Peripheral vascular involvement in transthyretin cardiac amyloidosis. A comparative analysis with AL amyloidosis

Abstract Background The pattern of peripheral vascular involvement in the wild type transthyretin-related cardiac amyloidosis (ATTRwt) and its diagnostic utility in differentiating this infiltrating cardiomyopathy from light chain (AL) cardiac amyloidosis (AL-CA) and heart failure with preserved ejection fraction (HFpEF) of different origin have not been explored. Aims To characterize the pattern of peripheral vascular involvement in ATTRwt and evaluate its value in differentiating ATTRwt from AL-CA and HFpEF. Methods Newly diagnosed patients with ATTRwt (n=42) were consecutively recruited from our amyloidosis center. These patients were matched 1:1 for age and sex to patients with AL-CA (n=32) and subjects without amyloidosis (n=32) and also matched 2:1 to HFpEF patients (n=16). All subjects underwent a series of non-invasive vascular examinations for the assessment of: 1. subclinical carotid atherosclerosis with B-mode ultrasonography, 2. Arterial stiffness with measurement of carotid-femoral pulse wave velocity, 3. Reactive vasodilation with flow-mediated dilation (FMD) and 4. Aortic blood pressures and arterial wave reflections with augmentation index (AI) and return time of reflected wave (Tr). Results ATTRwt patients had lower peripheral (pBP) and aortic blood pressure (aBP) markers compared to non-AL controls (p<0.05 for all). ATTRwt grouping was an independent determinant of these markers, after adjustment for cardiovascular risk factors (CVRF), including history of hypertension, hyperlipidemia and diabetes, glomerular filtration rate, body mass index and smoking status (core model). ATTRwt had lower aDBP and increased Tr compared to AL subjects. In a comparison between ATTRwt and AL patients with cardiac involvement, AI and Tr were higher and FMD lower in ATTRwt patients. ATTRwt was an independent determinant of these markers, after adjustment for the core model (p<0.05 for all). Compared to HFpEF, patients with ATTRwt had lower peripheral and central BP and higher Tr (p<0.05 for all). By ROC analysis, Tr provided high diagnostic value for ATTRwt vs. AL-CA (Area Under the Curve, AUC=0.809, CI: 0.65–0.96) and for ATTRwt vs combined AL-CA and HFpEF (AUC=0.880, CI: 0.79–0.97). Finally, AI was closely correlated with posterior (Spearman's Rho=−0.30) and intraventricular wall thickness (Rho=−0.329) and left ventricular global longitudinal strain (Rho=−0.4) and lower cDBP with higher Gilmore and New York Heart Association stage (p<0.05). Conclusion ATTRwt patients present differential characteristics of peripheral vascular function and aortic hemodynamics as compared to AL, HFpEF and healthy controls. The clinical value of these characteristics merit further investigation since differential diagnosis among amyloidosis types is clinically challenging, while it may have prognostic implications. FUNDunding Acknowledgement Type of funding sources: None.

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

Circulation ◽

10.1161/circ.142.suppl_3.15734 ◽

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.

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P1684 Cardiac amyloidosis hits the valve first

European Heart Journal - Cardiovascular Imaging ◽

10.1093/ehjci/jez319.1047 ◽

2020 ◽

Vol 21 (Supplement_1) ◽

Author(s):

S Iakovlev ◽

A I Kalinskaya

Keyword(s):

