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Cardiac amyloidosis

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https://www.readbyqxmd.com/read/28340285/the-six-minute-walk-test-in-patients-with-al-amyloidosis-a-single-centre-case-series
#1
Vina Pulido, Gheorghe Doros, John L Berk, Vaishali Sanchorawala
The six-minute walk test (6MWT) has been widely used as an objective evaluation of functional exercise capacity and response to medical intervention in cardiopulmonary diseases. However, little is known about the 6MWT in evaluating patients with AL amyloidosis. We performed a retrospective study of 120 adults with systemic AL amyloidosis (60 with cardiac involvement and 60 without cardiac involvement) who had their initial evaluation at the Amyloidosis Center between 2013 and 2015 and had undergone 6MWT as a measure of functional exercise capacity...
March 24, 2017: British Journal of Haematology
https://www.readbyqxmd.com/read/28334621/sequential-cyclophosphamide-bortezomib-dexamethasone-unmasks-the-harmful-cardiac-effect-of-dexamethasone-in-primary-light-chain-cardiac-amyloidosis
#2
Fabien Le Bras, Valerie Molinier-Frenkel, Aziz Guellich, Jehan Dupuis, Karim Belhadj, Soulef Guendouz, Karima Ayad, Magali Colombat, Nicole Benhaiem, Claire Marie Tissot, Anne Hulin, Arnaud Jaccard, Thibaud Damy
Chemotherapy combining cyclophosphamide, bortezomib and dexamethasone is widely used in light-chain amyloidosis. The benefit is limited in patients with cardiac amyloidosis mainly because of adverse cardiac events. Retrospective analysis of our cohort showed that 39 patients died with 42% during the first month. A new escalation-sequential regimen was set to improve the outcomes. Nine newly-diagnosed patients were prospectively treated with close monitoring of serum N-terminal pro-brain natriuretic peptide, troponin-T and free light chains...
March 20, 2017: European Journal of Cancer
https://www.readbyqxmd.com/read/28330445/cardiac-amyloidosis-mimicking-severe-aortic-valve-stenosis-a-case-report-demonstrating-diagnostic-pitfalls-and-role-of-dobutamine-stress-echocardiography
#3
Tim Salinger, Kai Hu, Dan Liu, Sebastian Herrmann, Kristina Lorenz, Georg Ertl, Peter Nordbeck
BACKGROUND: Aortic valve stenosis is a common finding diagnosed with high sensitivity in transthoracic echocardiography, but the examiner often finds himself confronted with uncertain results in patients with moderate pressure gradients and concomitant systolic heart failure. While patients with true-severe low-gradient aortic valve stenosis with either reduced or preserved left ventricular systolic function are primarily candidates for valve replacement, there is a relevant proportion of patients with pseudo-severe aortic valve stenosis anticipated not to benefit but actually rather deteriorate by interventional therapy or surgery...
March 22, 2017: BMC Cardiovascular Disorders
https://www.readbyqxmd.com/read/28330375/complex-p-t88n-w130r-mutation-in-the-lysozyme-gene-leading-to-hereditary-lysozyme-amyloidosis-with-biopsy-proven-cardiac-involvement
#4
Brett W Sperry, Angela Dispenzieri, Asad Ikram, Martha Grogan, Jason D Theis, Nelson Leung, W Edward Highsmith, Joseph J Maleszewski, Mazen Hanna
No abstract text is available yet for this article.
