scholarly journals Atrial function and geometry differences in transthyretin versus immunoglobulin light chain amyloidosis: a cardiac magnetic resonance study

2022 ◽  
Vol 12 (1) ◽  
Author(s):  
Cassady Palmer ◽  
Vien T. Truong ◽  
Jeremy A. Slivnick ◽  
Sarah Wolking ◽  
Paige Coleman ◽  
...  
Keyword(s):  
Cardiac Magnetic Resonance ◽  
Magnetic Resonance ◽  
Light Chain ◽  
Left Atrial ◽  
Immunoglobulin Light Chain ◽  
Sphericity Index ◽  
Significant Difference ◽  
Immunoglobulin Light Chain Amyloidosis ◽  
Minimum Volumes ◽  
Insight Into

AbstractTo determine the differences in left atrial (LA) function and geometry assessed by cardiac magnetic resonance (CMR) between transthyretin (ATTR) and immunoglobulin light chain (AL) cardiac amyloidosis (CA). We performed a retrospective analysis of 54 consecutive patients (68.5% male, mean age 67 ± 11 years) with confirmed CA (24 ATTR, 30 AL) who underwent comprehensive CMR examinations. LA structural and functional assessment including LA volume, LA sphericity index, and LA strain parameters were compared between both subtypes. In addition, 15 age-matched controls were compared to all groups. Patients with ATTR-CA were older (73 ± 9 vs. 62 ± 10 years, p < 0.001) and more likely to be male (83.3% vs. 56.7%, p = 0.036) when compared to AL-CA. No significant difference existed in LA maximum volume and LA sphericity index between ATTR-CA and AL-CA. LA minimum volumes were larger in ATTR-CA when compared with AL-CA. There was a significant difference in LA function with worse strain values in ATTR vs AL: left atrial reservoir [7.4 (6.3–12.8) in ATTR vs. 13.8 (6.90–24.8) in AL, p = 0.017] and booster strains [3.6 (2.6–5.5) in ATTR vs. 5.2 (3.6–12.1) in AL, p = 0.039]. After adjusting for age, LA reservoir remained significantly lower in ATTR-CA compared to AL-CA (p = 0.03), but not LA booster (p = 0.16). We demonstrate novel differences in LA function between ATTR-CA and AL-CA despite similar LA geometry. Our findings of more impaired LA function in ATTR may offer insight into higher AF burden in these patients.

P2728Prognostic impact of left atrial function in patients with cardiac amyloidosis: a cardiac magnetic resonance study

2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
S Morini ◽  
G D Aquaro ◽  
G Taborchi ◽  
R Martone ◽  
M Gabriele ◽  
...  
Keyword(s):  
Cardiac Magnetic Resonance ◽  
Magnetic Resonance ◽  
Light Chain ◽  
Cardiac Death ◽  
Left Atrial ◽  
Cardiac Amyloidosis ◽  
Atrial Function ◽  
Left Atrial Function ◽  
Transthyretin Amyloidosis ◽  
Kaplan Meier

Abstract Background Left atrial function (AEF) is a parameter of paramount importance that has a prognostic value in a number of heart conditions. Cardiac involvement in both light-chain and transthyretin amyloidosis is the main driver of prognosis and influences treatment strategies. Cardiac magnetic resonance (CMR) provides high quality images of the left and right atria using high temporal resolution steady state free precession (SSFP) cine sequences. Purpose The aim of our study was to assess by CMR left atrial function and his correlation with prognosis in patients with cardiac amyloidosis. Method We enrolled 80 consecutive patients with diagnosis of cardiac amyloidosis: 38 patients (47%) with light-chain and 42 patients (53%) with transthyretin one. CMR was performed using a 1.5-T scanner. In all subjects, the study of atria was obtained by acquiring cine steady-state free precession (SSFP). Left Atrial function was evaluated by the ratio between the maximum and the minimum LA volume. A median follow up of 937 days was performed and 36 patients (44%) died of cardiac causes. We evaluated cardiac death as endpoint. We split out all the patient in 4 different quartiles depending on left atrial function: in the first quartile patients with AEF≤14% that reflects severe atrial dysfunction, in the second quartile patients with AEF between 14 and 19%, in the third quartile patients with AEF between 19 and 36% and in the last one patients with AEF≥36% that represent patients with normal value of left atrial function. Results We found that CMR assessed left atrial function allowed to individuate and stratify the prognosis in patients with cardiac amyloidosis. The most effective parameter to evaluate cardiac death was left atrial function with a cut off ≤14% that could predict cardiac related mortality with the same accuracy in both light-chain and transthyretin amyloidosis patients. Kaplan Meier analysis showed that patients with AEF≤14% had a worse prognosis as compared to patient with AEF≥14% (log rank p. 0001). Furthermore patients with AEF≤14% have a cardiac death risk of 32% at 1 year and 61% at 3 years. Kaplan Meier analysis Conclusion Cardiac magnetic resonance is an imaging modality that allows to individuate with great accuracy left atrial function in patients with various heart conditions and especially cardiac amyloidosis. CMR left atrial function assessment clearly identifies a subgroup of cardiac amyloid patients with an increased risk of death.

