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Cardiac amyloidosis

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https://www.readbyqxmd.com/read/27900617/new-and-evolving-concepts-regarding-the-prognosis-and-treatment-of-cardiac-amyloidosis
#1
REVIEW
Stefano Perlini, Roberta Mussinelli, Francesco Salinaro
Systemic amyloidoses are rare and proteiform diseases, caused by extracellular accumulation of insoluble misfolded fibrillar proteins. Prognosis is dictated by cardiac involvement, which is especially frequent in light chain (AL) and in transthyretin variants (ATTR, both mutated, (ATTRm), and wild-type, (ATTRwt)). Recently, ATTRwt has emerged as a potentially relevant cause of a heart failure with preserved ejection fraction (HFpEF). Cardiac amyloidosis is an archetypal example of restrictive cardiomyopathy, with signs and symptoms of global heart failure and diastolic dysfunction...
November 29, 2016: Current Heart Failure Reports
https://www.readbyqxmd.com/read/27885144/treatment-of-multiple-myeloma-in-a-heart-transplant-recipient-focus-on-the-immunomodulatory-effects-of-lenalidomide
#2
Lola Xie, Bartosz Jozwik, Phillip Weeks, L Maximilian Buja, Robert Brown, Sriram Nathan, Keshava Rajagopal, Christina Paruthi, Biswajit Kar, Pranav Loyalka, Indranee Rajapreyar
Malignancy following solid organ transplant remains a significant threat to the survival of cardiac transplant recipients. Plasma cell dyscrasias including multiple myeloma have been encountered in this population, and medication treatments traditionally used to treat these disorders demonstrate immunomodulatory effects that may have implications on the transplanted allograft. Lenalidomide is an immunomodulatory agent that has been used to treat plasma cell disorders, including light-chain amyloidosis (AL) and multiple myeloma, and represents such a class of medications in which the risks and benefits in the solid organ transplant population remain to be fully elucidated...
November 24, 2016: Progress in Transplantation
https://www.readbyqxmd.com/read/27882404/reduced-trans-mitral-a-wave-velocity-predicts-the-presence-of-wild-type-transthyretin-amyloidosis-in-elderly-patients-with-left-ventricular-hypertrophy
#3
Satoru Yamamura, Yasuhiro Izumiya, Toshifumi Ishida, Yoshiro Onoue, Yuichi Kimura, Shinsuke Hanatani, Satoshi Araki, Koichiro Fujisue, Daisuke Sueta, Hisanori Kanazawa, Seiji Takashio, Hiroki Usuku, Koichi Sugamura, Kenji Sakamoto, Eiichiro Yamamoto, Megumi Yamamuro, Hisayo Yasuda, Sunao Kojima, Koichi Kaikita, Seiji Hokimoto, Hisao Ogawa, Kenichi Tsujita
Wild-type transthyretin amyloidosis (ATTRwt) is often overlooked in elderly patients with left ventricular hypertrophy (LVH). Impaired atrial function, in addition to ventricular diastolic dysfunction, is one of the hallmarks of cardiac amyloidosis. Here, we assessed the hypothesis that atrial function evaluated by A-velocity in pulse Doppler echocardiography is useful to differentiate ATTRwt in elderly patients with LVH. We analyzed 133 consecutive patients who underwent tissue biopsy to rule out infiltrative cardiomyopathy in our institute...
November 23, 2016: Heart and Vessels
https://www.readbyqxmd.com/read/27878699/differentiation-of-light-chain-cardiac-amyloidosis-from-hypertrophic-cardiomyopathy-using-myocardial-mechanical-parameters-by-velocity-vector-imaging-echocardiography
#4
Lu Zhang, Xiao Zhou, Jing Wang, Yang Mu, Bohan Liu, Wenqing Lv, Ye Wang, Hongwei Liu, Hongbin Liu, Guang Zhi
We aimed to evaluate the diagnostic efficacy of layered velocity vector imaging (VVI)-derived left ventricular (LV) mechanical parameters in the differential diagnosis of primary light-chain cardiac amyloidosis (AL-CA) and hypertrophic cardiomyopathy (HCM). We recruited 35 subjects with histologically-diagnosed AL-CA, 35 subjects with HCM, and 30 age-matched healthy controls. We used conventional echocardiography and electrocardiogram to evaluate general heart function and electrophysiology properties. Furthermore, we applied two-dimensional VVI echocardiography to assess the layered mechanical parameters during systole, including endocardial and epicardial longitudinal strain (ENDO and EPI LSsys), circumferential strain (CSsys), radial strain (RSsys), rotation (ROT) and twist (TWI), in different LV walls and levels...
