Artificial intelligence based left ventricular ejection fraction and global longitudinal strain in cardiac amyloidosis.

BACKGROUND: Assessment of left ventricular ejection fraction (LVEF) and global longitudinal strain (GLS) plays a key role in the diagnosis of cardiac amyloidosis (CA). However, manual measurements are time consuming and prone to variability. We aimed to assess whether fully automated artificial intelligence (AI) calculation of LVEF and GLS provide similar estimates and can identify abnormalities in agreement with conventional manual methods, in patients with pre-clinical and clinical CA. METHODS: We identified 51 patients (age 80 ± 10 years, 53% male) with confirmed CA according to guidelines, who underwent echocardiography before and/or at the time of CA diagnosis (median (IQR) time between observations 3.87 (1.93, 5.44 years). LVEF and GLS were quantified from the apical 2- and 4-chamber views using both manual and fully automated methods (EchoGo Core 2.0, Ultromics). Inter-technique agreement was assessed using linear regression and Bland-Altman analyses and two-way ANOVA. The diagnostic accuracy and time for detecting abnormalities (defined as LVEF ≤ 50% and GLS ≥ -15.1%, respectively) using AI was assessed by comparisons to manual measurements as a reference. RESULTS: There were no significant differences in manual and automated LVEF and GLS values in either pre-CA (p = .791 and p = .105, respectively) or at diagnosis (p = .463 and p = .722). The two methods showed strong correlation on both the pre-CA (r = .78 and r = .83) and CA echoes (r = .74 and r = .80) for LVEF and GLS, respectively. The sensitivity and specificity of AI-derived indices for detecting abnormal LVEF were 83% and 86%, respectively, in the pre-CA echo and 70% and 79% at CA diagnosis. The sensitivity and specificity of AI-derived indices for detecting abnormal GLS was 82% and 86% in the pre-CA echo and 100% and 67% at the time of CA diagnosis. There was no significant difference in the relationship between LVEF (p = .99) and GLS (p = .19) and time to abnormality between the two methods. CONCLUSION: Fully automated AI-calculated LVEF and GLS are comparable to manual measurements in patients pre-CA and at the time of CA diagnosis. The widespread implementation of automated LVEF and GLS may allow for more rapid assessment in different disease states with comparable accuracy and reproducibility to manual methods.

Echocardiographic Evaluation of the Athlete's Heart: Focused Review and Update.

PURPOSE OF REVIEW: The athlete's heart exhibits unique structural and functional adaptations in the setting of strenuous and repetitive athletic training which may be similarly found in pathologic states. The purpose of this review is to highlight the morphologic and functional changes associated with the athlete's heart, with a focus upon the insights that echocardiography provides into exercise-induced cardiac remodeling. RECENT FINDINGS: Recent studies are aiming to investigate the long-term effects and clinical consequences of an athlete's heart. The "gray-zone" continues to pose a clinical challenge and may indicate scenarios where additional imaging modalities, or longitudinal follow-up, provide a definitive answer. Echocardiography is likely to remain the first-line imaging modality for the cardiac evaluation of elite athletes. Multimodality imaging combined with outcome and long-term follow-up studies both during training and after retirement in both men and women may help further clarify the remaining mysteries in the coming years.

PR interval prolongation is significantly associated with aortic root abscess: An age- and gender-matched study.