March 22, 2017: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/28329248/clinical-characteristics-of-wild-type-transthyretin-cardiac-amyloidosis-disproving-myths
#5
Esther González-López, Christian Gagliardi, Fernando Dominguez, Cristina Candida Quarta, F Javier de Haro-Del Moral, Agnese Milandri, Clara Salas, Mario Cinelli, Marta Cobo-Marcos, Massimiliano Lorenzini, Enrique Lara-Pezzi, Serena Foffi, Luis Alonso-Pulpon, Claudio Rapezzi, Pablo Garcia-Pavia
Aims: Wild-type transthyretin amyloidosis (ATTRwt) is mostly considered a disease predominantly of elderly male, characterized by concentric LV hypertrophy, preserved LVEF, and low QRS voltages. We sought to describe the characteristics of a large cohort of ATTRwt patients to better define the disease. Methods and results: Clinical findings of consecutive ATTRwt patients diagnosed at 2 centres were reviewed. ATTRwt was diagnosed histologically or non-invasively (LV hypertrophy ≥12 mm, intense cardiac uptake at 99mTc-DPD scintigraphy and AL exclusion)...
March 1, 2017: European Heart Journal
https://www.readbyqxmd.com/read/28326465/pet-ct-evaluation-of-amyloid-systemic-involvement-with-18-f-florbetaben-in-patient-with-proved-cardiac-amyloidosis-a-case-report
#6
Dario Genovesi, Giuseppe Vergaro, Michele Emdin, Assuero Giorgetti, Paolo Marzullo
BACKGROUND: One of the most frequent disorders which lead to cardiac amyloidosis is transthyretin-related amyloidosis (ATTR). Some PET radio-pharmaceuticals for the detection of beta-amyloid deposits within the brain have shown to be able to detect also cardiac amyloid deposits. We present a case of a man with ATTR studied with [(18)F]-florbetaben PET-CT. RESULTS: Total-body scan showed a moderate uptake in the bone marrow, especially in correspondence of the vertebral column, while no significant myocardial uptake was present...
March 21, 2017: Journal of Nuclear Cardiology: Official Publication of the American Society of Nuclear Cardiology
https://www.readbyqxmd.com/read/28315077/setting-the-stage-for-the-next-step-in-cardiac-amyloidosis-imaging-serial-quantitative-studies-to-assess-disease-activity
#7
EDITORIAL
Prem Soman, Ahmad Masri
No abstract text is available yet for this article.
March 17, 2017: Journal of Nuclear Cardiology: Official Publication of the American Society of Nuclear Cardiology
https://www.readbyqxmd.com/read/28298289/cardiac-amyloidosis-a-zebra-hiding-in-plain-sight
#8
EDITORIAL
Frederick L Ruberg
No abstract text is available yet for this article.
March 2017: Circulation. Cardiovascular Imaging
https://www.readbyqxmd.com/read/28298286/echo-parameters-for-differential-diagnosis-in-cardiac-amyloidosis-a-head-to-head-comparison-of-deformation-and-nondeformation-parameters
#9
Efstathios D Pagourelias, Oana Mirea, Jürgen Duchenne, Johan Van Cleemput, Michel Delforge, Jan Bogaert, Tatyana Kuznetsova, Jens-Uwe Voigt
BACKGROUND: A plethora of echo parameters has been suggested for distinguishing cardiac amyloidosis (CA) from other causes of myocardial thickening with, however, scarce data on their head-to-head comparison. This study aimed at comparing the diagnostic accuracy of various deformation and conventional echo parameters in differentiating CA from other hypertrophic substrates, especially in the gray zone of mild hypertrophy (maximum wall thickness ≤16 mm) or normal ejection fraction (EF)...
March 2017: Circulation. Cardiovascular Imaging
https://www.readbyqxmd.com/read/28290973/total-artificial-heart-implantation-as-a-bridge-to-heart-transplantation-in-an-active-duty-service-member-with-amyloid-cardiomyopathy
#10
Michael S Scully, Dylan E Wessman, James M McKee, Gregory M Francisco, Keshav R Nayak, Jon A Kobashigawa
INTRODUCTION: Cardiac involvement by light-chain (AL) amyloid occurs in up to 50% of patients with primary AL amyloidosis. The prognosis of amyloid heart disease is poor with 1-year survival rates of 35 to 40%. Historically, heart transplantation was considered controversial for patients with AL amyloid cardiomyopathy (CM) given the systemic nature of the disease and poor survival. We present a case report of an active duty service member diagnosed with advanced cardiac amyloid who underwent total artificial heart transplant as a bridge to heart transplant and eventual autologous stem cell transplant...