Normal left atrial strain and strain rate using cardiac magnetic resonance feature tracking in healthy volunteers

2019 ◽  
Author(s):  
Vien T Truong ◽  
Cassady Palmer ◽  
Sarah Wolking ◽  
Brandy Sheets ◽  
Michael Young ◽  
...  
Keyword(s):  
Cardiac Magnetic Resonance ◽  
Magnetic Resonance ◽  
Strain Rate ◽  
Left Atrial ◽  
Feature Tracking ◽  
Healthy Population ◽  
Interobserver Reproducibility ◽  
Normal Ranges ◽  
Strain And Strain Rate ◽  
Sphericity Index

Abstract Aims The aim of our study was to establish normal ranges for left atrial (LA) strain and strain rate using cardiac magnetic resonance feature tracking (CMR-FT), LA sphericity index, and to compare LA strain using CMR-FT with 2D-speckle tracking echocardiography (STE) in a healthy population. Methods and results A total of 112 volunteers (45 male, 67 female) had adequate tracking for analysis on CMR-FT (Circle Cardiovascular Imaging, Calgary, Canada). The median age was 42 years (range 19–79 years, interquartile range 30–53 years). LA reservoir, conduit, booster strain, strain rate using CMR-FT, and sphericity index were evaluated. Of the 112 volunteers, 91 patients had adequate tracking on 2D-STE using three commonly applied zero-baseline time reference methods: R-R gating, P-P gating, and volume gating (defining end-systole at the LA maximum and end-diastole at the LA minimum). The LA strain, strain rate using CMR-FT, and sphericity index were reported and comparable between both genders (P > 0.05 for all). The LA booster function including strain and strain rate increased significantly with age (P < 0.001 for all), while the LA conduit function gradually decreased. In comparison with STE, the LA reservoir strain was comparable between CMR and volume-gating methods (38.48 ± 9.31 vs. 36.77 ± 6.46; P = 0.13) but not with R-R and P-P gating methods (P < 0.001 for all). LA strain, strain rate, and sphericity index using CMR-FT had good intraobserver and interobserver reproducibility. Conclusion LA strain, strain rate using CMR-FT, and sphericity index can be quickly assessed with good intraobserver and interobserver reproducibility.

scholarly journals A Unique Case of Combined Nodular and Tracheobronchial Amyloidosis

2021 ◽  
Vol 2021 (2) ◽  
Author(s):  
Feihong Ding ◽  
Yun Li ◽  
Shailesh Balasubramanian ◽  
Subha Ghosh ◽  
Jason N Valent ◽  
...  
Keyword(s):  
Light Chain ◽  
Systemic Disease ◽  
Amyloid Deposition ◽  
Misfolded Proteins ◽  
Immunoglobulin Light Chain ◽  
Anatomic Site ◽  
Unique Case ◽  
Pulmonary Amyloidosis ◽  
Immunoglobulin Light Chain Amyloidosis ◽  
Tracheobronchial Amyloidosis