BACKGROUND: Electrocardiographic abnormalities, such as PR interval prolongation, have been anecdotally reported in patients with aortic root abscess (ARA). An electrocardiographic marker may be useful in identifying those patients with aortic valve endocarditis who may progress to ARA. The objective of this study is to evaluate the change in the PR interval in patients with surgically confirmed ARA and compare it to age- and gender-matched controls with echocardiographically or surgically confirmed aortic valve endocarditis but without aortic root abscess and those hospitalized with diagnoses other than endocarditis. METHODS: Patients were eligible for enrollment if they were 18 years or older and were hospitalized for either ARA, aortic valve endocarditis, or for unrelated reasons and had at least one 12-lead electrocardiogram (ECG) prior to or on the day of hospitalization and at least one ECG after hospitalization but prior to any cardiac surgical procedure. Delta PR interval, defined as the difference between the pre- and post-admission PR interval, was the primary outcome of interest. The patients in the ARA group were age- and gender-matched to patients with aortic valve endocarditis and to those without endocarditis. Comparisons of demographic variables and study outcomes were performed. RESULTS: Eighteen patients with surgically confirmed ARA were enrolled. These patients were age- and gender-matched to 19 patients with aortic valve endocarditis and 18 patients with no past history or evidence of endocarditis during hospitalization. No difference was noted in the baseline PR interval between the groups. However, the PR interval following admission in the aortic root abscess group (201 ± 66 ms) was significantly longer than the PR interval in both the aortic valve endocarditis (162 ± 27 ms) (24%, p = .009) and no endocarditis (143 ± 24 ms) (40%, p < .001) groups. The primary outcome measure, delta PR interval, was significantly longer in the ARA group (35 ± 51 ms) than no endocarditis (-5 ± 17 ms) (p = .001) and aortic valve endocarditis groups (0.2 ± 18) (p = .003). CONCLUSIONS: The findings of our study support the notion that the PR interval is more likely to be prolonged in patients with ARA. Since ARA is associated with a high morbidity and mortality, PR interval prolongation in a patient with aortic valve endocarditis should prompt a thorough evaluation for aortic root involvement.

Degenerative mitral regurgitation.

PURPOSE OF REVIEW: Degenerative mitral regurgitation (DMR) continues to be an important cause of morbidity and mortality with surgical mitral valve repair remaining the gold standard for the treatment of severe disease. The purpose of this review is to summarize recent advances in the understanding of DMR as well as the progress made in its assessment with a focus on imaging techniques. RECENT FINDINGS: Recent insights into the anatomy and physiology of DMR challenge the assumption that fibroelastic deficiency and Barlow disease are part of a single DMR spectrum. Advances in echocardiography and cardiovascular MRI have the potential to improve quantification of mitral regurgitation, provide unique information on prognosis and impact of DMR, further the association between DMR and arrhythmic risk and aide in decision-making for DMR treatment. SUMMARY: With growing interest in the use of noninvasive transcatheter therapies in the mitral valve space, comprehensive assessment of the mitral valve is critical to instruct decision-making and guide therapeutic strategy.

A histopathologic schema to quantify the burden of cardiac amyloidosis: Relationship with survival and echocardiographic parameters.

BACKGROUND: Despite routine use of echocardiographic parameters to evaluate the severity of cardiac amyloidosis (CA), this methodology has not been well validated. We developed a histopathologic schema for quantifying CA burden and evaluated its relationship with clinical outcomes. Additionally, echocardiographic parameters were tested as potential noninvasive indices of CA burden. METHODS: We retrospectively studied 59 patients with CA (17 light chain, 42 transthyretin) who underwent endomyocardial biopsies. Light microscopy with staining was used to categorize CA burden as mild-to-moderate (<50%) or high (≥50%). Kaplan-Meier survival analysis was performed for the two groups. In 34 patients with good-quality echocardiograms, we measured left ventricular volumes, ejection fraction (EF), interventricular septal thickness (IVSt), posterior wall thickness (PWt), LV mass, lateral e'-velocity, and global longitudinal strain (GLS). These parameters were compared between the two groups. RESULTS: Thirty-five patients had mild-to-moderate and 24 severe amyloid burden. Kaplan-Meier curves demonstrated a trend toward worse mortality with high CA burden, which was more common and associated with higher mortality specifically in transthyretin-type patients. Echocardiography-derived IVSt, PWt, and LV mass were directly related to CA burden, while LV EF, e'-velocity, and GLS magnitude were inversely related to CA burden. CONCLUSIONS: Our findings provided a signal that CA burden is a clinically important entity with potentially valuable prognostic information. Echocardiographic parameters of LV anatomy and function correlate with histopathologic burden of CA, which is inversely related to survival. Further studies are needed to determine whether these parameters could be used as imaging biomarkers of treatment-related changes in CA burden.