March 2017: Military Medicine
https://www.readbyqxmd.com/read/28281017/heart-transplantation-in-cardiac-amyloidosis
#11
REVIEW
Matthew Sousa, Gregory Monohan, Navin Rajagopalan, Alla Grigorian, Maya Guglin
"Cardiac amyloidosis" is the term commonly used to reflect the deposition of abnormal protein amyloid in the heart. This process can result from several different forms, most commonly from light-chain (AL) amyloidosis and transthyretin (ATTR) amyloidosis, which in turn can represent wild-type (ATTRwt) or genetic form. Regardless of the origin, cardiac involvement is usually associated with poor prognosis, especially in AL amyloidosis. Although several treatment options, including chemotherapy, exist for different forms of the disease, cardiac transplantation is increasingly considered...
March 9, 2017: Heart Failure Reviews
https://www.readbyqxmd.com/read/28279425/current-concepts-of-cardiac-amyloidosis-diagnosis-clinical-management-and-the-need-for-collaboration
#12
REVIEW
Alexandra J Ritts, Robert F Cornell, Kris Swiger, Jai Singh, Stacey Goodman, Daniel J Lenihan
Cardiac amyloidosis is a complex and vexing clinical condition that requires a high degree of suspicion for the diagnosis with a substantial amount of discipline to discern the extent of disease and the best available therapy. There is a complex interplay between multiple organ systems, and the clinical presentation may involve a myriad of confusing clinical symptoms. The diagnosis of cardiac amyloidosis can be confirmed with a combination of physical findings, cardiac biomarkers, noninvasive testing, and, if necessary, myocardial biopsy...
April 2017: Heart Failure Clinics
https://www.readbyqxmd.com/read/28279033/-analysis-of-clinical-characteristics-and-outcome-of-patients-with-very-high-risk-primary-immunoglobulin-light-chain-amyloidosis
#13
J Feng, X F Huang, C L Zhang, K N Shen, C L Zhang, J Sun, Z Tian, X X Cao, L Zhang, D B Zhou, J Li
Objective: To evaluate the clinical characteristics and outcomes of very high risk patients with primary immunoglobulin light-chain amyloidosis (pAL) at a single center in China. Method: Clinical data, treatment and outcome of 205 pAL patients in Peking Union Medical College Hospital from January 2009 to February 2016 were retrospectively analyzed. A 'very high risk' group includes patients with Mayo 2004 stage Ⅲb and Mayo 2012 stage 4. Results: Of 205 patients, 34 (16.6%) were defined as very high risk pAL patients...
February 14, 2017: Zhonghua Xue Ye Xue za Zhi, Zhonghua Xueyexue Zazhi
https://www.readbyqxmd.com/read/28268091/automatic-quantification-of-the-myocardial-extracellular-volume-by-cardiac-computed-tomography-synthetic-ecv-by-cct
#14
Thomas A Treibel, Marianna Fontana, Jennifer A Steeden, Arthur Nasis, Jason Yeung, Steven K White, Sri Sivarajan, Shonit Punwani, Francesca Pugliese, Stuart A Taylor, James C Moon, Steve Bandula
BACKGROUND: The quantification of extracellular volume fraction (ECV) by Cardiac Computed Tomography (CCT) can identify changes in the myocardial interstitium due to fibrosis or infiltration. Current methodologies require laboratory blood hematocrit (Hct) measurement - which complicates the technique. The attenuation of blood (HUblood) is known to change with anemia. We hypothesized that the relationship between Hct and HUblood could be calibrated to rapidly generate a synthetic ECV without formally measuring Hct...