Heart Failure ◽

Case Report ◽

Tricuspid Valve ◽

Cardiac Amyloidosis ◽

Global Longitudinal Strain ◽

Left Ventricular ◽

Left Ventricular Ejection ◽

Al Amyloidosis ◽

Ventricular Ejection Fraction ◽

Arterial Blood

Abstract Case report. 60-year-old man without previous history of coronary artery disease was admitted to our hospital. He complained of weakness, low extremities edema. Physical examination revealed also enlargement of the liver, positive hepatojugular reflux, multiple ecchymoses on patient’s face, especially in periorbital area. The arterial blood pressure was 100/60 mm Hg. ECG showed sinus rhythm 83/min, low R waves in V2, V3 with biphasic T waves in V4-V6. The last coronary angiography revealed normal coronary arteries, it was performed 6 months ago because of atypical chest pain and inconclusive stress test. Echocardiography revealed severe tricuspid regurgitation (TR) due to leaflets restriction and malcoaptation. TR gradient was about 10 mm Hg. Interventricular septum 12 mm, left ventricle posterior wall 9 mm, mass index 87 g/m2, relative wall thickness 0.38; no LV regional wall motion abnormalities was noted, left ventricular ejection fraction (LV EF) was about 50%, mitral valve E/A ratio was 1,4, but average E/E" = 17. Left atrium volume 33 ml/m2. Insignificant amount of pericardial effusion also was found. 3D evaluation of tricuspid valve (TV) showed no leaflets defects and chordal ruptures. LV global longitudinal strain (LV GLS) was – 11,1 % with the apex/(mid + base) ratio 1,3 - apical sparing pattern. Cardiac MRI with gadolinium also showed severe TR and diffuse late subendocardial gadolinium enhancement in both ventricles. We suspected cardiac amyloidosis with significant tricuspid valve involvement, torrential TR, and right ventricle volume overload. The rectum biopsy was negative. The cardiac muscle biopsy with congo red straining was positive for amyloidosis. The patient was transferred to hematology clinic where the diagnosis of AL-amyloidosis was confirmed. The treatment with lenalidomide and prednisone was started. Unfortunately, one month later the patient died. The autopsy was not performed due to religious reasons. Discussion AL-amyloidosis is a systemic disease characterized by multiple organ and tissue changes and associated with poor prognosis. Cardiac involvement is a major prognostic factor as it accounts for approximately 75% of death due to heart failure or arrhythmias. Infiltration of myocardium with amyloid leads to diastolic than systolic dysfunction of the heart and to developing of the heart failure. The most common presentation of heart involvement in AL-amyloidosis is fatigue and dyspnea. In this case the main complaint of the patient was peripheral edema, echocardiography showed damaged TV and preserved LV systolic function. In literature, we found only one case report describing cardiac amyloidosis presented as severe TR. Conclusion In patients presenting with significant isolated valvular dysfunction and heart failure the cardiac amyloidosis can be suspected. The comprehensive echocardiography is the most useful tool to detect this problem. Abstract P1684 Figure. 3D picture of tricuspid valve

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Vascular-ventricular coupling during exercise is not affected by exaggerated blood pressures in endurance-trained athletes

Journal of Applied Physiology ◽

10.1152/japplphysiol.00108.2019 ◽

2019 ◽

Vol 127 (3) ◽

pp. 753-759 ◽

Cited By ~ 1

Author(s):

Katharine D. Currie ◽

Zion Sasson ◽

Jack M. Goodman

Keyword(s):

Blood Pressure ◽

Vascular Function ◽

Arterial Compliance ◽

Left Ventricular ◽

Left Ventricular Volumes ◽

Peripheral Vascular ◽

Arterial Elastance ◽

Coupling Index ◽

Cardiovascular Performance ◽

Stage 1

This study sought to examine whether cardiovascular performance during exercise, assessed using the vascular-ventricular coupling index (VVC), was affected by exaggerated blood pressure (EBP) responses in endurance-trained athletes. Subjects were middle-aged endurance-trained men and women. Blood pressure measurements and left ventricular echocardiography were performed in a semiupright position at rest and during steady-state cycling at workloads that elicited 100–110 beats/min ( stage 1) and 130–140 beats/min ( stage 2). These data were used to calculate effective arterial elastance index ( EaI), left ventricular end-systolic elastance index ( ELVI), and their ratio (VVC). Additional measurements of left ventricular volumes and function (i.e., stroke volume, cardiac output, and longitudinal strain) and indirect assessments of peripheral vascular function (i.e., total arterial compliance and peripheral vascular resistance) were examined. Fourteen subjects with EBP (EBP+, 50% men) and 14 sex-matched subjects without EBP (EBP−) participated, with results presented as EBP+ versus EBP−. EaI and ELVI increased from rest to exercise while VVC decreased, but only ELVI was different between groups at stage 1 [7.6 (1.8) vs. 6.4 (1.0) mmHg·ml−1·m−2, P = 0.045] and stage 2 [10.3 (1.6) vs. 8.0 (1.7) mmHg·ml−1·m−2, P < 0.001]. Additional comparisons revealed no group difference in the contribution of the Frank-Starling mechanism or left ventricular and peripheral vascular function during exercise. The cardiovascular adjustment to exercise in athletes with EBP is achieved through a matched increase in both EaI and ELVI, and the absence of between-group differences in left ventricular or peripheral vascular function suggests that other factors may contribute to the EBP response. NEW & NOTEWORTHY Cardiovascular performance during submaximal exercise, assessed using vascular-ventricular coupling, is unaffected by exaggerated blood pressure (EBP) responses in endurance-trained athletes. The underlying mechanisms of EBP in athletes remain unknown as changes in left ventricular and peripheral vascular function during exercise were similar in athletes with and without EBP.

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Can myocardial work indices contribute to the exploration of patients with cardiac amyloidosis?