Importance of the Left Atrium: More Than a Bystander?

Left atrial size and function parameters are associated with adverse outcomes in multiple disease states, including heart failure with reduced and preserved ejection fraction. Recent data suggest that phasic left atrial function and left atrial stain measurements also hold prognostic information. Three-dimensional echocardiography provides more accurate and reproducible quantification of left atrial volumes than 2-dimensional echocardiography when compared with cardiac magnetic resonance reference standards. Greater accessibility to these advanced imaging techniques allows for the integration of these parameters into routine clinical practice.

An introduction to left ventricular strain.

PURPOSE OF REVIEW: The assessment of left ventricular function by two-dimensional (2D) transthoracic echocardiography (TTE) is conventionally performed by measuring the ejection fraction, which has been shown to have important prognostic implications. However, left ventricular ejection fraction (LVEF) has notable shortcomings, including limited reproducibility, suboptimal inter/intraobserver variability and dependence on load/volume. Furthermore, subclinical left ventricular dysfunction cannot be measured with LVEF. With the advent of left ventricular deformation (strain) analysis, a new and robust means for assessing left ventricular function has emerged. RECENT FINDINGS: Contemporary research and guidelines have attempted to standardize the definition, acquisition and measurement of left ventricular strain. In addition, multiple studies have sought to establish normal values for left ventricular strain in addition to evaluating the benefits and prognostic value of strain assessment. SUMMARY: This article reviews the definition of left ventricular strain, outlines the types of strain and reviews how strain is acquired and measured. In addition, the advantages of strain analysis over LVEF as well as the incremental prognostic value of strain are examined. We further review the challenges associated with strain imaging as well as outline the future of strain imaging.

An insidious and deadly complication of mechanical chest compressions in a patient on anticoagulation and the subtle echocardiographic findings that enabled timely diagnosis.

Good-quality chest compressions improve outcomes in cardiac arrest. While manual chest compressions are suboptimal in this regard, the LUCAS device has been shown to improve the effectiveness of chest compressions during cardiopulmonary resuscitation (CPR). The complication rate associated with mechanical CPR, however, has not been adequately studied. Limited evidence suggests no difference in internal injury between manual and mechanical CPR. We report the case of a patient on anticoagulation who developed a mediastinal hematoma post mechanical CPR and on whom subtle findings on initial echocardiography could have alerted the clinician to this complication early during the clinical course. This case further suggests that there may be special populations of patients in whom we may need to be more vigilant in the use of mechanical CPR.

Residual native left ventricular function optimization using quantitative 3D echocardiographic assessment of rotational mechanics in patients with left ventricular assist devices.

Preservation of native left ventricular (LV) function in patients supported with LV assist device (LVAD) may be beneficial to attain optimal hemodynamics and enhance potential recovery. Currently, LVAD speed optimization is based on hemodynamic parameters, without considering residual native LV function. We hypothesized that alternatively, LV rotational mechanics can be quantified by 3D echocardiography (3DE), and may help preserve native LV function while optimizing LVAD speed. The goal of this study was to test the feasibility of quantifying the effects of LVAD implantation on LV rotational mechanics and to determine whether conventional speed optimization maximally preserves native LV function. We studied 55 patients with LVADs, who underwent 3DE imaging and quantitative analysis of LV twist. Thirty patients were studied before and after LVAD implantation. The remaining 25 patients were studied during hemodynamic ramp studies. The pump speed at which LV twist was maximal was compared with the hemodynamics-based optimal speed. LV twist decreased following LVAD implantation from 4.2 ± 2.7 to 2.3 ± 1.9° (P < 0.01), reflecting the constricting effects on native function. With lower pump speeds, no significant changes were noted in LV twist, which peaked at a higher speed. In 11/25 (44%) patients, the conventional hemodynamic/2DE methodology and 3DE assessment of maximal residual function did not indicate the same optimal conditions, suggesting that a higher pump speed would have better preserved native function. In conclusion, quantitative 3DE analysis of LV rotational mechanics provides information, which together with hemodynamics may help select optimal pump speed, while maximally preserving native LV function.