February 22, 2017: Journal of Cardiovascular Computed Tomography
https://www.readbyqxmd.com/read/28265790/value-of-tissue-doppler-derived-tei-index-and-two-dimensional-speckle-tracking-imaging-derived-longitudinal-strain-on-predicting-outcome-of-patients-with-light-chain-cardiac-amyloidosis
#15
Dan Liu, Kai Hu, Sebastian Herrmann, Maja Cikes, Georg Ertl, Frank Weidemann, Stefan Störk, Peter Nordbeck
Prognosis of patients with light-chain cardiac amyloidosis (AL-CA) is poor. Speckle tracking imaging (STI) derived longitudinal deformation parameters and Doppler-derived left ventricular (LV) Tei index are valuable predictors of outcome in patients with AL-CA. We estimated the prognostic utility of Tei index and deformation parameters in 58 comprehensively phenotyped patients with AL-CA after a median follow-up of 365 days (quartiles 121, 365 days). The primary end point was all-cause mortality. 19 (33%) patients died during follow-up...
March 6, 2017: International Journal of Cardiovascular Imaging
https://www.readbyqxmd.com/read/28256403/the-utility-of-99m-tc-dpd-scintigraphy-in-the-diagnosis-of-cardiac-amyloidosis-an-australian-experience
#16
Peter T Moore, Matthew K Burrage, Emily Mackenzie, W Philip Law, Dariusz Korczyk, Peter Mollee
BACKGROUND: The uptake of bone-seeking radiotracers in the amyloid heart is well recognised. 99(m)Tc-DPD has been shown to be highly sensitive for cardiac transthyretin (ATTR) amyloid in an overseas population, but is not registered for use in Australia. We explored its utility as a diagnostic tool within our population. METHODS: Patients diagnosed with AL and ATTR (wild-type and inherited) cardiac amyloidosis were prospectively recruited from the Princess Alexandra Hospital Amyloidosis Centre...
February 16, 2017: Heart, Lung & Circulation
https://www.readbyqxmd.com/read/28255101/amyloid-heart-disease-genetics-translated-into-disease-modifying-therapy
#17
REVIEW
Brett W Sperry, W H Wilson Tang
Given increased awareness and improved non-invasive diagnostic tools, cardiac amyloidosis has become an increasingly recognised aetiology of increased ventricular wall thickness and heart failure with preserved ejection fraction. Once considered a rare disease with no treatment options, translational research has harnessed novel pathways and led the way to promising treatment options. Gene variants that contribute to amyloid heart disease provide unique opportunities to explore potential disease-modifying therapeutic strategies...
March 2, 2017: Heart: Official Journal of the British Cardiac Society
https://www.readbyqxmd.com/read/28241246/prognosis-using-planar-imaging-in-cardiac-amyloidosis
#18
Brett W Sperry, Richard Brunken, Wael A Jaber
No abstract text is available yet for this article.
February 22, 2017: JAMA Cardiology
https://www.readbyqxmd.com/read/28241242/prognosis-using-planar-imaging-in-cardiac-amyloidosis-reply
#19
Adam Castaño, Sabahat Bokhari, Mathew S Maurer
No abstract text is available yet for this article.
February 22, 2017: JAMA Cardiology
https://www.readbyqxmd.com/read/28236336/cardiac-mr-elastography-for-quantitative-assessment-of-elevated-myocardial-stiffness-in-cardiac-amyloidosis
#20
Arvin Arani, Shivaram P Arunachalam, Ian C Y Chang, Francis Baffour, Phillip J Rossman, Kevin J Glaser, Joshua D Trzasko, Kiaran P McGee, Armando Manduca, Martha Grogan, Angela Dispenzieri, Richard L Ehman, Philip A Araoz
PURPOSE: To evaluate if cardiac magnetic resonance elastography (MRE) can measure increased stiffness in patients with cardiac amyloidosis. Myocardial tissue stiffness plays an important role in cardiac function. A noninvasive quantitative imaging technique capable of measuring myocardial stiffness could aid in disease diagnosis, therapy monitoring, and disease prognostic strategies. We recently developed a high-frequency cardiac MRE technique capable of making noninvasive stiffness measurements...
February 25, 2017: Journal of Magnetic Resonance Imaging: JMRI
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