ABSTRACT Amyloidosis is a heterogeneous group of diseases characterized by the extracellular deposition of misfolded proteins that can affect either systemically or locally confined to one system. Pulmonary amyloidosis is rare and can be classified into three forms according to the anatomic site of involvement: nodular pulmonary amyloidosis, tracheobronchial amyloidosis and diffuse alveolar-septal amyloidosis. The former two usually represent localized amyloid disease and the latter represents systemic disease. Typically lung parenchymal and tracheobronchial amyloidosis do not present together in localized forms of pulmonary amyloidosis. Here we report a unique case of localized pulmonary immunoglobulin light-chain amyloidosis, manifested as both parenchymal nodules and tracheobronchial amyloid deposition.

scholarly journals Genetic pathogenesis of immunoglobulin light chain amyloidosis: basic characteristics and clinical applications

2021 ◽  
Vol 10 (1) ◽  
Author(s):  
Linchun Xu ◽  
Yongzhong Su
Keyword(s):  
Plasma Cell ◽  
Light Chain ◽  
Amyloid Fibril ◽  
Treatment Modalities ◽  
Driver Gene ◽  
Immunoglobulin Light Chain ◽  
Disease Evolution ◽  
Genetic Aberrations ◽  
Light Chain Amyloidosis ◽  
Immunoglobulin Light Chain Amyloidosis

AbstractImmunoglobulin light chain amyloidosis (AL) is an indolent plasma cell disorder characterized by free immunoglobulin light chain (FLC) misfolding and amyloid fibril deposition. The cytogenetic pattern of AL shows profound similarity with that of other plasma cell disorders but harbors distinct features. AL can be classified into two primary subtypes: non-hyperdiploidy and hyperdiploidy. Non-hyperdiploidy usually involves immunoglobulin heavy chain translocations, and t(11;14) is the hallmark of this disease. T(11;14) is associated with low plasma cell count but high FLC level and displays distinct response outcomes to different treatment modalities. Hyperdiploidy is associated with plasmacytosis and subclone formation, and it generally confers a neutral or inferior prognostic outcome. Other chromosome abnormalities and driver gene mutations are considered as secondary cytogenetic aberrations that occur during disease evolution. These genetic aberrations contribute to the proliferation of plasma cells, which secrete excess FLC for amyloid deposition. Other genetic factors, such as specific usage of immunoglobulin light chain germline genes and light chain somatic mutations, also play an essential role in amyloid fibril deposition in AL. This paper will propose a framework of AL classification based on genetic aberrations and discuss the amyloid formation of AL from a genetic aspect.

scholarly journals ELECTROCARDIOGRAPHIC PREDICTORS OF LEFT ATRIAL STRUCTURE AND FUNCTION DEFINED BY CARDIAC MAGNETIC RESONANCE FEATURE TRACKING: THE CTRIP STUDY

2014 ◽  
Vol 63 (12) ◽  
pp. A1249
Author(s):  
Theingi Tiffany Win ◽  
Bharath A. Venkatesh ◽  
Gustavo J. Volpe ◽  
Nathan Mewton ◽  
Patricia Rizzi ◽  
...  
Keyword(s):  
Cardiac Magnetic Resonance ◽  
Magnetic Resonance ◽  
Left Atrial ◽  
Feature Tracking ◽  
Structure And Function ◽  
And Function

scholarly journals Beyond the plasma cell: emerging therapies for immunoglobulin light chain amyloidosis

Blood ◽  
2016 ◽  
Vol 127 (19) ◽  
pp. 2275-2280 ◽  
Author(s):  
Brendan M. Weiss ◽  
Sandy W. Wong ◽  
Raymond L. Comenzo
Keyword(s):  
Light Chain ◽  
Plasma Cells ◽  
Organ Damage ◽  
Immunoglobulin Light Chain ◽  
Organ Function ◽  
Fatal Disease ◽  
Mortality And Morbidity ◽  
Amyloid Deposits ◽  
Emerging Therapies ◽  
Immunoglobulin Light Chain Amyloidosis

Abstract Systemic immunoglobulin light chain (LC) amyloidosis (AL) is a potentially fatal disease caused by immunoglobulin LC produced by clonal plasma cells. These LC form both toxic oligomers and amyloid deposits disrupting vital organ function. Despite reduction of LC by chemotherapy, the restoration of organ function is highly variable and often incomplete. Organ damage remains the major source of mortality and morbidity in AL. This review focuses on the challenges posed by emerging therapies that may limit the toxicity of LC and improve organ function by accelerating the resorption of amyloid deposits.

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