Reproducibility and experience dependence of echocardiographic indices of left ventricular function: Side-by-side comparison of global longitudinal strain and ejection fraction.

BACKGROUND: Although left ventricular (LV) ejection fraction (EF) and global longitudinal strain (GLS) are recommended by the current echocardiographic chamber quantification guidelines, these measurements are not performed routinely. Because EF measurements rely on manual tracing of LV boundaries, and are subject to inter-reader variability and experience dependence, we hypothesized that semiautomated GLS measurements using speckle tracking would be more reproducible and less experience-dependent. METHODS: Images from 30 patients were analyzed to obtain biplane EF using manual tracing. GLS was measured in three long-axis views using EchoInsight software (Epsilon Imaging) that automatically detects LV endocardial boundary, which is edited manually as necessary and is then automatically tracked throughout the cardiac cycle. All measurements were performed by an expert echocardiographer and three first-year cardiology fellows. RESULTS: Semiautomated GLS analysis showed excellent correlation (r=.98) and small bias (-1.0±13% of measured value) between the experienced and less experienced readers, superior to EF (r=.91, bias 7.3±16%). Also, in repeated measurements, GLS showed higher intra-class correlation (ICC=.98) than EF (ICC=.89). Additionally, GLS analysis required ~1 minute per patient, while biplane EF measurements took twice as long. CONCLUSIONS: Semiautomated GLS measurements are fast, less experience-dependent, and more reproducible than conventional EF measurements. This is probably because, irrespective of experience, the readers' choice of boundary position varies less when asked to refine the automated detection than to draw borders without initial clues. This technique may facilitate the workflow of a busy laboratory and make a step forward toward incorporating quantitative analysis into everyday echocardiography practice.

3D echocardiographic analysis of aortic annulus for transcatheter aortic valve replacement using novel aortic valve quantification software: Comparison with computed tomography.

BACKGROUND: With the increasing use of transcatheter aortic valve replacement (TAVR) in patients with aortic stenosis (AS), computed tomography (CT) remains the standard for annulus sizing. However, 3D transesophageal echocardiography (TEE) has been an alternative in patients with contraindications to CT. We sought to (1) test the feasibility, accuracy, and reproducibility of prototype 3DTEE analysis software (Philips) for aortic annular measurements and (2) compare the new approach to the existing echocardiographic techniques. METHODS: We prospectively studied 52 patients who underwent gated contrast CT, procedural 3DTEE, and TAVR. 3DTEE images were analyzed using novel semi-automated software designed for 3D measurements of the aortic root, which uses multiplanar reconstruction, similar to CT analysis. Aortic annulus measurements included area, perimeter, and diameter calculations from these measurements. The results were compared to CT-derived values. Additionally, 3D echocardiographic measurements (3D planimetry and mitral valve analysis software adapted for the aortic valve) were also compared to the CT reference values. RESULTS: 3DTEE image quality was sufficient in 90% of patients for aortic annulus measurements using the new software, which were in good agreement with CT (r-values: .89-.91) and small (<4%) inter-modality nonsignificant biases. Repeated measurements showed <10% measurements variability. The new 3D analysis was the more accurate and reproducible of the existing echocardiographic techniques. CONCLUSIONS: Novel semi-automated 3DTEE analysis software can accurately measure aortic annulus in patients with severe AS undergoing TAVR, in better agreement with CT than the existing methodology. Accordingly, intra-procedural TEE could potentially replace CT in patients where CT carries significant risk.

