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Bostan, Cem, Umit Yasar Sinan, Polat Canbolat, and Serdar Kucukoglu. "Cardiac Amyloidosis Cases with Relative Apical Sparing of Longitudinal Strain." Echocardiography 31, no. 2 (2013): 241–44. http://dx.doi.org/10.1111/echo.12395.
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Sivapathan, S., A. Boyd, T. Deshmukh, et al. "Relative Apical Sparing Using Longitudinal Strain to Diagnose Cardiac Amyloidosis." Heart, Lung and Circulation 27 (2018): S270. http://dx.doi.org/10.1016/j.hlc.2018.06.505.
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Rapezzi, Claudio, and Marianna Fontana. "Relative Left Ventricular Apical Sparing of Longitudinal Strain in Cardiac Amyloidosis." JACC: Cardiovascular Imaging 12, no. 7 (2019): 1174–76. http://dx.doi.org/10.1016/j.jcmg.2018.07.007.
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Ishida, Miwa, Yoko Yamada, Tomohiro Mizutani, et al. "Cardiac Amyloidosis Mimicking Dilated Cardiomyopathy But Showing Relative Apical Sparing of Longitudinal Strain." Circulation Journal 82, no. 12 (2018): 3102–3. http://dx.doi.org/10.1253/circj.cj-18-0070.
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Fikrle, Michal, Tomas Palecek, Josef Marek, Petr Kuchynka, and Ales Linhart. "Simplified apical four-chamber view evaluation of relative apical sparing of longitudinal strain in diagnosing AL amyloid cardiomyopathy." Echocardiography 35, no. 11 (2018): 1764–71. http://dx.doi.org/10.1111/echo.14132.
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Saito, Makoto, Misaki Imai, Daisuke Wake, et al. "Semiquantitative assessment of the relative apical sparing pattern of longitudinal strain for cardiac amyloidosis identification." Echocardiography 37, no. 9 (2020): 1422–29. http://dx.doi.org/10.1111/echo.14833.
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Saito, Makoto, Misaki Imai, Daisuke Wake, et al. "Prognostic assessment of relative apical sparing pattern of longitudinal strain for severe aortic valve stenosis." IJC Heart & Vasculature 29 (August 2020): 100551. http://dx.doi.org/10.1016/j.ijcha.2020.100551.
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Sawada, Naoko, Masao Daimon, Hiroyuki Abe, et al. "An Autopsy Case of Cardiac Amyloidosis with Heterogeneous Deposition of Amyloid Protein: A Possible Mechanism for Relative Apical Sparing of Longitudinal Strain." CASE 4, no. 2 (2020): 54–56. http://dx.doi.org/10.1016/j.case.2019.09.009.
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Phelan, Dermot, Patrick Collier, Paaladinesh Thavendiranathan, et al. "Relative apical sparing of longitudinal strain using two-dimensional speckle-tracking echocardiography is both sensitive and specific for the diagnosis of cardiac amyloidosis." Heart 98, no. 19 (2012): 1442–48. http://dx.doi.org/10.1136/heartjnl-2012-302353.
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Bravo, Paco E., Kana Fujikura, Marie Foley Kijewski, et al. "Relative Apical Sparing of Myocardial Longitudinal Strain Is Explained by Regional Differences in Total Amyloid Mass Rather Than the Proportion of Amyloid Deposits." JACC: Cardiovascular Imaging 12, no. 7 (2019): 1165–73. http://dx.doi.org/10.1016/j.jcmg.2018.06.016.
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Domingues, Kevin, Mariana Saraiva, Liliana Marta, Isabel Monteiro, and Margarida Leal. "Light chain cardiac amyloidosis - a rare cause of heart failure in a young adult." Revista da Associação Médica Brasileira 64, no. 9 (2018): 787–90. http://dx.doi.org/10.1590/1806-9282.64.09.787.
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SUMMARY Cardiac amyloidosis is an infiltrative cardiomyopathy, resulting from amyloid deposition within the myocardium. In primary systemic (AL-type) amyloidosis, the amyloid protein is composed of light chains resulting from plasma-cell dyscrasia, and cardiac involvement occurs in up to 50% of the patients We present a case of a 43-year-old man, with complaints of periodical swollen tongue and xerostomia, bleeding gums and haematuria for two months. His blood results showed normocytic anaemia, thrombocytopenia and a high spontaneous INR, therefore he was referred to the Internal Medicine clinic. In the first visit, he showed signs and symptoms of overt congestive heart failure and was referred to the emergency department. The electrocardiogram showed sinus tachycardia and low voltage criteria. Echocardiography showed biventricular hypertrophy with preserved ejection fraction, restrictive physiology with elevated filling pressures, thickened interatrial septum and atrioventricular valves, small pericardial effusion and relative “apical sparing” on 2D longitudinal strain. Cardiac MRI showed diffuse subendocardial late enhancement. Serum protein electrophoresis was inconclusive, however urine analysis revealed nephrotic range proteinuria, positive Bence Jones protein and an immunofixation test with a monoclonal lambda protein band. Abdominal fat biopsy was negative for Congo red stain, nevertheless a bone marrow biopsy was performed, revealing lambda protein monoclonal plasmocytosis, confirming the diagnosis of primary systemic amyloidosis. This case represents a rare cause of heart failure in a young adult. Low-voltage QRS complexes and typical echocardiography features should raise the suspicion for cardiac amyloidosis. Prognosis is dictated by the level of cardiac involvement; therefore, early diagnosis and treatment are crucial.
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Gil, J., L. Abreu, H. Antunes, et al. "Apical sparing of longitudinal strain in speckle-tracking echocardiography." Netherlands Heart Journal 26, no. 12 (2018): 635. http://dx.doi.org/10.1007/s12471-018-1146-9.
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Zhang, Kathleen W., Justin S. Sadhu, Brent W. Miller, et al. "Apical Sparing Pattern of Longitudinal Strain and Positive Bone Scintigraphy in Metastatic Myocardial Calcification." JACC: Case Reports 2, no. 5 (2020): 809–13. http://dx.doi.org/10.1016/j.jaccas.2020.02.018.
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Tjahjadi, Catherina, Robert Adam, Philippe Debonnaire, et al. "CONSTRUCTIVE WORK AND LONGITUDINAL STRAIN-DERIVED APICAL SPARING PATTERN DIFFERENTIATE CARDIAC AMYLOIDOSIS FROM HYPERTROPHIC CARDIOMYOPATHY." Journal of the American College of Cardiology 77, no. 18 (2021): 1409. http://dx.doi.org/10.1016/s0735-1097(21)02767-4.
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Zhang, Kathleen, Ray Zhang, Elena Deych, et al. "GLOBAL LONGITUDINAL STRAIN IS PROGNOSTIC IN LIGHT CHAIN AND TRANSTHYRETIN CARDIAC AMYLOIDOSIS WHILE THE APICAL SPARING RATIO OF LONGITUDINAL STRAIN IS NOT." Journal of the American College of Cardiology 75, no. 11 (2020): 1657. http://dx.doi.org/10.1016/s0735-1097(20)32284-1.
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Lwin, M., N. Edwards, K. Koitka, et al. "Spectrum of Differential Left Ventricular Strain from Relative Apical Sparing in Cardiac Amyloidosis to Basal Sparing in Aortic Stenosis." Heart, Lung and Circulation 27 (2018): S274. http://dx.doi.org/10.1016/j.hlc.2018.06.514.
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Jamal, Fadi, Cyrille Bergerot, Laurent Argaud, Joseph Loufouat, and Michel Ovize. "Longitudinal strain quantitates regional right ventricular contractile function." American Journal of Physiology-Heart and Circulatory Physiology 285, no. 6 (2003): H2842—H2847. http://dx.doi.org/10.1152/ajpheart.00218.2003.
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The assessment of contractile function of the right ventricle (RV) is an important clinical issue, but this remains difficult because of its complex anatomy and structure. We thought to investigate whether new Doppler-derived myocardial deformation indexes may quantify regional contractile RV function during varying loading conditions. In nine pigs, ultrasonic crystals were inserted longitudinally in the RV inflow and outflow tracts to assess regional contractile function. The same RV segments and the interventricular septum were imaged using apical echocardiographic views. Regional function was assessed using two parameters: 1) systolic strain (SS), representing the relative magnitude of segmental systolic shortening; and 2) its temporal derivative, peak systolic strain rate (SR), i.e., the maximal velocity of segmental shortening. Data were acquired at baseline and during partial pulmonary artery constriction (PAC) and inferior vena cava occlusion (IVCO). SS decreased significantly after PAC and IVCO in both the inflow and outflow tracts but only during IVCO in the septum. SR was less sensitive to loading variations in all segments. A significant correlation was found between SS values derived from sonomicrometry and myocardial Doppler in RV segments ( r = 0.84, P < 0.001). Thus regional strain and SR provide complementary information on the heterogeneous RV contractile function and can be accurately and noninvasively quantified using Doppler myocardial imaging.
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Godlewski, Krzysztof, Paweł Dryżek, Elżbieta Sadurska, and Bożena Werner. "Left ventricular systolic function impairment in children after balloon valvuloplasty for congenital aortic stenosis assessed by 2D speckle tracking echocardiography." PLOS ONE 16, no. 4 (2021): e0248862. http://dx.doi.org/10.1371/journal.pone.0248862.