Open Heart ◽

10.1136/openhrt-2020-001346 ◽

2020 ◽

Vol 7 (2) ◽

pp. e001346

Author(s):

Aénora Roger-Rollé ◽

Eve Cariou ◽

Khailène Rguez ◽

Pauline Fournier ◽

Yoan Lavie-Badie ◽

...

Keyword(s):

Cardiac Amyloidosis ◽

Characteristic Curve ◽

Global Longitudinal Strain ◽

Left Ventricular ◽

Peak Oxygen Consumption ◽

Left Ventricular Ejection ◽

Ventricular Ejection Fraction ◽

Echocardiographic Parameters ◽

Global Work ◽

Better Than

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.

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Effects of Continuous-Flow Left Ventricular Assist Device Therapy on Peripheral Vascular Function

ASAIO Journal ◽

10.1097/mat.0000000000001447 ◽

2021 ◽

Vol Publish Ahead of Print ◽

Author(s):

Ersin Kaya ◽

Umut Kocabas ◽

Evrim Simsek ◽

Sanem Nalbantgil ◽

Umit Kahraman ◽

...

Keyword(s):

Left Ventricular Assist Device ◽

Ventricular Assist Device ◽

Vascular Function ◽

Continuous Flow ◽

Left Ventricular ◽

Peripheral Vascular ◽

Device Therapy ◽

Assist Device ◽

Ventricular Assist ◽

Left Ventricular Assist

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Abstract 15928: Relative Sparing of Apical Longitudinal Strain for Detection of Cardiac Amyloidosis: Intervendor Variation

Circulation ◽

10.1161/circ.142.suppl_3.15928 ◽

2020 ◽

Vol 142 (Suppl_3) ◽

Author(s):

Julia M Simkowski ◽

Michael Jiang ◽

NADIA El HANGOUCHE ◽

Jeesoo Lee ◽

Milica Marion ◽

...

Keyword(s):

Cardiac Amyloidosis ◽

Longitudinal Strain ◽

Global Longitudinal Strain ◽

High Sensitivity ◽

High Specificity ◽

Left Ventricular ◽

Control Group ◽

Lower Sensitivity ◽

Medical Systems ◽

Roc Curve Analysis

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.

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Cardiovascular actions of beta-phenylethylamine

AJP Heart and Circulatory Physiology ◽

10.1152/ajpheart.1979.236.4.h592 ◽

1979 ◽

Vol 236 (4) ◽

pp. H592-H595

Author(s):

C. S. Liang ◽

D. Sprecher

Keyword(s):

Blood Pressure ◽

Vascular Resistance ◽

Left Ventricular ◽

Peripheral Vascular Resistance ◽

Adrenergic Nerve ◽

Peripheral Vascular ◽

Vascular Effects ◽

Aortic Blood ◽

Total Peripheral Vascular Resistance ◽

Aortic Blood Pressure

beta-Phenylethylamine increased mean aortic blood pressure, total peripheral vascular resistance, left ventricular dP/dt, and (dP/dt)/P in chloralose-anesthetized dogs. Pretreatment with phentolamine reduced the increases in aortic blood pressure and total peripheral vascular resistance produced by beta-phenylethylamine, whereas, the effects of beta-phenylethylamine on left ventricular dP/dt and (dP/dt)/P were abolished by propranolol. beta-Phenylethylamine pretreatment, but increased both after phentolamine pretreatment. Furthermore, both the cardiac and vascular effects of beta-phenylethylamine were abolished by desipramine. These results indicate that beta-phenylethylamine exerts both positive inotropic and vasoconstrictory effects, probably by releasing endogenous norepinephrine from the adrenergic nerve endings.

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P940 Usefulness of the subendocardial and subepychardial longitudinal strain in the differential diagnosis between cardiac amyloidosis and hypertrophic myocardiopathy

European Heart Journal - Cardiovascular Imaging ◽

10.1093/ehjci/jez319.573 ◽

2020 ◽

Vol 21 (Supplement_1) ◽

Author(s):

R Ramos Polo ◽

S Moral Torres ◽

C Tiron De Llano ◽

M Morales Fornos ◽

J M Frigola Marcet ◽

...

Keyword(s):

Differential Diagnosis ◽

Transthoracic Echocardiography ◽

Cardiac Amyloidosis ◽

Longitudinal Strain ◽

Global Longitudinal Strain ◽

Left Ventricular ◽

Left Ventricular Ejection ◽

Deformation Analysis ◽

Ventricular Ejection Fraction ◽

Level Increase

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 >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

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Deep learning to diagnose cardiac amyloidosis from CMR findings

European Heart Journal ◽

10.1093/ehjci/ehaa946.0211 ◽

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

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

European Heart Journal - Cardiovascular Imaging ◽

10.1093/ehjci/jez319.162 ◽

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.

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