November 23, 2016: International Journal of Cardiovascular Imaging
https://www.readbyqxmd.com/read/27866030/diagnostic-accuracy-of-ct-for-the-detection-of-left-ventricular-myocardial-fibrosis-in-various-myocardial-diseases
#5
Hiroyuki Takaoka, Nobusada Funabashi, Masae Uehara, Yasunori Iida, Yoshio Kobayashi
PURPOSE: To evaluate the diagnostic accuracy of computed tomography (CT) for the detection of myocardial fibrosis, we compared the frequency of abnormal late enhancement (LE) in left ventricular myocardium (LVM) on CT with that on gadolinium-enhanced cardiac magnetic resonance (CMR) in patients with various myocardial diseases. METHODS: Fifty-six patients with suspected various myocardial diseases (19 with hypertrophic cardiomyopathy, 3 with cardiac amyloidosis, 3 with post myocarditis, 2 with dilated cardiomyopathy, 2 with cardiac sarcoidosis, 2 with cardiac tumor, 2 with previous myocardial infarction, 2 with hypertensive heart disease) underwent 1...
November 9, 2016: International Journal of Cardiology
https://www.readbyqxmd.com/read/27856462/clarifying-immunoglobulin-gene-usage-in-systemic-and-localized-immunoglobulin-light-chain-amyloidosis-by-mass-spectrometry
#6
Taxiarchis V Kourelis, Surendra Dasari, Jason D Theis, Marina Ramirez-Alvarado, Paul J Kurtin, Morie A Gertz, Steven R Zeldenrust, Roman M Zenka, Ahmet Dogan, Angela Dispenzieri
The goal of this study was to investigate the frequency of use of light chain variable region (IGVL) genes among patients with systemic (ALS) and localized (ALL) amyloidosis and to assess for associations between IGVL gene usage and organ tropism. We evaluated clinic charts from 821 AL patients seen at the Mayo Clinic who had bone marrow, fat pad and solid organ tissue samples typed by liquid chromatography tandem mass spectrometry (LC-MS). We identified 701 patients with ALs and 120 with ALL. Overall, we were able to identify an IGVL gene in 87 (72%) patients with ALL and 573 (82%) patients with ALS...
November 16, 2016: Blood
https://www.readbyqxmd.com/read/27846815/endomyocardial-biopsy-via-the-femoral-access-still-safe-and-valuable-diagnostic-tool
#7
Sylwia Sławek, Aleksander Araszkiewicz, Agnieszka Gaczkowska, Justyna Koszarska, Damian Celiński, Marek Grygier, Maciej Lesiak, Stefan Grajek
BACKGROUND: The endomyocardial biopsy has proven to be an integral diagnostic tool for surveillance of cardiac allograft rejection and identification of myocardial diseases. Nevertheless, this invasive procedure is not risk-free. This study focuses on the risk of complications and diagnostic performance of right ventricular endomyocardial biopsy (EMB). METHODS: In this single-center retrospective study, we analyzed 315 EMB procedures performed between July 2008 and May 2015 in 73 patients...
November 15, 2016: BMC Cardiovascular Disorders
https://www.readbyqxmd.com/read/27826773/a-tale-of-two-diagnoses-the-role-of-noninvasive-cardiovascular-imaging-to-differentiate-cardiac-amyloidosis
#8
David Kassop, Jason J Nam, Brennan R Cebula, Palak Shah, Ron Blankstein, Rodney H Falk, Edward A Hulten
No abstract text is available yet for this article.