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Aims The aim of the study was to evaluate left ventricular (LV) remodeling and systolic function using two-dimensional speckle tracking echocardiographic (2D STE) imaging in children at a long-term (more than 36 months, 107.5±57.8 months) after balloon valvuloplasty for aortic stenosis (BAV). Methods and results 40 patients (mean age 9,68 years, 75% male) after BAV and 62 control subjects matched to the age and heart rate were prospectively evaluated. The 2D STE assessment of LV longitudinal and circumferential strain and strain rate was performed. Left ventricular eccentric hypertrophy (LVEH) was diagnosed in 75% of patients in the study group. Left ventricular ejection fraction (LVEF) was normal in all patients. In study group, global longitudinal strain (GLS), global longitudinal strain rate (GLSr) were significantly lower compared with the controls: GLS (-19.7±2.22% vs. -22.3±1.5%, P< 0.001), GLSr (-0.89±0.15/s vs. -1.04 ±0.12/s, P < 0.001). Regional (basal, middle and apical segments) strain and strain rate were also lower compared with control group. Global circumferential strain (GCS), global circumferential strain rate (GCSr) as well as regional (basal, middle and apical segments) strain and strain rate were normal. Multivariable logistic regression analysis included: instantaneous peak systolic Doppler gradient across aortic valve (PGmax), grade of aortic regurgitation (AR), left ventricular mass index (LVMI), left ventricular relative wall thickness (LVRWT), left ventricular end-diastolic diameter (LVEDd), peak systolic mitral annular velocity of the septal and lateral corner (S’spt, S’lat), LVEF before BAV and time after BAV and showed that the only predictor of reduced GLS was LV eccentric hypertrophy [odds ratio 6.9; (95% CI: 1.37–12.5), P = 0.045]. Conclusion Patients at long-term observation after BAV present the subclinical LV systolic impairment, which is associated with the presence of its remodeling. Longitudinal deformation is the most sensitive marker of LV systolic impairment in this group of patients.
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Hirata, Yukina, Kenya Kusunose, Hirokazu Miki, and Hirotsugu Yamada. "Improvement of global longitudinal strain following high-dose chemotherapy and autologous peripheral blood stem cell transplantation in patients with amyloid light-chain cardiac amyloidosis: a case report." European Heart Journal - Case Reports 3, no. 4 (2019): 1–6. http://dx.doi.org/10.1093/ehjcr/ytz225.
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Abstract Background Cardiac amyloidosis (CA) is a secondary form of cardiomyopathy where abnormal accumulation of amyloid protein in the myocardial interstitium causes cardiac hypertrophy and myocardial fibrosis. If primary CA advances to heart failure, most patients do not survive for very long after the diagnosis. Case summary A 40-year-old man was admitted to our hospital for dyspnoea, progressive anaemia, and decreased appetite. He has diagnosed with amyloid light-chain (AL) amyloidosis. Although BD treatment (bortezomib + dexamethasone) and medical treatment were started, there was no sign of improvement. Then, high-dose chemotherapy followed by autologous peripheral blood stem cell transplantation (auto-PBSCT) was initiated. Pretreatment echocardiography revealed typical findings of CA, such as ventricular wall thickening, valvular thickening, diastolic dysfunction, and pericardial effusion. Global longitudinal strain (GLS) was significantly reduced, and bull's-eye mapping showed typical apical sparing. After auto-PBSCT, GLS gradually improved and was almost normal after 2 years. Other echocardiographic parameters, functional status, and laboratory data also showed that there was significant regression of CA. Discussion Although the prognosis in primary CA is extremely poor, we achieved long-term survival in a patient with effective high-dose chemotherapy and auto-PBSCT. Global longitudinal strain may be a useful marker of prognosis, regression, and recovery.
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Usuku, Hiroki, Seiji Takashio, Eiichiro Yamamoto, et al. "Usefulness of relative apical longitudinal strain index to predict positive 99m Tc‐labeled pyrophosphate scintigraphy findings in advanced‐age patients with suspected transthyretin amyloid cardiomyopathy." Echocardiography 37, no. 11 (2020): 1774–83. http://dx.doi.org/10.1111/echo.14892.
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Miyagawa, Sawa, Tadashi Miyamoto, and Yukihito Sato. "Soluble tumour necrosis factor-alpha receptor improved the function, hypertrophy, and granular sparkling appearance of the left ventricular myocardium in systemic amyloid A amyloidosis: a case report." European Heart Journal - Case Reports 4, no. 3 (2020): 1–7. http://dx.doi.org/10.1093/ehjcr/ytaa048.
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Abstract Background About 7% of amyloid A (AA) amyloidosis cases are accompanied by heart disease. Although several studies have recently reported that specific biologicals improved renal function in AA amyloidosis, little evidence is available regarding heart disease in AA amyloidosis. Case summary A 57-year-old woman with rheumatoid arthritis presented with sudden worsening of renal function. Echocardiography revealed granular sparkling appearance in the ventricular septum and posterior wall (PW). Echocardiography indicated left ventricular (LV) diastolic dysfunction. Global longitudinal strain (GLS) exhibited an apical sparing pattern. Cardiac biopsy demonstrated amyloid A deposition on immunostaining. Soluble tumour necrosis factor-alpha receptor etanercept therapy was initiated. Four years later, echocardiography showed improved diastolic function, including E/A and E/e’, and decreased wall thickness in both the interventricular septum and PW of the left ventricle. Granular sparkling appearance had diminished. Moreover, the LV dysfunction improved on GLS. Five years later, the medication was gradually losing effect and the patient had worsening pain in the joints; moreover, articular destruction was observed on radiography. The patient was switched to abatacept therapy. Echocardiography showed recurrence of LV hypertrophy and electrocardiogram showed down-sloped ST depression in V4–6 leads. Discussion This case indicates that etanercept can be effective for heart disease in AA amyloidosis. Of particular, interest is the improvement of granular sparkling appearance in addition to cardiac function improvement noted in this case.
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Khamis, Hanan, Sara Shimoni, Andreas Hagendorff, Nahum Smirin, Zvi Friedman, and Dan Adam. "Feasibility of reproducible vendor independent estimation of cardiac function based on first generation speckle tracking echocardiography." Journal of Biomedical Engineering and Informatics 2, no. 2 (2015): 57. http://dx.doi.org/10.5430/jbei.v2n2p57.
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Background: The clinical approval of speckle tracking echocardiography (STE) as an accepted measure of myocardial strain and of LV function is hindered by the discordance of the results among the vendors. Since echocardiography images are noisy, the measured displacements are smoothed or regularized, an operation affecting the strain results. We introduce an “Error-dependent weighted speckle tracking” (EWST) algorithm that allows sensitivity analysis to the different operations affecting noise and accuracy. The aim here was to study whether by modifying the properties of the post block-matching weighted smoothing in the EWST algorithm it was possible to assess the expected inter-vendor strain differences. Methods and results: 48 echocardiographic clips generated by a software-based phantom were used as “gold standard” for validation of the EWST algorithm. Also, a cohort of 435 normal subjects and a cohort of 47 patients, scanned/re- scanned at 2 frame-rates (~70; ~35), were studied using the EWST. The results were compared to those produced by a commercial product of a leading manufacturer (STELV). Peak global longitudinal strains [PRLS, (%)] and peak regional longitudinal strains [PRLS, (%)] were calculated and compared. Sensitivity to the region (ROI) determination was tested by shifting the apical endocardial boundary. The differences between the measured PGLS and the ground truth produced by the software-based phantom (average ± standard deviation) were 0.4% ± 0.6% and 1.0% ± 0.7% for the EWST and STELV, respectively. Normal values were calculated for 435 subjects: -18.82% ± 2.45%, -20.2% ± 5.6%, -19.62% ± 3.62%, 18.77% ± 4.31% by the EWST, and -21.24% ± 2.91%, -26.5% ± 5.0%, -21.1% ± 3.7%, -18.0% ± 3.9%, by the STELV, respectively, for the PGLS, the peak longitudinal apical, mid-ventricle and basal regions, respectively. A low bias, but significant, was found between PGLS, when calculated for the cohort of 47 patients scanned/re-scanned at 2 frame-rates: -0.80% ± 2.61% and -1.66% ± 2.66% for the EWST and STELV, respectively. When the apex location (and thus the ROI) was shifted, the bias (mm) (average ± standard deviation) relative to the default position was: 0.82 ± 1.04; 0.61 ± 0.72; -1.06 ± 0.75; and -1.87 ± 0.72, for displacement of 5 cm, 2.5 cm, -2.5 cm, and -5 cm, respectively, for the STELV. The EWST proved similarly sensitive to the shifting of the apex location. Conclusions: STE is sensitive to the characteristics and amount of smoothing, as well as to the ROI positioning. Modification of the smoothing can produce different stain results, and different distribution of the regional strains. Thus it is preferable to use automatic determination of the ROI and methods that employ minimal smoothing or regularization.
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Cappellini, Iacopo, Alessandra Melai, Lucia Zamidei, Maddalena Parise, Simone Cipani, and Guglielmo Consales. "Levosimendan and Global Longitudinal Strain Assessment in Sepsis (GLASSES 1): a study protocol for an observational study." BMJ Open 10, no. 9 (2020): e037188. http://dx.doi.org/10.1136/bmjopen-2020-037188.