Cardiac MRI right ventricle / left ventricle (RV/LV) volume ratio improves detection of RV enlargement.

PURPOSE: To determine the normal range of the ratio of right ventricular (RV) end-diastolic volume to left ventricular (LV) end-diastolic volume by magnetic resonance imaging (MRI) and examine whether combining this volume ratio with RVEDV indexed to body surface area (RVEDVi) increased the detection of RV dilation in patients with pulmonary arterial hypertension (PAH). MATERIALS AND METHODS: MRI-derived ventricular function and volumes were measured in a control group (n = 152) and in patients with PAH (n = 46). Images were acquired with a 1.5T Siemens or a 1.5T Philips scanner using a steady-state free procession sequence. Proposed criteria for the detection of RV enlargement, including RVEDVi alone, RV/LV volume ratio alone, and combining both criteria, were evaluated in both groups. RESULTS: The range (mean ± 2 standard deviations) for the volume ratio in the normal population was found to be 0.906-1.266; there was no difference between genders (P = 0.70). Combining this ratio with RVEDVi detected RV enlargement in 21.7% (P < 0.001) PAH patients (volume ratio ≥1.27) who were not identified by the RVEDVi alone (>104 mL/m(2) for females and >113 mL/m(2) for males). CONCLUSION: Combining RV/LV volume ratio with indexed RVEDV increased detection of RV enlargement in a PAH population. This result may have potential impact in RV size assessment. J. Magn. Reson. Imaging 2016;43:1379-1385.

Echocardiographic Diagnosis of Acute Pulmonary Embolism in Patients with McConnell's Sign.

BACKGROUND: "McConnell's sign" (McCS), described as hypo- or akinesis of the right ventricular (RV) free wall with preservation of the apex, is associated with acute pulmonary embolism (aPE). However, the sensitivity of McCS for the detection of aPE is limited. We sought to evaluate in patients with McCS, whether echocardiographic parameters of global and regional RV function could differentiate between patients with and without aPE. METHODS: We reviewed echocardiograms of 81 patients with McCS, who underwent CT or V/Q studies for suspected PE, and 40 normal controls (NL). Echocardiograms were analyzed to measure pulmonary artery systolic pressure (PASP), tricuspid regurgitation (TR) by vena contracta width, conventional indices of RV function, and speckle tracking-derived longitudinal free wall strain. ROC analysis was performed to evaluate the diagnostic accuracy of these parameters for diagnosis of aPE. RESULTS: Fifty-five of eighty-one (68%) had PE (McCS + PE), while 26 of 81 (32%) did not (McCS - PE). Compared to NL, global and segmental RV strain were lower in patients with McCS, contrary to the notion of normal apical function. In McCS + PE, compared to McCS - PE: (1) PASP, fractional area change and TR were significantly lower; (2) strain magnitude was significantly lower globally and in basal and apical segments. Individual parameters had similar diagnostic accuracy by ROC analysis, which further improved by combining parameters. In McCS - PE, 69% of patients had pulmonary hypertension (PH). CONCLUSIONS: McCS and aPE are not synonymous. RV free wall strain may aid in differential diagnosis of patients with McCS evaluated for aPE. Specifically, McCS should prompt an inquiry for evidence of PH, which would indicate that aPE is less likely.

Improved detection of myocardial damage in sarcoidosis using longitudinal strain in patients with preserved left ventricular ejection fraction.

