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Cardiac amyloidosis

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https://www.readbyqxmd.com/read/28822085/nuclear-imaging-of-cardiac-amyloidosis
#1
EDITORIAL
Efstathia Andrikopoulou, Pradeep Bhambhvani
No abstract text is available yet for this article.
August 18, 2017: Journal of Nuclear Cardiology: Official Publication of the American Society of Nuclear Cardiology
https://www.readbyqxmd.com/read/28812126/myocardial-iodine-concentration-measurement-using-dual-energy-computed-tomography-for-the-diagnosis-of-cardiac-amyloidosis-a-pilot-study
#2
Virgile Chevance, Thibaud Damy, Vania Tacher, François Legou, Fourat Ridouani, Alain Luciani, Hicham Kobeiter, Alain Rahmouni, Jean-François Deux
OBJECTIVE: To measure myocardium iodine concentration (MIC) in patients with cardiac amyloidosis (CA) using dual-energy computed tomography (DECT). METHODS: Twenty-two patients with CA, 13 with non-amyloid hypertrophic cardiomyopathies (CH) and 10 control patients were explored with pre-contrast, arterial and 5-minute DECT acquisition (Iomeprol; 1.5 mL/kg). Inter-ventricular septum (IVS) thickness, blood pool iodine concentration (BPIC), MIC (mg/mL), iodine ratio and extra-cellular volume (ECV) were calculated...
August 10, 2017: European Radiology
https://www.readbyqxmd.com/read/28808991/light-chain-al-amyloidosis-the-journey-to-diagnosis
#3
Kristen L McCausland, Michelle K White, Spencer D Guthrie, Tiffany Quock, Muriel Finkel, Isabelle Lousada, Martha S Bayliss
BACKGROUND: Light chain (AL) amyloidosis is a rare, complex disease associated with significant morbidity and mortality. Delays in diagnosis are common and may have detrimental consequences on patients' prognosis. Too little is known regarding the patient journey to diagnosis. OBJECTIVE: The objective of this study was to describe the patient-reported journey to a correct diagnosis for AL amyloidosis. METHODS: Using a mixed-methods approach, data were collected from clinician (n = 4) and patient (n = 10) interviews and a survey of community-based patients with AL amyloidosis (n = 341)...
August 14, 2017: Patient
https://www.readbyqxmd.com/read/28802308/vitreous-amyloidosis-with-autonomic-neuropathy-of-the-digestive-tract-associated-with-a-novel-transthyretin-p-gly87arg-variant-in-a-bangladeshi-patient-a-case-report
#4
Benjamin Terrier, Magali Colombat, Caroline Beugnet, Astrid Quéant, Jonathan London, Jean-Baptiste Daudin, Claire Le Jeunne, Luc Mouthon, Dominique Monnet, Cécile Cauquil, Catherine Lacroix, David Adams, Antoine Brézin, Sophie Valleix
BACKGROUND: Hereditary transthyretin amyloidosis is an autosomal dominant inherited disorder, first described in families with sensorimotor and autonomic neuropathy. Since its first description, more than 120 amyloidogenic transthyretin mutations have been reported with various geographic distributions and associated with a wide range of phenotypes involving the peripheral nerve, the heart, the gastrointestinal tract, the eyes, the central nervous system, or the kidneys. In some cases of transthyretin amyloidosis, the first clinical manifestation is vitreous opacity...
August 13, 2017: Journal of Medical Case Reports
https://www.readbyqxmd.com/read/28801204/improved-post-processing-strategy-for-molli-based-tissue-characterization-allows-application-in-patients-with-dyspnoe-and-impaired-left-ventricular-function
#5
Thomas Kampf, Wolfgang Rudolf Bauer, Theresa Reiter
Contrast and non-contrast MRI based characterization of myocardium by T1-mapping will be of paramount importance to obtain biomarkers, e.g. fibrosis, which determines the risk of heart failure patients. T1-mapping by the standard post-processing of the modified look-locker inversion recovery (MOLLI) lacks of accuracy when trying to reduce its duration, which on the other hand, is highly desirable in patients with heart failure. The recently suggested inversion group fitting (IGF) technique, which considers more parameters for fitting, has a superior accuracy for long T1 times despite a shorter duration...