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IntroductionCardiogenic shock is a condition of low cardiac output that represents the end stage of a progressive deterioration of cardiac function. The main cause is ischaemic heart disease, but there are several non-ischaemic causes, including sepsis. The use of levosimendan in cardiogenic shock during sepsis is still under debate.MethodsWe are conducting an observational, single-centre, not-for-profit study enrolling patients aged 18–80 years old admitted to the intensive care unit with a diagnosis of septic shock. Patients will be monitored with the EV1000/VolumeView device (Edwards Lifesciences, Irvine, USA). Patients with cardiac index (CI) values <2.5 L/min/m2 and/or stroke volume index (SVI) <30 mL/beat/m2 are considered eligible for the study. Enrolled participants will undergo an echocardiographic examination using the Vivid S6 ultrasound machine (General Electric, Northville, Michigan) and a 3.6 MHz cardiology probe through which the apical projections of chambers 2, 3 and 4 will be acquired; this is necessary to calculate the global longitudinal strain (GLS) using EchoPAC* Clinical Workstation Software (General Electric). A dobutamine infusion will be started in these patients; 24 hours later CI and SVI will be recalculated using EV1000/VolumeView and then a levosimendan infusion will begin for 24 hours. Once the infusion cycle of the calcium-sensitising drug has been carried out, the infusion of dobutamine will be reduced until it stops, and the CI, SVI, GLS and arterial elastance (Ea):Ventricular Elastance (Ees) will be re-evaluated. The primary endpoint is recovery of GLS ≥15% and the secondary endpoint is a relative reduction in mortality of 15%.Ethics and disseminationThe investigators declare that the study will be conducted in full compliance with international regulations (EU Directive 2016/679/EC) and national implementation (DM 15 July 1997; 211/2003; 200/2007) regarding the clinical trial and the principles of the Declaration of Helsinki. Study results will be disseminated through peer-reviewed journals and conferences. Ethical approval for this study has been given by Comitato Etico Regione Toscana - Area Vasta Centro, Florence, Italy (ethical committee number: 13875_oss) on 25 May 2019 (Chairperson Professor Marco Marchi).Trial registration numberNCT04141410.
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Li, Jiahui, Aili Li, Jiali Wang, Yu Zhang, and Ying Zhou. "Early Left Ventricular Dysfunction Detected by Speckle Tracking in Long-Term Hemodialysis Patients with Valvular Calcification." Cardiorenal Medicine 9, no. 1 (2018): 22–30. http://dx.doi.org/10.1159/000491679.
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Purpose: Cardiac valve calcification (VC) is very common in patients on hemodialysis. However, the definite effect of VC on left ventricular (LV) geometry and function in this population is unknown, especially when LV ejection fraction (LVEF) is normal. The aim of this study was to determine the effect of VC on LV geometry and function in long-term hemodialysis patients by conventional echocardiography and two-dimensional speckle tracking echocardiography (2D-STE). Methods: A total of 47 hemodialysis patients (2–3 times weekly for 5 years or more) were enrolled in this study. Cardiac VC was defined as bright echoes of more than 1 mm on one or more cusps of the aortic valve or mitral valve or mitral annulus using echocardiography as the screening method. LV longitudinal global strain (GLS) was assessed on the apical four-chamber view and calculated as the mean strain of 6 segments. LV global circumferential strain was acquired on the LV short axis view at the level of papillary muscles. Results: Twenty-five patients with VC had higher mean values of interventricular septum thickness, LV posterior wall thickness, LV mass index, relative wall thickness, and LV mass/end-diastolic volume than 22 patients without VC (p < 0.05, respectively), indicating more obvious LV hypertrophy (LVH). VC patients had higher mitral annular E/E′ values, especially at the septal side representing increased LV filling pressure compatible with diastolic dysfunction, while only the E/E′ ratio of the septal side was significantly different between the 2 groups (16.7 ± 4.1 vs. 12.3 ± 4.4, p < 0.01). When assessed by GLS, LV longitudinal systolic function was also lower in in patients with VC compared with those without VC (–0.18 ± 0.03 vs. –0.25 ± 0.04; p < 0.01). Conclusions: Cardiac VC diagnosed by echocardiography when it occurs in long-term hemodialysis patients may indicate more severe LVH, myocardial damage, and worse heart function in comparison to those without VC. Tissue Doppler imaging and 2D-STE can detect the subtle change of heart function in this population in the early stage of LV dysfunction when LVEF is normal.
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Nevzorova, V. A., N. V. Zakharchuk, E. U. Shapkina, E. A. Kondrashova, and D. V. Kondrashov. "COPD and preclinical cardiovascular disease." South Russian Journal of Therapeutic Practice 2, no. 2 (2021): 70–79. http://dx.doi.org/10.21886/2712-8156-2021-2-2-70-79.
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Objective: to assess cerebral blood flow and reveal early myocardial remodeling in COPD patients with varying degrees of airflow restriction. Materials and methods: the research included 105 patients with COPD from 1 to 4 degrees of severity, depending on the degree of restriction of FEV1 without CVD, diabetes mellitus, chronic kidney disease, obesity, other systemic and oncological diseases. Average age was 57.12 ± 0.68 years, men 45%. 5 groups were identified: mild severity of COPD (GOLD1, = 24), moderate (COLD2, n = 39), severe (GOLD3, n = 30), very severe (GOLD4, n = 12). Control group (n = 37) was tobacco free and CVD. Blood pressure and ultrasound tracranial dopplerography were performed in all groups. Transtoral echocardiography with assessment of global and local LV longitudinal deformation by the strain method and determination of left ventricular diastolic dysfunction (DDLV) was performed in GOLD1 and GOLD2 groups. Parameters of average values of deformation in basal, medial and apical segments are evaluated. Results were processed with Microsoft Excel 2016 and STATISTICA 10 (StatSoft, Inc., USA). Results: arterial hypertension (AH) was detected in 56.4% of patients in the COLD2 group; 56.7% of patients in the GOLD3 group and 100% of patients in the GOLD4. Сhanges in cerebral blood flow were not found in the GOLD1-3 groups. Significant increase of linear blood flow rate of middle cerebral arteries and index of peripheral vascular resistance were detected in group GOLD4 relative to control and GOLD1-3 groups (p < 0.05). DDLV of 1 type was revealed in 27.7% of patients of COPD and was higher at patients with COPD and AH - 62.5% (χ²=11.5, р =0.009). Pathological patterns were identified at the level of the basal and medial parts of the left ventricle in patients with COPD. Conclusion: preclinical signs of target organ involvement identified in COPD patients without cardiovascular disease. Changes in cerebral blood flow in the form of an increase in linear blood flow rate and peripheral vascular resistance index were detected in the GOLD4 group. DDLV of 1 type was detected in the GOLD1-2 groups and was found more frequently in the combination of COPD with AH. Pathological patterns were identified at the basal and medial left ventricular levels in a combination of COPD and AH. Changes in target organs indicate the need for an in-depth search to reclassify cardiovascular risk and identify an individual prevention plan.
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Bastos Fernandez, M., D. Lopez Otero, J. Lopez Pais, et al. "1024 Left ventricle myocardial deformation (strain) pattern in severe aortic valve stenosis. AMY-TAVI study." European Heart Journal - Cardiovascular Imaging 21, Supplement_1 (2020). http://dx.doi.org/10.1093/ehjci/jez319.619.
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Abstract OnBehalf AMY-TAVI study PURPOSE. To study left ventricular (LV) myocardial deformation in patients with severe symptomatic aortic stenosis (AS), through the analysis of the Regional and Global Longitudinal Strain (GLS), as well as the phenotypic pattern of peak systolic longitudinal strain represented in the bull´s eye. METHODS. A total of 42 patients with severe symptomatic AS were prospectively and consecutively included. Conventional morphological and functional parameters were analyzed, along with LV strain parameters and the strain pattern phenotype using two-dimensional speckle-tracking echocardiography. Indices derived from strain accepted as suggestive of cardiac amyloidosis were calculated (RELAPS: relative apical sparing: defined using the equation (average apical LS/(average basal LS + mid-LS); Eyection Fraction strain ratio (EFSR= LVEF/GLS). Scintigraphy with technetium pyrophosphate99 and blood protein electrophoresis were performed in all patients for the diagnosis / exclusion of cardiac amyloidosis. RESULTS The mean age was 80 ± 7 years, and 52% were women. The mean aortic valvular area was 0.6 ± 0.1 cm2 and the left ventricular ejection fraction (LVEF) was 56 ± 16%. 19 patients (45.2%) presented a pattern of relative apical sparing of LV longitudinal strain (RELAPS&gt; 1); and 16 patients (38%) showed an EFSR&gt; 4.1. Cardiac amyloidosis was excluded in all patients. In the univariate analysis, RELAPS&gt; 1 was significantly associated with higher degree of LV hypertrophy, lower LV end-diastolic volume, and greater myocardial contraction fraction. CONCLUSIONS. In our series, patients with severe symptomatic AS have with high frequency a "relative apical sparing" longitudinal strain pattern and Eyection Fraction Strain Ratio similar to those described in cardiac amyloidosis. Our results suggest that the classic patterns of cardiac amyloidosis are common in patients with severe AS in the absence of said pathology, findings that we believe may have important clinical implications. Abstract 1024 Figure. Peak systolic LS patterns in severe AS
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Simkowski, Julia M., Michael Jiang, NADIA El HANGOUCHE, et al. "Abstract 15928: Relative Sparing of Apical Longitudinal Strain for Detection of Cardiac Amyloidosis: Intervendor Variation." Circulation 142, Suppl_3 (2020). http://dx.doi.org/10.1161/circ.142.suppl_3.15928.
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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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Korthals, Dennis, Grigorios Chatzantonis, Michael Bietenbeck, Claudia Meier, Philipp Stalling, and Ali Yilmaz. "CMR-based T1-mapping offers superior diagnostic value compared to longitudinal strain-based assessment of relative apical sparing in cardiac amyloidosis." Scientific Reports 11, no. 1 (2021). http://dx.doi.org/10.1038/s41598-021-94650-2.