November 8, 2016: Journal of Nuclear Cardiology: Official Publication of the American Society of Nuclear Cardiology
https://www.readbyqxmd.com/read/27825470/monoclonal-igm-related-al-amyloidosis
#9
REVIEW
Paolo Milani, Giampaolo Merlini
Monoclonal immunoglobulin M (IgM)-related light chain (AL) amyloidosis, which accounts for 5%-7% of all AL amyloidosis cases, is a distinct clinical entity that poses specific challenges to clinicians. Several studies reported that although there is a substantial overlap, the pattern of organ involvement is peculiar, with higher frequencies of lung, lymph nodes, and peripheral nervous system involvement. A recent collaborative study from three European referral centers, defined that cardiac involvement, advanced Mayo disease stage, neuropathic, and liver involvement were independent factors that had impact on survival in IgM-AL amyloidosis patients...
June 2016: Best Practice & Research. Clinical Haematology
https://www.readbyqxmd.com/read/27809600/plasma-hepatocyte-growth-factor-is-a-novel-marker-of-al-cardiac-amyloidosis
#10
Kristopher J Swiger, Eitan A Friedman, Evan L Brittain, Kelsey A Tomasek, Shi Huang, Yan R Su, Douglas B Sawyer, Daniel J Lenihan
BACKGROUND: Cardiac amyloidosis is an infiltrative cardiomyopathy that is challenging to diagnose. We hypothesized that the novel biomarkers hepatocyte growth factor (HGF), galectin-3 (GAL-3), interleukin-6 (IL-6), and vascular endothelial growth factor (VEGF) would be elevated in cardiac amyloidosis and may be able to discriminate from non-cardiac systemic amyloidosis or other cardiomyopathies with similar clinical or morphologic characteristics. METHODS: Patients were selected from the Vanderbilt Main Heart Registry according to the following groups: (1) amyloid light-chain (AL) cardiac amyloidosis (n = 26); (2) transthyretin (ATTR) cardiac amyloidosis (n = 7); (3) left ventricular hypertrophy (LVH) (n = 45); (4) systolic heart failure (n = 42); and (5) non-cardiac systemic amyloidosis (n = 7)...
December 2016: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/27806283/biochemical-and-electrophysiological-modification-of-amyloid-transthyretin-on-cardiomyocytes
#11
Laura Sartiani, Monica Bucciantini, Valentina Spinelli, Manuela Leri, Antonino Natalello, Daniele Nosi, Silvia Maria Doglia, Annalisa Relini, Amanda Penco, Sofia Giorgetti, Elisabetta Gerace, Guido Mannaioni, Vittorio Bellotti, Stefania Rigacci, Elisabetta Cerbai, Massimo Stefani
Transthyretin (TTR) amyloidoses are familial or sporadic degenerative conditions that often feature heavy cardiac involvement. Presently, no effective pharmacological therapy for TTR amyloidoses is available, mostly due to a substantial lack of knowledge about both the molecular mechanisms of TTR aggregation in tissue and the ensuing functional and viability modifications that occur in aggregate-exposed cells. TTR amyloidoses are of particular interest regarding the relation between functional and viability impairment in aggregate-exposed excitable cells such as peripheral neurons and cardiomyocytes...
November 1, 2016: Biophysical Journal
https://www.readbyqxmd.com/read/27806176/abrupt-onset-of-refractory-heart-failure-associated-with-light-chain-amyloidosis-in-hypertrophic-cardiomyopathy
#12
Benedetta Tomberli, Francesco Cappelli, Federico Perfetto, Iacopo Olivotto
Importance: The natural history of hypertrophic cardiomyopathy (HCM) is complex and may include progressive heart failure and severe left ventricular dysfunction. When disease progression is abrupt, however, other coexisting diseases should be ruled out. This may be difficult in the case of amyloidosis, which classically mimics HCM. Results: We present an example of severe clinical deterioration in a patient with HCM due to superimposed amyloid light-chain amyloidosis...