August 8, 2017: Zeitschrift Für Medizinische Physik
https://www.readbyqxmd.com/read/28801077/myocardial-amyloid-quantification-with-look-locker-magnetic-resonance-sequence-in-cardiac-amyloidosis-diagnostic-accuracy-in-clinical-practice-and-histological-validation
#6
Eduardo Pozo, Jose M Castellano, Anubhav Kanwar, Rajiv Deochand, Mireia Castillo-Martin, Pablo Pazos-López, Carlos González-Lengua, Keren Osman, Matthew Cham, Carlos Cordon-Cardo, Jagat Narula, Valentin Fuster, Javier Sanz
BACKGROUND: Cardiac magnetic resonance (CMR) has demonstrated its utility in the non-invasive diagnosis of cardiac amyloidosis (CA). Our aim was to evaluate the ability of standard Look-Locker sequences to quantify amyloid deposition in CA. METHODS AND RESULTS: Consecutive patients referred for CMR for possible CA were retrospectively evaluated. Positive cardiac biopsy and/or typical pattern of late gadolinium enhancement were required for the diagnosis of CA. Post-contrast T1 values were obtained from Look-Locker sequences, and correlated with markers of severity of disease and major events...
August 8, 2017: Journal of Cardiac Failure
https://www.readbyqxmd.com/read/28772045/translational-registry-for-cardiomyopathies-torch-rationale-and-first-results
#7
Claudia Seyler, Benjamin Meder, Tanja Weis, Thea Schwaneberg, Kerstin Weitmann, Wolfgang Hoffmann, Hugo A Katus, Andreas Dösch
AIMS: Non-ischemic cardiomyopathies (CMPs) comprise heart muscle disorders of different causes with high variability in disease phenotypes and clinical progression. The lack of national structures for the efficient recruitment, clinical and molecular classification, and follow-up of patients with non-ischemic CMPs limit the thorough analysis of disease mechanisms and the evaluation of novel diagnostic and therapeutic strategies. This paper describes a national, prospective, multicenter registry for patients with non-ischemic CMPs...
August 2017: ESC Heart Failure
https://www.readbyqxmd.com/read/28768311/temporal-relation-between-myocardial-fibrosis-and-heart-failure-with-preserved-ejection-fraction-association-with-baseline-disease-severity-and-subsequent-outcome
#8
Erik B Schelbert, Yaron Fridman, Timothy C Wong, Hussein Abu Daya, Kayla M Piehler, Ajay Kadakkal, Christopher A Miller, Martin Ugander, Maren Maanja, Peter Kellman, Dipan J Shah, Kaleab Z Abebe, Marc A Simon, Giovanni Quarta, Michele Senni, Javed Butler, Javier Diez, Margaret M Redfield, Mihai Gheorghiade
Importance: Among myriad changes occurring during the evolution of heart failure with preserved ejection fraction (HFpEF), cardiomyocyte-extracellular matrix interactions from excess collagen may affect microvascular, mechanical, and electrical function. Objective: To investigate whether myocardial fibrosis (MF) is similarly prevalent both in those with HFpEF and those at risk for HFpEF, similarly associating with disease severity and outcomes. Design, Setting, and Participants: Observational cohort study from June 1, 2010, to September 17, 2015, with follow-up until December 14, 2015, at a cardiovascular magnetic resonance (CMR) center serving an integrated health system...
August 2, 2017: JAMA Cardiology
https://www.readbyqxmd.com/read/28760472/mitochondrial-dysfunction-in-atrial-tissue-of-patients-developing-postoperative-atrial-fibrillation
#9
Jelliffe Jeganathan, Rabya Saraf, Feroze Mahmood, Anam Pal, Manoj K Bhasin, Thomas Huang, Aaron Mittel, Ziyad Knio, Russell Simons, Kamal Khabbaz, Venkatachalam Senthilnathan, David Liu, Frank Sellke, Robina Matyal
BACKGROUND: Mitochondria are the major site of cellular oxidation. Metabolism and oxidative stress have been implicated as possible mechanisms for postoperative atrial fibrillation (POAF) after cardiac operations. Establishing the precise nature of mitochondrial dysfunction as an etiologic factor for oxidative stress-related cell death and apoptosis could further the understanding of POAF. To establish this relationship, mitochondrial function was studied in patients undergoing cardiac operations that developed POAF and compared it with patients without POAF...
July 28, 2017: Annals of Thoracic Surgery
https://www.readbyqxmd.com/read/28754546/comparison-of-different-stem-cell-mobilization-regimen-in-al-amyloidosis-patients
#10
Katharina Lisenko, Patrick Wuchter, Marion Hansberg, Anja Mangatter, Axel Benner, Anthony D Ho, Hartmut Goldschmidt, Ute Hegenbart, Stefan Schönland
High-dose melphalan (HDM) and autologous blood stem cell transplantation (ABSCT) is an effective treatment for transplant-eligible patients with systemic light chain (AL) amyloidosis. Whereas most centers use granulocyte colony-stimulating factor (G-CSF) alone for mobilization of peripheral blood stem cells (PBSC), the application of mobilization chemotherapy might offer specific advantages. We retrospectively analyzed 110 patients with AL amyloidosis who underwent PBSC collection. Major eligibility criteria included age <70 years and cardiac insufficiency NYHA ≤III°...