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AbstractCardiac amyloidosis (CA) is an infiltrative disease. In the present study, we compared the diagnostic accuracy of cardiovascular magnetic resonance (CMR)-based T1-mapping and subsequent extracellular volume fraction (ECV) measurement and longitudinal strain analysis in the same patients with (a) biopsy-proven cardiac amyloidosis (CA) and (b) hypertrophic cardiomyopathy (HCM). N = 30 patients with CA, N = 20 patients with HCM and N = 15 healthy control patients without relevant cardiac disease underwent dedicated CMR studies. The CMR protocol included standard sequences for cine-imaging, native and post-contrast T1-mapping and late-gadolinium-enhancement. ECV measurements were based on pre- and post-contrast T1-mapping images. Feature-tracking analysis was used to calculate 3D left ventricular longitudinal strain (LV-LS) in basal, mid and apical short-axis cine-images and to assess the presence of relative apical sparing. Receiver-operating-characteristic analysis revealed an area-under-the-curve regarding the differentiation of CA from HCM of 0.984 for native T1-mapping (p < 0.001), of 0.985 for ECV (p < 0.001) and only 0.740 for the “apical-to-(basal + midventricular)”-ratio of LV-LS (p = 0.012). A multivariable logistical regression analysis showed that ECV was the only statistically significant predictor of CA when compared to the parameter LV-LS or to the parameter “apical-to-(basal + midventricular)” LV-RLS-ratio. Native T1-mapping and ECV measurement are both superior to longitudinal strain measurement (with assessment of relative apical sparing) regarding the appropriate diagnosis of CA.
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Saito, M., Y. Nakao, R. Higaki, et al. "Clinical significance of the relative apical sparing pattern of longitudinal strain in patients with cardiac amyloidosis." European Heart Journal 41, Supplement_2 (2020). http://dx.doi.org/10.1093/ehjci/ehaa946.1012.
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Abstract Background The relative apical sparing pattern (RASP) of left ventricular (LV) longitudinal strain (LS) is frequently associated with cardiac amyloidosis (CA). However, some patients with CA do not show the RASP, and their clinical characteristics have not been fully clarified. We sought to investigate the clinical significance of RASP in patients with CA. Methods One hundred consecutive CA patients who were diagnosed by biopsy or myocardial pyrophosphate scintigraphy and evaluated for RASP (mean age: 76 years, male: 77%, LV mean wall thickness: 13.5 mm, light-chain [AL] type: 33 cases, transthyretin [TTR] type: 67 cases) were retrospectively enrolled. The RASP was semi-quantitatively and quantitatively assessed. Semi-quantitative RASP was defined as reduction of LS (≥−10%) in ≥5 (of 6) basal segments relative to preserved LS (&lt;−15%) in ≥1 apical segment. Quantitative RASP was calculated according to the following formula: Quantitative RASP = [Average apical LS] / [Average basal LS + Average mid LS]. We adapted three validated thresholds (&gt;1.00, &gt;0.90, and &gt;0.87) according to the literature. Results Semi-quantitative and binalized quantitative RASP (&gt;1.00, &gt;0.90, and &gt;0.87) were observed in 55, 55, 63, and 65 patients, respectively. RASP in each definition was more prevalent in the TTR group than in the AL group. Additionally, RASP was significantly associated with higher LV wall thickness even after adjustment for the CA subtypes (all, p&lt;0.05, Figure). After the RASP assessment, 35 all-cause deaths and 26 cardiac deaths were observed during the follow-up period (median, 1.1 years). Although these events were significantly associated with poor nutrition, lower blood pressure, higher New York Heart Association class, and the AL group, no association was found with RASP and LV wall thickness. Conclusions The incidence of RASP is low in the case of thin LV wall thickness in CA patients, which may indicate the difficulty of early diagnosis of CA using RASP in patients with mild LV hypertrophy. The prognostic prediction using RASP may be challenging in this cohort. Figure 1 Funding Acknowledgement Type of funding source: None
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Saito, M., M. Imai, D. Wake, R. Higaki, T. Sumimoto, and S. Inaba. "P305Prognostic value of relative apical sparing pattern of longitudinal strain in patients with severe aortic stenosis." European Heart Journal 40, Supplement_1 (2019). http://dx.doi.org/10.1093/eurheartj/ehz747.0140.
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Abstract Background The relative apical sparing pattern (RASP) of left ventricular longitudinal strain (LS) is determined on the strain polar map, while global longitudinal strain (GLS) is measured using speckle-tracking echocardiography and is frequently associated with cardiac amyloidosis (CA). According to recent reports, some elderly patients with aortic stenosis (AS) suffer from transthyretin CA and have a poor prognosis. Accordingly, we aimed to investigate the association of RASP and outcome of patients with severe AS. Methods We retrospectively studied 157 consecutive patients (age: 81±10 years, 33% men) with severe AS (mean transaortic pressure gradient: 49 mmHg) and preserved ejection fraction (>50%). After measuring GLS, RASP was semi-quantitatively and quantitatively assessed. Semi-quantitative RASP (sRASP) was defined as reduction of LS (more than −10%), showing light red or blue in ≥5 segments out of the basal six segments, relative to apical LS (less than −15%) showing red. This analysis was independently performed in a blinded manner by two observers. Quantitative RASP (qRASP) was calculated using the following formula: average apical LS/(average basal LS + average mid-ventricle LS), then qRASP ≥1 was determined as positive according to the previous paper. Patients were followed up to determine their outcomes, i.e., sudden cardiac death or unexpected admission due to heart failure over a median duration of 1.9 years. Concordance of sRASP was assessed using the kappa statistic, and a Cox proportional hazards model was used to assess the association between the parameters and primary outcome. Results The consistency in the observations of the two sonographers in identifying sRASP was found to be excellent (κ = 1.00). sRASP and qRASP were observed in 24 (15%) and 42 (27%) patients, respectively, and were significantly associated with the primary outcome (n=44; 28%). The representative case is shown in figure (left panel). Further, positive sRASP was associated with the outcome after adjusting for the Euro score, NYHA ≥II, and GLS (hazard ratio = 2.69, p=0.01). The model based on these covariates significantly improved following the addition of sRASP (Figure; right panel). In addition, sRASP was observed in four patients out of 50 patients who underwent aortic valve replacement. Of these, one patient had the primary outcome (25%). On the other hand, in the remaining 46 patients without sRASP, four patients had the outcome (9%). Figure 1 Conclusions RASP was observed in some patients with severe AS and has been shown to have increasing importance in predicting adverse cardiac events in such patients.
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Matsuda, K., H. Okayama, T. Kazatani, et al. "Clinical usefulness of relative apical sparing pattern for predicting functional recovery after transcatheter aortic valve implantation in patients with severe aortic stenosis." European Heart Journal 41, Supplement_2 (2020). http://dx.doi.org/10.1093/ehjci/ehaa946.0132.
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Abstract Background Relative apical sparing pattern (RASP) is thought to be associated with prognosis in patients with cardiac amyloidosis or left ventricular hypertrophy (LVH). Although almost all patients with severe aortic stenosis (AS) have LVH, little is known about the effect of transcatheter aortic valve implantation (TAVI) in patients with severe AS exhibiting a RASP. Purpose This study aimed to elucidate the effect of TAVI on left ventricular global longitudinal strain (LS; LVGLS) in patients with severe AS exhibiting a RASP. Methods Eighty-four patients who underwent transfemoral or subclavian TAVI were evaluated. They were divided into the RASP and non-RASP groups. The average apical LS divided by the sum of the average mid and basal LS values of &gt;1.0 was defined as the RASP. We analyzed the difference between pre- and post-TAVI LVGLS (ΔGLS = post-TAVI LVGLS − pre-TAVI LVGLS). Results Of the 84 patients (mean age, 84.5±3.9 years; 24 men), 15 (17.9%) exhibited a RASP. No significant difference in mean pre-TAVI LVGLS was found between the RASP and non-RASP groups (−16.6% ± 3.8% vs. −15.8% ± 3.9%). The ΔGLS in the RASP group was significantly higher than that in the non-RASP group (−0.97% ± 2.5% vs. −2.6% ± 3.0%; P&lt;0.05). Multivariate analysis revealed that relative apical longitudinal strain was an independent predictor of ΔGLS (β = 0.35, p=0.002). Conclusion Relative apical longitudinal strain was associated with LVGLS recovery. The effect of TAVI on LVGLS in patients with a RASP is inferior to that in patients without a RASP. Funding Acknowledgement Type of funding source: None
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Jiang, Michael, Julia M. Simkowski, Nadia El Hangouche, et al. "Abstract 17246: Sensitivity and Specificity of Relative Sparing of Apical Longitudinal Strain for Detection of Systemic Light-Chain Amyloidosis versus Transthyretin Amyloidosis." Circulation 142, Suppl_3 (2020). http://dx.doi.org/10.1161/circ.142.suppl_3.17246.
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Introduction: Relative apical sparing of longitudinal strain (RALS, the ratio of apical strain vs the rest of the heart) on echocardiography has been found to have high sensitivity and specificity for differentiating cardiac amyloidosis (CA) from other causes of left ventricular hypertrophy. Previous studies have shown no significant difference between amyloid subtypes, systemic light-chain amyloidosis (AL) and transthyretin amyloidosis (ATTR) Hypothesis: There will be a significant difference in sensitivity and specificity of RALS to detect CA across amyloid subtypes. Methods: A cohort of patients with either AL or ATTR amyloid was identified, with a control cohort of patients with left ventricular hypertrophy (LVH) of other etiologies. Speckle tracking echocardiography was performed on EchoPAC (GE Medical Systems) software to obtain values of basal, mid, and apical longitudinal strain for each patient; relative apical strain was then calculated. Results: The TTR group (n=22) was older (66.4±7.9, 76.6±11.6, p=0.001) and more likely to be female (p=0.009) than the AL group (n=30), both groups had similar rates of hypertension, diabetes mellitus, and end stage renal disease. Echocardiographic markers of diastolic function were decreased in both groups; the AL group had decreased left ventricle end diastolic volume (60.9±25.5, 94.9±50.2, p=0.012) and mean wall thickness (1.4±0.3, 1.6±0.4 p=0.017). ROC analysis using a RALS cutoff of 2 to differentiate AL and ATTR from the LVH control group revealed similar specificity (AL 85%, ATTR 85%) and sensitivity (AL 40%, ATTR 50%). Difference in area-under-curve (AUC) was not significant (p=0.2) (figure). Conclusions: ATTR and AL amyloid have similar specificity, but ATTR has a trend towards improved sensitivity over AL for detection of CA using RALS with the previously validated threshold of 2. This might become significant with a larger sample, work that is currently on-going..
