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Cardiac amyloidosis

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https://www.readbyqxmd.com/read/28528458/cardiac-amyloidosis-diagnosis-and-treatment-strategies
#1
REVIEW
Mirela Tuzovic, Eric H Yang, Arnold S Baas, Eugene C Depasquale, Mario C Deng, Daniel Cruz, Gabriel Vorobiof
Cardiac amyloidosis in the United States is most often due to myocardial infiltration by immunoglobulin protein, such as in AL amyloidosis, or by the protein transthyretin, such as in hereditary and senile amyloidosis. Cardiac amyloidosis often portends a poor prognosis especially in patients with systemic AL amyloidosis. Despite better understanding of the pathophysiology of amyloid, many patients are still diagnosed late in the disease course. This review investigates the current understanding and new research on the diagnosis and treatment strategies in patients with cardiac amyloidosis...
July 2017: Current Oncology Reports
https://www.readbyqxmd.com/read/28523319/amyloidosis-a-rare-cause-of-refractory-heart-failure-in-a-young-female
#2
Irina Iuliana Costache, Claudia Florida Costea, Mihai Danciu, Victor Vlad Costan, Viviana Aursulesei, Gabriela FlorenŢa Dumitrescu, Mihaela Dana Turliuc, Anca Sava
Cardiac amyloidosis may occur in any type of systemic amyloidosis. The clinical picture is often characterized by restrictive cardiomyopathy. We report the case of a 41-year-old female patient admitted to the Department of Cardiology with clinical signs of right heart failure: congested jugular veins, hepatomegaly, peripheral edema, ascites associated with atrial fibrillation, low values of arterial blood pressure and oliguria. Echocardiographic findings were helpful for the diagnosis of cardiac amyloidosis: enlarged atrial cavities, normal size ventricles, thickened ventricular septum and posterior left ventricle wall with normal left ventricular ejection fraction, mitral and tricuspid regurgitation...
2017: Romanian Journal of Morphology and Embryology, Revue Roumaine de Morphologie et Embryologie
https://www.readbyqxmd.com/read/28522573/lenalidomide-melphalan-dexamethasone-in-newly-diagnosed-patients-with-al-amyloidosis-results-of-a-prospective-phase-2-study-with-long-term-follow-up
#3
Ute Hegenbart, Tilmann Bochtler, Axel Benner, Natalia Becker, Christoph Kimmich, Arnt V Kristen, Jörg Beimler, Ernst Hund, Markus Zorn, Anja Freiberger, Marianne Gawlik, Hartmut Goldschmidt, Dirk Hose, Anna Jauch, Anthony D Ho, Stefan O Schönland
Chemotherapy in light chain amyloidosis aims to normalize the involved free light chain in serum, which leads to an improvement or at least stabilization of organ function in most responding patients. We performed a prospective single center phase 2 trial with 50 untreated patients not eligible for high-dose treatment. The treatment schedule comprised 6 cycles of oral lenalidomide, melphalan and dexamethasone every 4 weeks. After 6 months complete remission was achieved in 9 patients (18%), very good partial remission in 16 (32%) and partial response in 9 (18%)...
May 18, 2017: Haematologica
https://www.readbyqxmd.com/read/28508350/targeted-nuclear-imaging-probes-for-cardiac-amyloidosis
#4
REVIEW
Paco E Bravo, Sharmila Dorbala
PURPOSE OF REVIEW: The aim of the present manuscript is to review the latest advancements of radionuclide molecular imaging in the diagnosis and prognosis of individuals with cardiac amyloidosis. RECENT FINDINGS: (99m)Technetium labeled bone tracer scintigraphy had been known to image cardiac amyloidosis, since the 1980s; over the past decade, bone scintigraphy has been revived specifically to diagnose transthyretin cardiac amyloidosis. (18)F labeled and (11)C labeled amyloid binding radiotracers developed for imaging Alzheimer's disease, have been repurposed since 2013, to image light chain and transthyretin cardiac amyloidosis...
July 2017: Current Cardiology Reports
https://www.readbyqxmd.com/read/28494620/genetic-testing-improves-identification-of-transthyretin-amyloid-attr-subtype-in-cardiac-amyloidosis
#5
Emily E Brown, Yi Zhen Joan Lee, Marc K Halushka, Charles Steenbergen, Nicole M Johnson, Johana Almansa, Ryan J Tedford, Oscar Cingolani, Stuart D Russell, Kavita Sharma, Daniel P Judge
Amyloidosis is a group of conditions characterized by the accumulation of amyloid deposits in various tissues. Among these disorders, ATTR amyloidosis occurs either with or without a TTR pathogenic variant. Treatment for amyloidosis depends on the subtype, which is often identified through a tissue biopsy followed by liquid chromatography tandem mass spectrometry (LC-MS/MS). Genetic testing may be done to confirm these results for patients with ATTR amyloidosis; however, the necessity of genetic testing after LC-MS/MS has not been evaluated...