July 25, 2017: Biology of Blood and Marrow Transplantation
https://www.readbyqxmd.com/read/28754231/editorial-commentary-cardiac-amyloidosis-reversing-the-mindset-and-the-cardiomyopathy
#11
EDITORIAL
Brett W Sperry
No abstract text is available yet for this article.
July 18, 2017: Trends in Cardiovascular Medicine
https://www.readbyqxmd.com/read/28739313/cardiac-amyloidosis-an-update-on-pathophysiology-diagnosis-and-treatment
#12
REVIEW
Omar K Siddiqi, Frederick L Ruberg
The amyloidoses are a group of systemic diseases characterized by organ deposition of misfolded protein fragments of diverse origins. The natural history of the disease, involvement of other organs, and treatment options vary significantly based on the protein of origin. In AL amyloidosis, amyloid protein is derived from immunoglobulin light chains, and most often involves the kidneys and the heart. ATTR amyloidosis is categorized as mutant or wild-type depending on the genetic sequence of the transthyretin (TTR) protein produced by the liver...
July 13, 2017: Trends in Cardiovascular Medicine
https://www.readbyqxmd.com/read/28728693/broadening-the-phenotypic-spectrum-and%C3%A2-the-diagnostic-needs-of-ttr-related-cardiac-amyloidosis
#13
EDITORIAL
Claudio Rapezzi, Anna Laura Tinuper, Massimiliano Lorenzini
No abstract text is available yet for this article.
July 25, 2017: Journal of the American College of Cardiology
https://www.readbyqxmd.com/read/28728692/magnetic-resonance-in-transthyretin%C3%A2-cardiac-amyloidosis
#14
Ana Martinez-Naharro, Thomas A Treibel, Amna Abdel-Gadir, Heerajnarain Bulluck, Giulia Zumbo, Daniel S Knight, Tushar Kotecha, Rohin Francis, David F Hutt, Tamer Rezk, Stefania Rosmini, Candida C Quarta, Carol J Whelan, Peter Kellman, Julian D Gillmore, James C Moon, Philip N Hawkins, Marianna Fontana
BACKGROUND: Cardiac transthyretin amyloidosis (ATTR) is an increasingly recognized cause of heart failure. Cardiac magnetic resonance (CMR), with late gadolinium enhancement (LGE) and T1 mapping, is emerging as a reference standard for diagnosis and characterization of cardiac amyloidosis. OBJECTIVES: The authors used CMR with extracellular volume fraction (ECV) measurement to characterize cardiac involvement in relation to outcome in ATTR. METHODS: Subjects comprised 263 patients with cardiac ATTR corroborated by grade 2 to 3 (99m)Tc-DPD ((99m)Tc-3,3-diphosphono-1,2-propanodicarboxylic acid) cardiac uptake, 17 with suspected cardiac ATTR (grade 1 (99m)Tc-DPD), and 12 asymptomatic individuals with amyloidogenic transthyretin (TTR) mutations...
July 25, 2017: Journal of the American College of Cardiology
https://www.readbyqxmd.com/read/28719236/applying-an-artificial-neural-network-model-for-developing-a-severity-score-for-patients-with-hereditary-amyloid-polyneuropathy
#15
Shenia Novis, Felipe Machado, Victor B Costa, Debora Foguel, Marcia W Cruz, José Manoel de Seixas
Hereditary (familial) amyloid polyneuropathy (FAP) is a systemic disease that includes a sensorimotor polyneuropathy related to transthyretin (TTR) mutations. So far, a scale designed to classify the severity of this disease has not yet been validated. This work proposes the implementation of an artificial neural network (ANN) in order to develop a severity scale for monitoring the disease progression in FAP patients. In order to achieve this goal, relevant symptoms and laboratory findings were collected from 98 Brazilian patients included in THAOS - the Transthyretin Amyloidosis Outcomes Survey...
July 18, 2017: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/28705053/cardiac-amyloidosis-pathogenesis-clinical-context-diagnosis-and-management-options
#16
Hassan Alkhawam, Darshan Patel, James Nguyen, Saumya Mariam Easaw, Mohammed Al-Sadawi, Umer Syed, Feras Zaiem, Maher Homsi, Timothy J Vittorio
Amyloidosis covers a group of disorders that can manifest in virtually any organ system in the body and is thought to be secondary to misfolding of extracellular proteins with subsequent deposition in tissues. The precursor protein that is produced in excess defines the specific amyloid type. This requires histopathological confirmation using Congo-red dye with its characteristic demonstration of green birefringence under cross-polarized light. There are three main types of amyloidosis associated with cardiac involvement: light-chain (AL), familial or senile (ATTR), and secondary (AA) amyloidosis...