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Fikrle, M., J. Marek, P. Kuchynka, and T. Palecek. "P5400The utility of simplified apical 4-chamber view evaluation of relative apical sparing of longitudinal strain in diagnosing AL amyloid cardiomyopathy." European Heart Journal 38, suppl_1 (2017). http://dx.doi.org/10.1093/eurheartj/ehx493.p5400.
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Abecasis, J., G. Mendes, A. Ferreira, et al. "Relative apical sparing in patients with severe aortic stenosis: prevalence and significance." European Heart Journal 41, Supplement_2 (2020). http://dx.doi.org/10.1093/ehjci/ehaa946.1987.
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Abstract Relative apical sparing (RAS) of LV longitudinal strain (LS) is a red flag for diagnostic suspicion of amyloid cardiomyopathy (AC). However, it may present in pts with aortic stenosis (AS), where the prevalence of transthyretin AC is being increasingly reported. Aim To describe the prevalence of RAS deformation pattern in patients with AS and its clinical significance. Methods We prospectively studied 53 pts (71±8y, 54.7% men) with severe symptomatic AS - mean gradient (AVM): 54.6 mmHg; aortic valve area 0.74cm2, referred for surgical replacement with no previous history of ischemic cardiomyopathy. Beyond ECG and transthoracic echo (TTE), all pts underwent CMR, with tissue characterization before surgery. RAS was defined as average apical LS / average basal LS + average mid LS &gt;1 at 2D LV LS analysis. Aortic valve replacement and septal myocardial biopsy were already performed in 26 pts. AS severity indexes, LV remodelling and tissue characterization were compared in both groups, with and without RAS. Results RAS was present in 16 pts (30.8%). There were neither pseudoinfarct pattern or low voltage at ECG, nor infiltration suspicion from CMR study (native T1 value 1047ms [IQR 1028–1084]; ECV 22% [IQR 18–25]). Furthermore, none of the pts had suspicion of amyloid deposition at histopathology. Median CMR LVEF was 64.5% [IQR 51.3–70.8%] and 36 pts (67.9%) had non-ischemic DE, with a median fraction of 6.0% [IQR 4.9–12.7%] of LV mass. Comparing both groups, RAS cohort showed a significantly higher AVM, relative wall thickness, maximum septal thickness, peak systolic dispersion and higher LV indexed mass, DE and lower LVEF at CMR. RAS group has also higher NT pro BNP (Table). Conclusions RAS is common in this group of pts despite the absence of clinical and histological signs of myocardial infiltration. RAS occurs with worse indexes of LV remodeling and fibrosis consistent with a more advanced stage of the disease. Funding Acknowledgement Type of funding source: None
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Saito, M., M. Imai, D. Wake, R. Higaki, T. Sumimoto, and S. Inaba. "3078Usefulness of a semi-quantitative and layer-specific assessment of the relative apical sparing pattern of longitudinal strain for the identification of cardiac amyloidosis." European Heart Journal 40, Supplement_1 (2019). http://dx.doi.org/10.1093/eurheartj/ehz745.0034.
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Abstract Background The relative apical sparing pattern (RASP) of left ventricular longitudinal strain (LS) is determined using a strain polar map, while global longitudinal strain is measured using speckle-tracking echocardiography, and it is frequently associated with cardiac amyloidosis (CA). However, the definition of visual RASP is ambiguous, and this leads to insufficient reproducibility, whereas quantitative RASP takes time and leads to difficulty in the clinical application. Generally, amyloid predominantly accumulates in the endo-myocardial layer. As such, layer-specific analysis of RASP may more accurately identify CA. Therefore, the aims of this study were to explore the reproducible and easy definition of RASP for identifying CA and investigate the effect of layer-specific analysis on the assessment. Methods A total of 40 patients with CA diagnosed by biopsy and technetium pyrophosphate scintigraphy were compared with 120 control patients matched for mean left ventricular wall thickness (40 aortic stenosis, 40 hypertrophic cardiomyopathy, and 40 hypertensive heart disease). We compared the discriminative abilities of three definitions of RASP (visual, quantitative, and semi-quantitative). According to a previous paper, visual RASP was defined as visual reduction of LS in the basal and middle LS segments (light red or blue) relative to the apical LS (red). Quantitative RASP was calculated using the following formula: average apical LS/(average basal LS + average mid-ventricle LS), then binarized by the optimal cut-off value for predicting CA. Semi-quantitative RASP was defined as reduction of LS (≥-10%) in five or more segments out of the basal six segments, relative to apical LS (≤-15%). Sample cases are shown in Figure (left). Visual and semi-quantitative RASP were independently assessed by two blinded sonographers. The RASP at the endo-myocardial and all layers was evaluated using customized software. The concordance was assessed using the kappa statistic, whereas the discriminative ability was assessed using receiver operating characteristic curve analysis. Results The concordance of visual RASP was modest but its semi-quantitative RASP was perfect (Table right). The discriminative ability of semi-quantitative RASP at each layer was significantly better than that of visual RASP and close to that of the binary quantitative RASP. Additionally, the discriminative abilities of visual (p=0.10) and semi-quantitative (p=0.11) RASP at the endo-myocardial layer appeared to be better than those at all layers. Conclusions The assessment method of semi-quantitative RASP is easy and highly reproducible. Furthermore, it accurately discriminates CA. In addition, assessment at the endo-myocardial layer potentially improves the discriminative ability.
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Canonico, M. E., L. Fiorillo, C. Santoro, et al. "Prominent involvement of basal left ventricular longitudinal strain in patients with monoclonal gammopathy of undetermined significance." European Heart Journal 41, Supplement_2 (2020). http://dx.doi.org/10.1093/ehjci/ehaa946.2139.
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Abstract Background In cardiac amyloidosis the application of Speckle Tracking Echocardiography allows to identify a specific left ventricular (LV) longitudinal strain (LS) pattern characterized by “apical sparing”, with a prominent involvement of basal and middle segments and normal LS of apical cap. The pattern of regional LS has been never investigated in monoclonal gammopathy of undetermined significance (MGUS), a condition which can predispose to cardiac amyloidosis. Purpose To compare LV regional LS patterns and LS base-to-apex behaviour of patients affected by MGUS in comparison with healthy subjects. Methods We enrolled 40 patients affected by MGUS (M/F=20/20; age 62.6±13.8 years), asymptomatic for cardiac symptoms, and a control group of 40 healthy subjects, matched for sex and age. Nineteen (47%) MGUS patients showed prevalent free K light chain and 21 (53%) had prevalent free λ light chain. Participants underwent standard echo-Doppler exam, including Speckle Tracking of the three apical views. Global longitudinal strain (GLS), the average LS of six basal (BLS), six middle (MLS), and six apical (ALS) segments (considered in absolute values) and relative regional strain ratio RRSR [=ALS/(BLS+MLS)] were computed. Exclusion criteria were overt heart failure, LV ejection fraction &lt;53%, coronary artery and congenital heart disease, moderate to severe valvular disease, primary cardiomyopathies, atrial fibrillation and inadequate echo imaging. Results The two groups were comparable for body mass index, blood pressure and heart rate. LV mass index, relative wall thickness, left atrial volume index and Doppler-derived LV diastolic parameters did not differ significantly between the two groups. LV ejection fraction was also similar in MGUS and healthy controls. GLS resulted significantly lower in MGUS group than in controls (20.5±3.0 vs. 22.4±2.0%, p&lt;0.02). BLS (17.1±3.7 vs. 19.2±2.2%, p=0.004), MLS (24.9±3.8 vs. 27.1±3.6%, p&lt;0.01) and ALS (25.1±3.8 vs. 27.1±3.5%, p&lt;0.01) were significantly lower in MGUS than in controls. The intergroup difference of RRSR (0.60±0.05 vs. 0.58±0.04) did not achieve the statistical significance (p=0.26) and none of the MGUS patients had RRSR&gt;1. The figure depicts a LS bull'eye of a MGUS patient showing the prominent involvement of LV basal segments. Conclusions In presence of a normal LV ejection fraction, MGUS patients show a subclinical LV longitudinal systolic dysfunction. This is testified by a reduction of GLS and of regional LS which involves mainly LV basal segments, without substantial changes of relative regional strain ratio. LV regional longitudinal dysfunction could be useful to monitor LV myocardial mechanics during follow-up of MGUS patients. LS bull's eye in a MGUS patient Funding Acknowledgement Type of funding source: None
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Bastos Fernandez, M., D. Lopez Otero, J. Lopez Pais, et al. "Left ventricle myocardial deformation pattern in severe aortic valve stenosis without cardiac amyloidosis. AMY-TAVI study." European Heart Journal 41, Supplement_2 (2020). http://dx.doi.org/10.1093/ehjci/ehaa946.0083.