BACKGROUND: Cardiac infiltration is an important cause of death in sarcoidosis. Transthoracic echocardiography (TTE) has limited sensitivity for the detection of cardiac sarcoidosis (CS). Late gadolinium enhancement (LGE) cardiovascular magnetic resonance (CMR) is used to diagnose CS but has limitations of cost and availability. We sought to determine whether TTE-derived global longitudinal strain (GLS) may be used to identify individuals with CS, despite preserved left ventricular ejection fraction (LVEF), and whether abnormal GLS is associated with major cardiovascular events (MCE). METHODS: We studied 31 patients with biopsy-proven extra-cardiac sarcoidosis, LVEF>50% and LGE on CMR (CS+ group), and 31 patients without LGE (CS- group), matched by age, sex, and severity of lung disease. GLS was measured using vendor-independent speckle tracking software. Parameters of left and right ventricular systolic and diastolic function were also studied. Receiver-operating characteristic curves were used to identify GLS cutoff for CS detection, and Kaplan-Meier plots to determine the ability of GLS to predict MCE. RESULTS: LGE was associated with reduced GLS (-19.6±1.9% in CS- vs -14.7±2.4% in CS+, P<.01) and with reduced E/A ratio (1.1±0.3 vs 0.9±0.3, respectively, P =.01). No differences were noted in other TTE parameters. GLS magnitude inversely correlated with LGE burden (r=-.59). GLS cutoff of -17% showed sensitivity and specificity 94% for detecting CS. Patients who experienced MCE had worse GLS than those who did not (-13.4±0.9% vs -17.7±0.4%, P=.0003). CONCLUSIONS: CS is associated with significantly reduced GLS in the presence of preserved LVEF. GLS measurements may become part of the TTE study performed to screen for CS.

Early improvement in serial echocardiographic studies in heart failure patients predicts long term survival-a pilot study.

BACKGROUND: Several echocardiographic measures have prognostic value in heart failure (HF). However, no definitive data exist on how changes in these parameters with treatment affect survival in this patient population. We hypothesized that early improvement on echocardiography could predict long-term survival. METHODS AND RESULTS: We conducted a retrospective review of 404 patients seen in the HF clinic from 2002 to 2008 (6.5 years). Patients had one echocardiogram ≤1 year before and another ≥1 month (10 ± 7 months) after treatment onset. We studied changes in standard echocardiographic parameters, including left (LV) and right (RV) ventricular size and/or function (systolic and/or diastolic), valvular (mitral and tricuspid) function, and pulmonary artery pressure. Survival curves and hazard ratios were generated for patients showing improvement on the 2nd echocardiogram versus those who did not. Multivariable analyses were performed adjusting for age, sex, ischemic etiology, and significant baseline echocardiographic parameters. Average follow-up was 2.9 ± 1.5 years. Improvement in LV end-systolic dimension, RV function, and mitral regurgitation were independent predictors of 5-year survival (P < .05) and, importantly, more predictive than baseline values of these parameters alone (higher hazard ratios). CONCLUSIONS: Early echocardiographic improvement is strongly associated with 5-year survival in patients with HF. Serial echocardiography may aid in stratifying patient care.

Three-dimensional echocardiographic evaluation of longitudinal and non-longitudinal components of right ventricular contraction: results from the World Alliance of Societies of Echocardiography study.

AIMS: Right ventricular (RV) functional assessment is mainly limited to its longitudinal contraction. Dedicated three-dimensional echocardiography (3DE) software enabled the separate assessment of the non-longitudinal components of RV ejection fraction (EF). The aims of this study were (i) to establish normal values for RV 3D-derived longitudinal, radial, and anteroposterior EF (LEF, REF, and AEF, respectively) and their relative contributions to global RVEF, (ii) to calculate 3D RV strain normal values, and (iii) to determine sex-, age-, and race-related differences in these parameters in a large group of normal subjects (WASE study). METHODS AND RESULTS: 3DE RV wide-angle datasets from 1043 prospectively enrolled healthy adult subjects were analysed to generate a 3D mesh model of the RV cavity (TomTec). Dedicated software (ReVISION) was used to analyse RV motion along the three main anatomical planes. The EF values corresponding to each plane were identified as LEF, REF, and AEF. Relative contributions were determined by dividing each EF component by the global RVEF. RV strain analysis included longitudinal, circumferential, and global area strains (GLS, GCS, and GAS, respectively). Results were categorized by sex, age (18-40, 41-65, and >65 years), and race. Absolute REF, AEF, LEF, and global RVEF were higher in women than in men (P < 0.001). With aging, both sexes exhibited a decline in all components of longitudinal shortening (P < 0.001), which was partially compensated in elderly women by an increase in radial contraction. Black subjects showed lower RVEF and GAS values compared with white and Asian subjects of the same sex (P < 0.001), and black men showed significantly higher RV radial but lower longitudinal contributions to global RVEF compared with Asian and white men. CONCLUSION: 3DE evaluation of the non-longitudinal components of RV contraction provides additional information regarding RV physiology, including sex-, age-, and race-related differences in RV contraction patterns that may prove useful in disease states involving the right ventricle.