November 2, 2016: JAMA Cardiology
https://www.readbyqxmd.com/read/27804150/progressive-refractory-light-chain-amyloidosis-and-multiple-myeloma-patients-are-responsive-to-the-addition-of-clarithromycin-to-imid-based-therapy
#13
Adir Shaulov, Chezi Ganzel, Noam Benyamini, Yossef Barshay, Neta Goldschmidt, David Lavie, Diana Libster, Alex Gural, Batia Avni, Moshe E Gatt
Multiple myeloma (MM) and primary systemic light chain amyloidosis (AL) are both chronic plasma cell dyscrasias with different clinical expression but limited treatment options for relapsed refractory disease. We report the effect of the addition of clarithromycin on 31 MM and 17 AL with relapsed or refractory disease who had an insufficient response or disease progression while on an IMiD based therapy. In this high risk population, hematological response was reported in 48% of MM patients and 94% of AL patients...
November 2, 2016: American Journal of Hematology
https://www.readbyqxmd.com/read/27804073/early-phase-myocardial-uptake-intensity-of-99m-tc-hmdp-vs-99m-tc-dpd-in-patients-with-hereditary-transthyretin-related-cardiac-amyloidosis
#14
Mukedaisi Abulizi, Anne-Ségolène Cottereau, Aziz Guellich, Stéphanie Vandeventer, Arnault Galat, Axel Van Der Gucht, Violaine Plante-Bordeneuve, Jean-Luc Dubois-Randé, Diane Bodez, Jean Rosso, Thibaud Damy, Emmanuel Itti
BACKGROUND: This study sought to compare the intensity of early-phase myocardial uptake of two phosphonate-based radiotracers, (99m)Tc-hydroxymethylene diphosphonate (HMDP) and (99m)Tc-3,3-diphosphono-1,2-propanodicarboxylic acid (DPD), in patients with hereditary transthyretin-related cardiac amyloidosis (TTR-CA). METHODS: Six patients with biopsy-proven diagnosis of TTR-CA and characteristic amyloid fibril composition underwent early-phase (99m)Tc-HMDP myocardial scintigraphy as part of their routine workup; they were later assessed by (99m)Tc-DPD scintigraphy after having signed informed written consent...
November 1, 2016: Journal of Nuclear Cardiology: Official Publication of the American Society of Nuclear Cardiology
https://www.readbyqxmd.com/read/27801739/18f-florbetaben-a-new-tool-for-amyloidosis-staging
#15
Emmanuel D'Estanque, Benjamin Chambert, Olivier Moranne, Pierre Olivier Kotzki, Vincent Boudousq
We report the case of a 73-year-old man with a documented (renal biopsy) light-chain amyloidosis (AL) imaged with F-AV-1 (F-florbetaben) compared with a volunteer. A cardiac amyloidosis was suspected. As it was an AL and not a transthyretin amyloidosis, F-FDG and F-florbetaben PET/CT were preferred to bone scan. F-FDG scintigraphy showed a focal cardiac hypermetabolism. In addition of the heart, F-florbetaben scintigraphy showed an intense spleen and thyroid pathologic uptake and a moderate salivary gland and kidney uptake...
October 31, 2016: Clinical Nuclear Medicine
https://www.readbyqxmd.com/read/27798053/relationship-between-aetiology-and-left-ventricular-systolic-dysfunction-in-hypertrophic-cardiomyopathy
#16
Stefania Rosmini, Elena Biagini, Costantinos O'Mahony, Heerajnarain Bulluck, Niccolo' Ruozi, Luis R Lopes, Oliver Guttmann, Patricia Reant, Cristina C Quarta, Antonis Pantazis, Maria Tome-Esteban, William J Mckenna, Claudio Rapezzi, Perry M Elliott
BACKGROUND: Severe left ventricular (LV) systolic dysfunction is an uncommon complication of hypertrophic cardiomyopathy (HCM) that is associated with poor prognosis. Small observational series suggest that patients with rare causes of HCM are more likely to develop systolic impairment than those with idiopathic disease or mutations in cardiac sarcomeric protein genes. The aim of this study was to test this hypothesis by comparing the prevalence of systolic dysfunction and its impact on prognosis in patients with different causes of HCM...