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Abstract Background The Longitudinal Strain (LS) pattern in cardiac amyloidosis (CA) typically spares the apex of the heart, and this is a sensitive and specific finding that can be used to distinguish AC from other causes of left ventricular (LV) hypertrophy. Purpose To assess the clinical profitability of the LV deformation echocardiographic criteria derived from LS described as suggestive of CA, in patients with severe symptomatic aortic stenosis (AS) without amyloidosis referred for TAVI. Methods Within AMY-TAVI study (NCT03984877). Prior to TAVI implantation, conventional echocardiographic parameters were analyzed, along with LV deformation parameters and strain phenotype using Speckle-Tracking Echocardiography. Strain derived Indices accepted for CA screening were calculated: RELAPS: relative apical LS (average apical LS/sum of the average basal and mid LS); SAB: septal apical to base ratio (apical septal LS/basal septal LS); EFSR: ejection fraction strain ratio (LVEF/GLS). After implant, technetium pyrophosphate99 scintigraphy and proteinogram were performed to diagnose or exclude CA, and those patients in which CA was excluded were selected. Results 109 patients were consecutively included. The mean age was 81±6 yo, 58% were women. The mean aortic valve area (AVA) was 0.7±0.1 cm2 and the mean LVEF was 57.8±15%. Strain analysis could only be performed in 92 patients. Of these, 39 (42%) presented a LV strain pattern with relative apical sparing of LS respect to basal and middle segments (RELAPS&gt;1 pattern); 82 patients (89%) SAB was &gt;2.1; and 39 (42%) showed EFSR &gt;4.1. The RELAPS&gt;1 pattern was significantly associated with greater severity of AS based on AVA (0.7 cm2 in RELAPS &lt;1 vs 0,6 cm2 in RELAPS &gt;1, p=0.041), maximum velocity (4,4 vs 4,7 m/s, p=0.018), maximum aortic valve gradient (81 vs 91 mmHg, p=0.021) and medium gradient (49 vs 56 mmHg, p=0.020); higher degree of LV hypertrophic remodeling (Maximum wall thickness 14,3 vs 16,1 mm, p=0,003; Relative wall thickness 0,5 vs 0,6 mm, p=0,008); LV mass index: 168 vs 192 gr/m2, p=0,005; LV end-diastolic volume 112 vs 91 ml, p=0,005), and significantly lower myocardial contraction fraction (0,22 vs. 0,18, p=0,001). Conclusions In our series, patients with severe symtomatic AS without CA referred for TAVI frequently present a strain phenotype with relative apical preservation and a LVEF/GLS ratio similar to those described in CA. Our results suggest that the classic patterns of CA are common in patients with severe AS, in absence of said pathology, which limits its use for CA screening in these patients. Polar map patterns according to RELAPS Funding Acknowledgement Type of funding source: None
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Brand, A. M., G. Baldenhofer, D. Frumkin, A. Huebscher, K. Stangl, and F. Knebel. "1038 Comparative assessment of phasic left atrial and regional left ventricular strain in patients with cardiac amyloidosis and LV hypertrophy." European Heart Journal - Cardiovascular Imaging 21, Supplement_1 (2020). http://dx.doi.org/10.1093/ehjci/jez319.630.
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Abstract Background Echocardiographic hallmarks of cardiac amyloidosis (CA), such as increased wall thickness of the LV and sparkling appearance of the myocardium, are limited by a reduced diagnostic accuracy. Purpose We sought to evaluate the diagnostic value of phasic left atrial strain alterations and of regional global longitudinal systolic LV strain (LVGLS) reductions in patients with CA and with other forms of LV hypertrophy. Methods Standard apical 4-chamber views were stored for offline analysis (Vivid E9, GE, Vingmed, Horton) in 54 patients who underwent endomyocardial biopsy for unclear LV hypertrophy. We then analyzed LVGLS as well as LA reservoir, conduit, and contraction strain using 2D speckle tracking echocardiography (2DSTE; EchoPAC software, GE). To assess regional LVGLS, the average of apical strain values / (average of mid + basal LV strain values) was calculated (relative apical sparing; RELAPS). Receiver operating characteristic (ROC) curve analyses and a multivariate logistic regression analysis were performed to investigate the diagnostic value of the respective LA and LV deformation analysis. Results CA was bioptically confirmed in 34 patients (13 TTR, 1 AA, 20 AL amyloidosis). In 18 patients, myocardial biopsy revealed other forms of LV hypertrophy, such as hypertensive heart disease (n = 2), hypertrophic cardiomyopathy (n = 12), and inflammatory myocardial diseases (n = 4). Mean septal wall thickness (17.7 ± 2.9 mm and 17.9 ± 4.3 mm) and left atrial volume index (43.8 ± 12.2 and 44.1 ± 17.2) were not different between groups. RELAPS was significantly higher in patients with CA (1.37 ± 0.94 vs. 0.86 ± 0.29, p&lt;.007). Phasic atrial mechanics were significantly worse in CA (LA reservoir, conduit, and contraction strain 10.0 ± 5.2%, -6.5 ± 3.5%, and -5.0 ± 4.1%, respectively, in CA; and 22.7 ± 7.8%, -13.9 ± 5.2%, and -13.0 ± 5.5%, in LVH, respectively; p&lt;.001). With an area under the curve (AUC) of 0.91, and a sensitivity and specificity of 91.2 and 84.2% for a cut-off value of &lt;15.8%, LA reservoir strain showed a higher diagnostic accuracy in discriminating CA from LVH than the parameter RELPAS (AUC 0.74, sensitivity and specificity 60% and 71% for a cut-off of &gt;1.0; p&lt;.05). LA conduit and contraction strain performed significantly better than RELAPS as well (AUC 0.87 for conduit, and AUC 0.86 for contraction function; p&lt;.001 each). Of all echocardiographic parameters, LA reservoir strain remained significantly associated with CA in a multivariate regression model. Conclusions LA strain during all three phases of the atrial cycle was significantly reduced in patients with CA compared to other forms of LVH, and showed a markedly higher diagnostic accuracy than regional LV strain analysis, with LA reservoir strain showing highest discriminative value. The assessment of LA strain, as part of a comprehensive echocardiographic assessment, may be useful to rule-in the possible diagnosis of CA in patients with unclear LV hypertrophy.
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Somarakis, K., and H. Ahmed. "P1707 Strain imaging - resolving the diagnostic conundrum of cardiac amyloidosis." European Heart Journal - Cardiovascular Imaging 21, Supplement_1 (2020). http://dx.doi.org/10.1093/ehjci/jez319.1070.
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Abstract Introduction Cardiac amyloidosis is a common cardiac condition and is still significantly underdiagnosed. Autopsy studies revealed that myocardial tranthyretin amyloid deposition is found in up to 30% of patients with HFpEF (heart failure with preserved ejection fraction). A study in 2015 reported that 13% of patients hospitalised due to HFpEF (older than 60 years old and with left ventricular hypertrophy - LVH) had moderate to severe uptake on the Technetium DPD scintigraphy. Strain imaging through Echocardiography can be a useful diagnostic tool and can provide valuable clues towards the aetiology of LVH. Relative "apical sparing" pattern of longitudinal strain has been reported to have good sensitivity and specificity in differentiating patients with cardiac amyloidosis from controls. Case A 66 year old man presented with progressive breathlessness and peripheral oedema. His past medical history included peripheral neuropathy, bilateral decompression procedures for carpal tunnel syndrome and IgM Monoclonal Gammopathy of Undetermined Significance (MGUS). His B-type natriuretic peptide levels were elevated and his ECG showed normal sinus rhythm, RsR" pattern on the anterior leads and no evidence of LVH. The transthoracic echocardiogram showed moderate concentric LVH, normal left ventricular systolic function and mildly impaired left ventricular diastolic function. No evidence of pericardial effusion. Urine protein:creatinine ratio findings were consistent with nephrotic range proteinuria. He had a cardiac MRI that confirmed normal biventricular wall motion and systolic function and a moderate increase in the wall thickness of both ventricles, but showed no evidence of late gadolinium enhancement. At this point, we repeated his transthoracic echocardiogram with the use of strain imaging and it revealed a pattern of apical sparing suggestive of cardiac amyloidosis. After collaboration with the Haematology team, a bone marrow biopsy was performed that showed that the MGUS had progressed to IgM multiple myeloma. There was however no evidence of amyloid deposits. He was subsequently referred to the national amyloidosis centre (as per family request), where a SAP (serum amyloid P) scan showed renal and splenic amyloid deposits confirming Light-chain Amyloidosis. To exclude the possibility of TTR Amyloidosis, he also had a 99mTc-DPD scintigraphy which did not detect any amyloid deposits. He was treated under the care of the Haematology team with Velcade/Cyclophosphamide/Dexamethasone chemotherapy. Conclusion In patients with a clinical suspicion of cardiac amyloidosis, Echocardiography with strain imaging can be very useful and should be performed routinely. Furthermore, in patients with high clinical suspicion of cardiac amyloidosis, diagnostic investigations should persist even if the initial workup does not yield specific findings. Abstract P1707 Figure. Strain imaging findings
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Nakao, Y., M. Saito, R. Higaki, et al. "Utility of scoring system including relative apical sparing pattern for screening cardiac amyloidosis in patients with left ventricular hypertrophy." European Heart Journal 41, Supplement_2 (2020). http://dx.doi.org/10.1093/ehjci/ehaa946.0997.
