec-Arena 1.2, Germany) and calculated as an average of 4-, 2-, and 3- chamber based measurements. Paired student's t-test was used to compare echocardiographic variables at screening and 12 weeks after therapy start with CAEL-101. GLS was correlated with NT-proBNP using Pearson correlation coefficient. Results: The median age of patients (N=19) was 63 years with 68% male and 32% female. Ten out of 19 patients had cardiac involvement with a median NT-proBNP of 1186 (range 699-3964) at screening. Six out of 10 patients (60%) with cardiac involvement met cardiac response criteria by having a decrease in NT-proBNP >30% (3). Among echocardiographic parameters, mean GLS improved significantly in 9/10 patients from -15.58 ± -4.14% at screening to -17.37 ± -3.53% at week 12, p = 0.004 (Figure 1) of the trial. One patient who did not have improvement in GLS was dosed at low dose level 2 (5mg/m2) in the study. Under the null hypothesis, the probability of 9 or more patients improving without drug effect, is ~0.0107, suggesting that improvement of GLS in 9 of 10 is a highly unlikely outcome unless the there is a drug association. In contrast to the improved GLS in cardiac patients, CAEL-101 had no significant effect (p=0.4829) on GLS in the 9 patients without cardiac involvement (GLS -22.77 ± -3.12 at screening and -22.36 ± -3.02 at end of treatment), suggesting a specific effect of CAEL-101 on cardiac amyloid deposits (Figure 2 and Table 1). Pearson correlation coefficient between NT-proBNP response and GLS response (in 8 cardiac evaluable patients) was 0.345, further confirming the efficiency of Cael-101 in amyloid resolution resulting in structural remodeling of the heart muscle. Conclusion: Improvement in GLS correlates with improvement in NT-proBNP in in patients with cardiac AL Amyloidosis treated with CAEL-101. The short timeframe in which the improvement of the GLS occurred (12 weeks after entering the trial) suggests that this effect is Ab related and GLS along with NT-proBNP should be evaluated in larger studies as markers for early cardiac response in patients with AL Amyloidosis. References:Wechalekar AD, Schonland SO, Kastritis E et al. A European collaborative study of treatment outcomes in 346 patients with cardiac stage III AL amyloidosis. Blood.2013;121(17):3420-7.Wall JS, Kennel SJ, Stuckey AC et al. Radioimmunodetection of amyloid deposits in patients with AL amyloidosis. Blood. 2010 Sep 30;116(13):2241-4.Edwards CV, Gould J, Langer AL et al. Final Analysis of the Phase 1a/b Study of Chimeric Fibril-Reactive Monoclonal Antibody 11-1F4 in Patients with Relapsed or Refractory AL Amyloidosis. Blood 2017 130:509.Salinaro F, Meier-Ewert HK, Miller EJ et al. Longitudinal systolic strain, cardiac function improvement and survival following treatment of light-chain (AL) cardiac amyloidosis. Eur Heart J Cardiovasc Imaging.2017 Sep 1;18(9):1057-1064. *Dr. Lentzsch recused herself from the Phase 1a/b trial in 11/2017. Disclosures Maurer: Glaxo Smith kline: Other: personal fees ; Eidos: Other: Personal fees, Research Funding; Pfizer: Other: Personal fees, Research Funding; Prothena: Research Funding; Alnylam: Research Funding; Ionis: Other: Personal fees, Research Funding; Akcea: Other: Personal fees. Lentzsch:BMS: Consultancy; Caelum Biosciences: Consultancy, Other: Dr. Lentzsch recused herself as an investigator from the Phase 1a/b trial testing CAEL-101 in 11/2017., Patents & Royalties: Shareholder for Caelum Biosiences; Bayer: Consultancy; Janssen: Consultancy.

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