October 24, 2016: Heart: Official Journal of the British Cardiac Society
https://www.readbyqxmd.com/read/27771830/solid-state-nmr-chemical-shift-assignments-for-al-09-vl-immunoglobulin-light-chain-fibrils
#17
Dennis W Piehl, Luis M Blancas-Mejía, Marina Ramirez-Alvarado, Chad M Rienstra
Light chain (AL) amyloidosis is a systemic disease characterized by the formation of immunoglobulin light-chain fibrils in critical organs of the body. The light-chain protein AL-09 presents one severe case of cardiac AL amyloidosis, which contains seven mutations in the variable domain (VL) relative to its germline counterpart, κI O18/O8 VL. Three of these mutations are non-conservative-Y87H, N34I, and K42Q-and previous work has shown that they are responsible for significantly reducing the protein's thermodynamic stability, allowing fibril formation to occur with fast kinetics and across a wide-range of pH conditions...
October 22, 2016: Biomolecular NMR Assignments
https://www.readbyqxmd.com/read/27769906/transthyretin-cardiac-amyloidosis-in-older-americans
#18
REVIEW
Danielle L Brunjes, Adam Castano, Autumn Clemons, Jonah Rubin, Mathew S Maurer
Wild-type transthyretin cardiac amyloidosis (ATTRwt), formerly called senile cardiac amyloidosis (SCA), is almost exclusively a disorder of older adults. As the population ages, the diagnosis of ATTRwt will increase, making it the most common form of cardiac amyloidosis. An important precondition to reduce underdiagnosis and misdiagnosis is to maintain a high index of suspicion for cardiac amyloidosis. Several clues can be gleaned from the clinical history, physical exam, electrocardiography, and noninvasive imaging techniques...
December 2016: Journal of Cardiac Failure
https://www.readbyqxmd.com/read/27743139/myocardial-strain-pattern-in-patients-with-cardiac-amyloidosis-secondary-to-multiple-myeloma-a-cardiac-mri-feature-tracking-study
#19
Sabha Bhatti, Srikanth Vallurupalli, Stephanie Ambach, Adam Magier, Evan Watts, Vien Truong, Abdul Hakeem, Wojciech Mazur
Cardiac MRI is frequently used in the diagnosis of cardiac amyloidosis. Feature tracking is a novel method of analyzing myocardial strain at the myocardial borders. We investigated myocardial deformation mechanics of both the right and left ventricles in patients with multiple myeloma with suspected cardiac amyloidosis. Comprehensive strain analysis was performed in 43 patients with multiple myeloma and suspected cardiac amyloidosis. MRI strain by feature tracking was measured using 2D cardiac performance analysis MR software (Tomtec, Germany)...
October 14, 2016: International Journal of Cardiovascular Imaging
https://www.readbyqxmd.com/read/27741127/anti-tnf%C3%AE-therapy-for-chronic-inflammatory-disease-in-kidney-transplant-recipients-clinical-outcomes
#20
Cyril Garrouste, Dany Anglicheau, Nassim Kamar, Claire Bachelier, Joseph Rivalan, Bruno Pereira, Sophie Caillard, Julien Aniort, Philippe Gatault, Martin Soubrier, Johnny Sayegh, Charlotte Colosio, Anthony Buisson, Eric Thervet, Nicolas Bouvier, Anne Elisabeth Heng
Anti-tumor necrosis factor-α (TNFα) therapy has improved the prognosis of many chronic inflammatory diseases. It appears to be well-tolerated by liver-transplant patients. However, their use and their safety in kidney-transplant patients have yet to be determined.In this retrospective study, we identified 16 adult kidney-transplant patients aged 46.5 years (34-51.8) who received anti-TNFα therapy from 7 kidney transplantation centers. The indications for this treatment included: chronic inflammatory bowel disease (n = 8), inflammatory arthritis (n = 5), AA amyloidosis (n = 1), psoriasis (n = 1), and microscopic polyangiitis (n = 1)...
October 2016: Medicine (Baltimore)
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