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Abstract Background Cardiac amyloidosis (CA) is an infiltrative disease mimicking left ventricular hypertrophy (LVH), although its prognosis is poorer than other diseases with LVH. Moreover, because CA is treatable, appropriate screening for CA is an important area of study for clinicians to prevent and treat the disease. Several imaging predictors of CA have been reported so far;. in particular, deformation parameters such as relative apical sparing patterns of longitudinal strain (RASP) may diagnose CA with better precision than conventional parameters. Accordingly, we hypothesized that the inclusion of deformation parameters into the established diagnostic parameters would permit derivation of a risk score for CA screening in patients with LVH. Thus, we aimed to 1) investigate the incremental benefits of deformation parameters over established diagnostic parameters for CA screening in patients with LVH; 2) determine the risk score to screen CA patients with LVH using all of these variables; and 3) externally validate the score. Methods We retrospectively studied 295 consecutive non-ischemic patients with LVH who underwent echocardiography as well as the detailed work-up for LVH (biopsy, technetium pyrophosphate scintigraphy (99mTc-PYP) or cardiac magnetic resonance imaging) (median age, 67 years; MWT, 12 mm). CA was diagnosed by biopsy or 99mTc-PYP. The base model consisted of age (≥65 [male], ≥70 [female]), low voltage in electrocardiography, and posterior wall thickness ≥14 mm in reference to previous studies. Continuous echocardiographic variables were binarized by the use of generally accepted external cutoff points to avoid best clinical scenario. Incremental benefits were assessed using receiver operating characteristic curve analysis and area under the curve (AUC) comparison. Multiple logistic regression analysis was performed to determine the risk score. The score was then validated in the external validation sample (N=178, median age, 70 years; MWT, 12 mm). Results CA was observed in 54 patients (18%) and of the several echocardiographic parameters studied, only RASP demonstrated a significant incremental benefit for the screening of CA over the base model (Figure A). After multiple logistic regression analysis in the prediction of CA with 4 variables (RASP and basal model components), each was assigned a numeric value based on its relative effect (Figure B). The incidence rate of CA clearly increased as the sum of the risk score increased (Figure C). The score had good discrimination ability, with an AUC of 0.87, a total score of ≥2 with 70% sensitivity and 90% specificity. Similarly, the discrimination ability of the score in the validation cohort was sufficient (AUC = 0.87). Conclusion Overall, we determined a simple risk score including RASP to screen CA. This score takes into account 4 common parameters used in daily practice, and therefore, has potential utility in risk stratification and management of patients with LVH. Figure 1 Funding Acknowledgement Type of funding source: Private hospital(s). Main funding source(s): Kitaishikai
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Brand, Anna, David Frumkin, Anne Hübscher, et al. "Phasic left atrial strain analysis to discriminate cardiac amyloidosis in patients with unclear thick heart pathology." European Heart Journal - Cardiovascular Imaging, April 3, 2020. http://dx.doi.org/10.1093/ehjci/jeaa043.
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Abstract Aims Traditional echocardiographic parameters for the assessment of suspected cardiac amyloidosis (CA) are of limited diagnostic accuracy. We sought to explore differences and the discriminative value of phasic left atrial strain (LAS) reductions and of regional longitudinal left ventricular (LV) strain alterations (relative apical sparing; RELAPS) in CA and other causes of LV wall thickening (LVH). Methods and results We included 54 patients with unclear LVH (mean septal diastolic wall thickness 17.8 ± 3.5 mm); CA was bioptically confirmed in 35 patients (8 mATTR, 6 wtATTR, 20 AL, and 1 AA amyloidosis) and LVH in 19 subjects. We analysed RELAPS as well as LA reservoir (LASr), conduit (LAScd), and contraction strain (LASct) using 2D speckle tracking echocardiography (EchoPAC software, GE). RELAPS was higher (1.37 ± 0.94 vs. 0.86 ± 0.29, P &lt; 0.007), whereas atrial mechanics were significantly reduced in CA (LASr, LAScd, and LASct: 9.7 ± 5.2%, −6.5 ± 3.5%, and −5.0 ± 4.1% in CA; and 22.7 ± 7.8%, −13.9 ± 5.2%, and −13.0 ± 5.5% in LVH, respectively; P &lt; 0.001 each). With an area under the curve (AUC) of 0.91 [95% confidence interval (CI) 0.82–0.99], LASr showed a higher diagnostic accuracy in discriminating CA than RELAPS (AUC 0.74, 95% CI 0.59–0.88). LASr and LAScd remained significantly associated with CA in a multivariate regression model. Conclusion Phasic LAS was significantly reduced in patients with CA and showed a higher diagnostic accuracy in discriminating CA than RELAPS. The additional assessment of phasic LAS may be useful to rule in the possible diagnosis of CA in patients with unclear LVH.
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Ferreira, V., S. Aguiar Rosa, I. Rodrigues, et al. "Prognostic impact of suspected cardiac amyloidosis in aortic stenosis patients referred for transcatheter aortic valve implantation." European Heart Journal 41, Supplement_2 (2020). http://dx.doi.org/10.1093/ehjci/ehaa946.2113.
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Abstract Background The prevalence of cardiac amyloidosis (CA) and aortic stenosis (AS) both increase with age. Transcatheter aortic valve implantation (TAVI) expands the number of patients (P) eligible for treatment of AS, emphasizing the need to understand the prevalence of CA in AS and its prognostic associations. Echocardiography with speckle tracking has emerged as a useful method to enhance the clinical suspicion and to provide prognostic information. Purpose To estimate the prevalence of CA in P with severe AS referred for TAVI and to evaluate the impact of concomitant CA in prognosis. Methods 94 consecutive AS P who underwent TAVI with maximum left ventricular wall thickness (LVWT)&gt;12 mm were retrospectively identified. Clinical data, pre TAVI echocardiographic parameters and follow up (FU) data regarding all-cause mortality and MACE (including all-cause mortality, admission for heart failure, pacemaker implantation and stroke) were analysed. We registered apical sparing pattern in bull's eye plots (ASPB), calculated relative apical longitudinal strain formula (RALS) [average apical LS/(average basal LS + mid-LS)] and ejection fraction/global longitudinal strain (EF/GLS) ratio. Results Mean age was 82.2±5.8 years (Y), with 43 men (45.7%). 27.7% were in NYHA functional class II, 64.9% in functional class III and 7.4% in functional class IV. Median EF was 57±15% and 26.6% presented EF&lt;50%. Suspected CA evaluated by ASPB was found in 39 P (41.5%) and RALS &gt;1 was identified in 22 P (23.4%). An EF/GLS ratio &gt;4.1 was obtained in 53 P (56.4%). Over a median follow-up of 13.4±25.8 months, 28 deaths (29.8%) and 31 MACEs (33.0%) occurred. The presence of ASPB was associated with increased all-cause mortality (33.3% vs. 5.6%, p=0.002) and MACE (48.7% vs 22.2%, p=0.01). RALS&gt;1 correlated also with all-cause mortality (31.8% vs. 12.5%, p=0.04) and with new bundle branch block and indication for pacemaker implantation (46.2% vs 37.0%, p=0.05). P with GLS&gt;−14.8% and ASPB had significantly worse prognosis regarding all-cause mortality (p=0.003) and MACE (p=0.007). Kaplan–Meier survival analysis showed that survival was significantly worse for P with ASPB (log-rank 0.002). With multivariate Cox regression analysis, ASPB was independently associated with all-cause mortality (HR=4.49, p=0.039). Conclusions Suspected CA appears prevalent among patients with AS and associates with all-cause mortality. The importance of screening for CA in older AS patients and optimal treatment strategies in those with CA warrant further investigation, especially in the era of transcatheter aortic valve implantation. Funding Acknowledgement Type of funding source: None
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Ferreira, V., S. Aguiar Rosa, I. Rodrigues, et al. "1226 Prognostic impact of concomitant cardiac amyloidosis in aortic stenosis patients referred for transcatheter aortic valve implantation." European Heart Journal - Cardiovascular Imaging 21, Supplement_1 (2020). http://dx.doi.org/10.1093/ehjci/jez319.688.
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Abstract Background The prevalence of cardiac amyloidosis (CA) and aortic stenosis (AS) both increase with age. Transcatheter aortic valve implantation (TAVI) expands the number of patients (P) eligible for treatment of AS, emphasizing the need to understand the prevalence of CA in AS and its prognostic associations. Echocardiography with speckle tracking has emerged as a useful method to enhance the clinical suspicion and to provide prognostic information. Purpose To estimate the prevalence of CA in P with severe AS referred for TAVI and to evaluate the impact of concomitant CA in prognosis. Methods 94 consecutive AS P who underwent TAVI with maximum left ventricular wall thickness (LVWT)&gt;12 mm were retrospectively identified. Clinical data, pre TAVI echocardiographic parameters and follow up (FU) data regarding all-cause mortality and MACE (including all-cause mortality, admission for heart failure, pacemaker implantation and stroke) were analysed. We registered apical sparing pattern in bull’s eye plots (ASPB), calculated relative apical longitudinal strain formula (RALS) [average apical LS/(average basal LS + mid-LS)] and ejection fraction/global longitudinal strain (EF/GLS) ratio. Results Mean age was 82.2 ± 5.8 years (Y), with 43 men (45.7%). 27.7% were in NYHA functional class II, 64.9% in functional class III and 7.4% in functional class IV. Median EF was 57 ± 15% and 26.6% presented EF &lt; 50%. Suspected CA evaluated by ASPB was found in 39 P (41.5%) and RALS &gt; 1 was identified in 22 P (23.4%). An EF/GLS ratio &gt; 4.1 was obtained in 53 P (56.4%). Over a median follow-up of 13.4 ± 25.8 months, 28 deaths (29.8%) and 31 MACEs (33.0%) occurred. The presence of ASPB was associated with increased all-cause mortality (33.3% vs. 5.6%, p = 0.002), new bundle branch block and indication for pacemaker implantation (46.2% vs 37.0%, p = 0.05) and MACE (48.7% vs 22.2%, p = 0.01). All-cause mortality was also higher in P with RALS (31.8% vs. 12.5%, p = 0.04). P with GLS&gt;-14.8% and ASPB had significantly worse prognosis regarding all-cause mortality (p = 0.003) and MACE (p = 0.007). Kaplan–Meier survival analysis showed that survival was significantly worse for P with ASPB (log-rank 0.002). With multivariate Cox regression analysis, ASPB was independently associated with all-cause mortality (HR = 4.49, p = 0.039). Conclusions Suspected CA appears prevalent among patients with AS and associates with all-cause mortality. The importance of screening for CA in older AS patients and optimal treatment strategies in those with CA warrant further investigation, especially in the era of transcatheter aortic valve implantation. Abstract 1226 Figure. Kaplan–Meier curves and ASPB
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Saito, M., Y. Nakao, R. Higaki, et al. "Incremental benefits of echocardiographic indices over clinical parameters for screening cardiac amyloidosis in patients with left ventricular hypertrophy." European Heart Journal 41, Supplement_2 (2020). http://dx.doi.org/10.1093/ehjci/ehaa946.0084.