Right Ventricular Thrombus on Echocardiography.

Right ventricular thrombi (RVTs) have been almost exclusively studied in patients with pulmonary embolism (PE). The implications of an isolated RVT, a finding typically encountered on transthoracic echocardiography (TTE), are lacking. In this study, we sought to identify the echocardiographic and clinical features associated with the presence of RVTs. Between 1998 and 2023, 138 patients with RVT documented on TTE were retrospectively identified. Demographic data, presence of intracardiac devices, hypercoagulable conditions, history of deep vein thrombosis (DVT), PE, and/or left ventricular thrombus were abstracted from electronic chart review. Measurements of right and left ventricular size, and function were performed on TTE. Of the total population of patients with RVT, <1/2 had intracardiac devices, 29% had a documented hypercoagulable state (e.g., cancer or a clotting disorder). Most patients had dilated (77%) and dysfunctional (72%) right ventricles. Approximately 50% of RVTs were discovered in nonstandard imaging planes, suggesting that the presence of RVT is likely underestimated in clinical practice. Of those evaluated for PE, 80% had PE. Of those evaluated for DVT, 53% had DVT. In conclusion, further investigations are warranted to better guide when to investigate the right ventricle for RVTs on TTE and the impact of RVTs on patient outcomes.

Defining echocardiographic predictors of outcome in cardiac amyloidosis by subtype.

BACKGROUND: Current echocardiographic risk factors for prognosis in cardiac amyloidosis (CA) do not distinguish between the two main subtypes: transthyretin cardiomyopathy (TTR) and immunoglobulin light chain cardiomyopathy (AL), each of which require distinct diagnostic and therapeutic approaches. Additionally, only traditional parameters have been studied with little data on advanced techniques. Accordingly, we sought to determine whether differences exist in 2D transthoracic echocardiography (2DE) predictors of survival between the CA subtypes using a comprehensive approach. METHODS: 220 patients (72±12 years) with confirmed CA (AL=89, TTR=131) who underwent 2DE at the time of CA diagnosis were enrolled. Left ventricular (LV) dimensions, indexed mass (LVMi), global longitudinal strain (LVGLS), apical-sparing ratio (LVASR), diastology, right ventricular (RV) size and function indices including tricuspid annular systolic excursion (TAPSE), RV free-wall (RVFWS) and global (RVGLS) strain, indexed left (LA) and right atrial volumes (LAVi and RAVi), LA strain (reservoir and booster) and RV systolic pressure (RVSP) were measured. A propensity-score weighted stepwise variable selection Cox proportional hazards model derived from NYHA class and renal impairment status at diagnosis was used to determine the associations between 2DE parameters and mortality specific to CA subtype over a median follow-up of 36-months. RESULTS: After adjusting for age, atrial fibrillation and treatment, parameters associated with survival were RVFWS (p=0.003, HR 1.15, 95% CI[1.053,1.245]) and RVSP (p=0.03, HR 1.03, 95% CI[1.004,1.063]) in AL and LVASR (p=0.007, HR 6.68, 95% CI[1.75,25.492]) and RAVi (p=0.049, HR 1.03, 95% CI[1.000,1.052]) in TTR. CONCLUSIONS: Echocardiographic prognosticators for survival are specific to cardiac amyloid subtype. These results potentially provide information critical for clinical decision-making and follow-up in these patients.