August 2017: Acta Cardiologica
https://www.readbyqxmd.com/read/28699793/predictors-of-early-treatment-failure-following-initial-therapy-for-systemic-immunoglobulin-light-chain-amyloidosis
#17
Nidhi Tandon, Surbhi Sidana, S Vincent Rajkumar, Angela Dispenzieri, Morie A Gertz, Martha Q Lacy, Robert A Kyle, Francis K Buadi, David Dingli, Suzanne R Hayman, Amie L Fonder, Miriam A Hobbs, Wilson I Gonsalves, Prashant Kapoor, Yi Lisa Hwa, Nelson Leung, Ronald S Go, John A Lust, Stephen J Russell, Steven R Zeldenrust, Shaji K Kumar
We analysed factors predicting early treatment failure (ETF), after first-line therapy for light-chain amyloidosis (AL). AL amyloidosis patients seen at Mayo Clinic within 90 days of diagnosis, from 2006 to 2015, excluding those who died within 3 months of initial therapy, were analysed retrospectively. ETF was defined as progression requiring treatment change or death within 12 (ETF12) or 24 (ETF24) months of first-line treatment. Non-ETF included those with a follow-up of more than 12 or 24 months who had progression beyond 12 or 24 months...
July 12, 2017: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/28699650/elevation-of-serum-lactate-dehydrogenase-in-al-amyloidosis-reflects-tissue-damage-and-is-an-adverse-prognostic-marker-in-patients-not-eligible-for-stem-cell-transplantation
#18
Eli Muchtar, Angela Dispenzieri, Martha Q Lacy, Francis K Buadi, Prashant Kapoor, Suzanne R Hayman, Wilson Gonsalves, Rahma Warsame, Taxiarchis V Kourelis, Rajshekhar Chakraborty, Stephen Russell, John A Lust, Yi Lin, Ronald S Go, Steven Zeldenrust, David Dingli, Nelson Leung, S Vincent Rajkumar, Robert A Kyle, Shaji K Kumar, Morie A Gertz
The significance of serum lactate dehydrogenase (LDH) in light chain (AL) amyloidosis has not been previously explored. We studied 1019 newly diagnosed patients and correlated the elevation of LDH above the upper limit of normal (ULN) with disease characteristics and outcome. Four hundred and nine patients had an LDH above ULN, representing 40% of the study population. Patients with an elevated LDH were older, were less likely to be male and had more extensive organ involvement compared to patients with a normal LDH...
July 12, 2017: British Journal of Haematology
https://www.readbyqxmd.com/read/28689003/primary-amyloidosis-with-renal-involvement-outcomes-in-77-consecutive-patients-at-a-single-center
#19
Sandy W Wong, Denis Toskic, Melissa Warner, Cindy Varga, Alejandro Moreno-Koehler, Daniel Fein, Teresa Fogaren, Lisa Lee, Colin M Oliver, Spencer D Guthrie, Raymond L Comenzo
BACKGROUND: Outcomes in primary amyloid renal patients are of interest as the era of monoclonal antibody therapies begins. PATIENTS AND METHODS: We studied 77 consecutive primary amyloid renal patients (58% men) for renal progression (end stage renal disease [ESRD]), renal response (RR), and overall survival (OS). RESULTS: At diagnosis median age was 63 (range, 35-81) years, estimated glomerular filtration rate 70 mL/min (range, 5-114), difference between involved and uninvolved free light chains 127 mg/L (range, 1-9957), ESRD 4%, renal stage 2 and 3 78%, and cardiac stage 2 and 3 56%...
June 17, 2017: Clinical Lymphoma, Myeloma & Leukemia
https://www.readbyqxmd.com/read/28687586/a-rash-with-a-heavy-heart
#20
Sergio Barros-Gomes, Niyada Naksuk, Dragan Jevremovic, Hector R Villarraga
Cardiac amyloidosis (CA) is relatively rare and frequently misdiagnosed. Other disorders presenting with increased left ventricular (LV) mass can mimic its diagnosis. This case illustrates unique findings of primary light chain (AL) amyloidosis in a patient with remarkable signs of CA. Here, we report a 49-year-old man with prior diagnosis of hypertrophy cardiomyopathy (HCM) based on an echocardiogram performed 1 year earlier that presented with 8 weeks of periorbital rash. He had numbness in the past 3 years...
July 7, 2017: Echo Research and Practice
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