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Abstract Background Cardiac amyloidosis (CA), characterized by amyloid protein deposition in the heart, is a treatable disease. Although left ventricular (LV) wall thickness is the most established imaging predictor for CA, several echocardiographic indices including deformation parameters also contribute to the screening of CA. However, it is unclear whether additive values of echocardiographic indices have greater benefit over the conventional clinical predictors for the screening of CA. Therefore, we sought to compare the incremental benefits of echocardiographic indices over the clinical parameters for the screening of CA and externally validate their incremental benefits. Methods We retrospectively studied 295 consecutive patients (median age, 67 years; male, 65%; mean LV wall thickness (MWT), 12 mm) with LV hypertrophy who underwent echocardiography as well as the detailed work-up for myocardium (Biopsy, technetium pyrophosphate scintigraphy (99mTc-PYP) or cardiac magnetic resonance imaging). CA was diagnosed through biopsy or 99mTc-PYP. The clinical model considers patients' age and the low-voltage in electrocardiography in reference to previous studies. Continuous echocardiographic variables were represented in binary through generally accepted external cutoff points. The incremental benefits of the echocardiography findings over the clinical model were assessed using with the help of both receiver-operated characteristic curve analysis and comparison of area under the curves. Furthermore, these incremental benefits were validated in the external validation sample (median age, 70 years; male, 69%; MWT, 12 mm). Results Among the enrolled patients, CA was observed in 18% of cases. Table presents the results of this study. Of the echocardiographic parameters, relative apical sparing pattern (RASP) was the greatest contributor for improvement of diagnostic accuracy of the clinical model. The next greatest contributor was LV wall thickness, followed by left atrial reservoir strain (LAS), E/e', left atrial volume index, ejection fraction strain ratio, and pericardial effusion, respectively. Similarly, RASP, LV wall thickness, global longitudinal strain, ejection fraction, LAS, and granular sparkling showed significant incremental benefit in the validation cohort. Only mean wall thickness, LV wall thickness, LAS, E/e' and RASP consistently improved the diagnostic accuracy of the clinical model. Conclusion During the screening process, adding LV wall thickness, LAS, and RASP to the clinical parameters may be useful for the accurate diagnosis of CA in patients with LV hypertrophy. Figure 1 Funding Acknowledgement Type of funding source: None
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Binder, C. B., F. Duca, S. Aschauer, et al. "P895Apical sparing in patients without cardiac amyloidosis." European Heart Journal 40, Supplement_1 (2019). http://dx.doi.org/10.1093/eurheartj/ehz747.0491.
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Abstract Background Apical sparing describes a reduced longitudinal strain in the basal segments and preserved or supranormal longitudinal strain in the apical segments of the left ventricular (LV) myocardium. This pattern has been described as a typical finding in patients with cardiac amyloidosis (CA) and restrictive cardiomyopathy. However, apical sparing is not a quantitative parameter and is fairly subjective to the echocardiographer's judgement. It is not known, if a certain degree of apical sparing is also present in patients with only mild LV hypertrophy and diastolic dysfunction such as it is present in heart failure with preserved ejection fraction (HFpEF). Methods Patients with cardiac transthyretin and light chain amyloidosis and patients with HFpEF were included in a clinical registry at our outpatient clinic. CA was diagnosed according to current guidelines. All patients underwent a comprehensive transthoracic echocardiography (TTE) exam at the time of study inclusion. The TTE protocol included standard and speckle-tracking imaging to assess the presence of apical sparing as well as the basal to apical strain gradient. Patients with known coronary artery disease were excluded. Results In total 115 patients were included in this study. Of these, 87 (75.7%) were diagnosed with CA and 28 (24.3%) with HFpEF. Not surprisingly, apical sparing was found in a majority (86.2%) of patients with CA, however mild forms of this phenomenon were also present in 67.9% of patients with HFpEF (p=0.029, Figure 1). Median basal longitudinal strain was significantly more impaired in patients with CA (p<0.001) but there was no difference between longitudinal strain in the apical segments when comparing CA to HFpEF (p=0.443). This resulted in a higher median apical to basal strain gradient in patients with CA (2.3 (IQR 1.7–3.83) versus 1.13 (IQR 1.5–1.8), p<0.001). Figure 1 Conclusion Mild forms of apical sparing can be found in patients without CA. Gradual reduction in strain from base to apex could be an unspecific pathophysiologic mechanism which is remarkably pronounced in patients with CA.
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Zhang, K. W., R. Zhang, Y. Soyama, M. Karmpalioti, D. J. Lenihan, and J. Gorcsan. "P2724Diagnosis of transthyretin versus light chain cardiac amyloidosis by apical sparing strain ratio in patients with clinically suspected disease." European Heart Journal 40, Supplement_1 (2019). http://dx.doi.org/10.1093/eurheartj/ehz748.1041.
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Abstract Background Apical sparing by longitudinal strain imaging has reported utility for the diagnosis of cardiac amyloidosis. However, potential differences in the apical sparing pattern in light chain (AL) versus transthyretin (ATTR) amyloidosis in patients with high clinical suspicion for cardiac amyloidosis is not clear. Purpose Our objective was to test the hypothesis that echocardiographic strain imaging could determine differences in patients with clinically suspected AL and ATTR cardiac amyloidosis. Methods We studied 206 patients, aged 64±11, with clinically suspected cardiac amyloidosis. Routine longitudinal strain imaging analyses was performed (EchoPAC, GE Healthcare) with bulls-eye plots. After 27 exclusions (8 arrhythmia/frame rate, 19 missing/poor images), there were 179 patients. Included were 129 patients with cardiac amyloid: 42 by endomyocardial biopsy, 4 by technetium pyrophosphate scan, 65 by non-cardiac biopsy with suggestive cardiac imaging (interventricular septal thickness ≥1.2cm by echocardiography or characteristic cardiac MRI findings), 15 with multiple myeloma and suggestive cardiac imaging, and 3 by autopsy; 50 patients had a negative endomyocardial biopsy or autopsy for cardiac amyloid. The apical sparing ratio by strain imaging was calculated as the (average of apical segments) / (average of mid segments + average of basal segments). Results Cardiac amyloidosis patients were 79% with AL and 21% with ATTR. Applying the previously published apical sparing ratio cut-off of 1.0 for longitudinal strain imaging, sensitivity and specificity were 29% and 78%, respectively, for diagnosis of cardiac amyloidosis. Applying a ratio cut-off of 0.81 improved sensitivity to 72% with specificity of 64% and area under the curve (AUC) of 0.66. Positive and negative predictive values were 85% and 46%, respectively, at this ratio cut-off. The apical sparing ratio was significantly higher in AL and ATTR as compared to the biopsy negative group (p<0.001). Furthermore, the apical sparing ratio was significantly higher in ATTR as compared to AL (p<0.05). Apical sparing pattern and ratio Conclusions Among patients with high clinical suspicion for cardiac amyloidosis, the apical sparing ratio by echocardiographic strain imaging can demonstrate differences for AL and ATTR cardiac amyloidosis and has potential for clinical utility.
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Iakovlev, S., and A. I. Kalinskaya. "P1684 Cardiac amyloidosis hits the valve first." European Heart Journal - Cardiovascular Imaging 21, Supplement_1 (2020). http://dx.doi.org/10.1093/ehjci/jez319.1047.
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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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Bui, Q., G. Ma, M. Kraushaar, et al. "Apical sparing strain pattern observed in danon disease: insights from a global registry." European Heart Journal 41, Supplement_2 (2020). http://dx.doi.org/10.1093/ehjci/ehaa946.1019.
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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
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Huntjens, Peter, Kathleen Zhang, Yuko Soyama, Maria Karmpalioti, Daniel Lenihan, and John Gorcsan. "Abstract 15734: Left Atrial Reservoir Strain as a Novel Prognostic Marker in Biopsy-proven Light-chain Amyloidosis." Circulation 142, Suppl_3 (2020). http://dx.doi.org/10.1161/circ.142.suppl_3.15734.
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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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Lagies, Ruth, Bodo B. Beck, Bernd Hoppe, Narayanswami Sreeram, and Floris E. A. Udink ten Cate. "Apical Sparing of Longitudinal Strain, Left Ventricular Rotational Abnormalities, and Short-Axis Dysfunctionin Primary Hyperoxaluria Type 1." Circulation: Heart Failure 6, no. 4 (2013). http://dx.doi.org/10.1161/circheartfailure.113.000432.
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