Prevalence of valvular heart disease in cardiac amyloidosis and impact on survival.

BACKGROUND: Limited data exists on the prognostic impact of valvular heart disease in cardiac amyloidosis (CA). We therefore sought to define the prevalence of valvular disease in patients with CA and assess the effects of significant valve disease on survival. METHODS: This multi-center retrospective cohort study included consecutive patients with confirmed transthyretin (TTR) or light chain (AL) amyloidosis. Echocardiographic data closest to the date of amyloid diagnosis was reviewed, and severity was graded according to ASE guidelines. Kaplan-Meier survival analysis was performed to compare survival between patients with moderate or greater valve disease against those with mild or less disease. RESULTS: We included 345 patients (median age 76 years; 73 % men; 110 AL, 235TTR). The median survival for the total patient cohort with cardiac amyloidosis was 2.92 years, with 30 % of patients surviving at five years after their diagnosis. Median survival comparing AL vs ATTR was 2.58 years vs 2.82 years (p = 0.67) The most common valvular abnormalities in the total cohort were mitral (62 %) and tricuspid (66 %).regurgitation There was a statistically significant difference in median survival between patients with no or mild MR compared to those with moderate or severe MR (2.92 years vs 3.35 years, p = 0.0047) (Fig. 5). There was a statistically significant difference in median survival in patients with no or mild TR compared to those with moderate or severe TR (3.35 years vs 2.3 years, p = 0.015). CONCLUSION: Our study demonstrates a significant prevalence of mitral and tricuspid regurgitation in CA, with patients with moderate to severe MR and TR having a poorer prognosis.

Normative Values of Echocardiographic Chamber Size and Function in Older Healthy Adults: The Multi-Ethnic Study of Atherosclerosis.

BACKGROUND: Echocardiographic (2-dimensional echocardiography) thresholds indicating disease or impaired functional status compared with normal physiological aging in individuals aged ≥65 years are not clearly defined. In the present study, we sought to establish standard values for 2-dimensional echocardiography parameters related to chamber size and function in older adults without cardiopulmonary or cardiometabolic conditions. METHODS: In this cross-sectional study of 3032 individuals who underwent 2-dimensional echocardiography at exam 6 in the MESA (Multi-Ethnic Study of Atherosclerosis), 608 participants fulfilled our inclusion criteria of healthy aging, with normative values defined as the mean ± 1.96 standard deviation and compared across sex and race and ethnicity. Functional status measures included NT-proBNP (N-terminal pro-B-type natriuretic peptide), 6-minute walk distance, and Kansas City Cardiomyopathy Questionnaire. Prognostic performance using MESA cutoffs was compared with established guideline cutoffs using time-to-event analysis. RESULTS: The normative aging cohort (69.5±7.0 years, 46.2% male, 47.5% White) had lower NT-proBNP, higher 6-minute walk distance, and higher (better) Kansas City Cardiomyopathy Questionnaire summary values. Women had significantly smaller chamber sizes and better biventricular systolic function. White participants had the largest chamber dimensions, whereas Chinese participants had the smallest, even after adjustment for body size. Current guidelines identified 81.6% of healthy older adults in MESA as having cardiac abnormalities. CONCLUSIONS: Among a large, diverse group of healthy older adults, we found significant differences in cardiac structure and function by sex and race/ethnicity, which may signal sex-specific cardiac remodeling with advancing age. It is crucial for existing guidelines to consider the observed and clinically significant differences in cardiac structure and function associated with healthy aging. Our study highlights that existing guidelines, which grade abnormalities in echocardiographic cardiac chamber size and function based on younger individuals, may not adequately address the anticipated changes associated with normal aging.