May 11, 2017: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/28493201/nuclear-cardiology-practice-in-spain
#6
Amelia Jimenez-Heffernan, Santiago Aguade-Bruix, Irene Casans-Tormo
BACKGROUND: In Spain, nuclear cardiology (NC) procedures represent the second most frequently performed studies in nuclear medicine (NM) centers. METHODS: The NC Working Group of the Spanish Society of Nuclear Medicine and Molecular Imaging invited NM departments across the country to answer an online questionnaire regarding 2014 activity. RESULTS: Data on 40,161 patients from 42 centers were collected. The responding public centers served 39% of Spain´s population...
May 10, 2017: Journal of Nuclear Cardiology: Official Publication of the American Society of Nuclear Cardiology
https://www.readbyqxmd.com/read/28475415/transthyretin-amyloidosis-a-phenocopy-of-hypertrophic-cardiomyopathy
#7
Alexa M C Vermeer, Anneloes Janssen, Peter C Boorsma, Marcel M A M Mannens, Arthur A M Wilde, Imke Christiaans
OBJECTIVES: Hypertrophic cardiomyopathy (HCM) is an inherited cardiac disorder that affects over one in 500 persons worldwide. The autosomal dominant transmission of HCM implies that many relatives are at risk for HCM associated morbidity and mortality, therefore genetic testing and counselling is of great importance. However, in only 50-60% of the patients a mutation is found, which hampers predictive genetic testing in relatives. In HCM patients in whom the causal mutation has not been identified (yet), phenocopies of HCM - i...
May 5, 2017: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/28460244/late-onset-cardiomyopathy-as-presenting-sign-of-attr-a45g-amyloidosis-caused-by-a-novel-ttr-mutation-p-a65g
#8
Sebastiaan H C Klaassen, Henny H Lemmink, Johan Bijzet, Andor W J M Glaudemans, Reinhard Bos, Wouter Plattel, Maarten P van den Berg, Riemer H J A Slart, Hans L A Nienhuis, Dirk J van Veldhuisen, Bouke P C Hazenberg
OBJECTIVE: The clinical description of a novel TTR gene mutation characterized by a late onset amyloid cardiomyopathy. METHODS AND RESULTS: A 78-year-old man of Dutch origin with recent surgery for bilateral carpal tunnel syndrome (CTS) was admitted to our hospital because of heart failure with preserved ejection fraction (55%). Cardiac ultrasound showed thickened biventricular walls, and cardiac magnetic resonance imaging also showed late gadolinium enhancement...
April 18, 2017: Cardiovascular Pathology: the Official Journal of the Society for Cardiovascular Pathology
https://www.readbyqxmd.com/read/28457657/stem-cell-transplantation-for-immunoglobulin-light-chain-amyloidosis
#9
REVIEW
Taimur Sher, Morie A Gertz
Systemic chemotherapy aimed at eradicating transformed plasma cells is the mainstay of treatment for immunoglobulin light chain amyloidosis (AL). Autologous stem cell transplantation (SCT) is a highly effective treatment for AL and can lead to long term survival in excess of 10 years in patients who achieve complete remission. Since AL is a unique disease characterized by multiple organ dysfunction, SCT poses unique challenges in this disease. Morbidity and mortality of SCT has remarkably improved over time primarily due to careful selection of patients and evolution of predictive and prognostic models based on serum immunoglobulin light chains and cardiac biomarkers...
March 16, 2017: Current Problems in Cancer
https://www.readbyqxmd.com/read/28456755/light-chain-cardiac-amyloidosis-strategies-to-promote-early-diagnosis-and-cardiac-response
#10
REVIEW
Martha Grogan, Angela Dispenzieri, Morie A Gertz
Amyloid light chain (AL) amyloidosis is a systemic disease characterised by the aggregation of misfolded immunoglobulin light chain (LC), predominantly in the heart and kidneys, causing organ failure. If untreated, the median survival of patients with cardiac AL amyloidosis is 6 months from the onset of heart failure. Protracted time to establish a diagnosis, often lasting >1 year, is a frequent factor in poor treatment outcomes. Cardiologists, to whom patients are often referred, frequently miss the opportunity to diagnose cardiac AL amyloidosis...
April 29, 2017: Heart: Official Journal of the British Cardiac Society
https://www.readbyqxmd.com/read/28447284/apical-sparing-pattern-of-left-ventricular-myocardial-99m-tc-hmdp-uptake-in-patients-with-transthyretin-cardiac-amyloidosis
#11
Axel Van Der Gucht, Anne-Ségolène Cottereau, Mukedaisi Abulizi, Aziz Guellich, Paul Blanc-Durand, Jean-Marc Israel, Arnault Galat, Violaine Plante-Bordeneuve, Jean-Luc Dubois-Randé, Diane Bodez, Jean Rosso, Thibaud Damy, Emmanuel Itti
BACKGROUND: A decreased longitudinal strain in basal segments with a base-to-apex gradient has been described in patients with cardiac amyloidosis (CA). OBJECTIVES: Aim was to investigate the left ventricular (LV) regional distribution of early-phase (99m)Tc-Hydroxymethylene diphosphonate ((99m)Tc-HMDP) uptake in patients with transthyretin-related cardiac amyloidosis (TTR-CA). METHODS: All patients underwent a whole-body planar (99m)Tc-HMDP scintigraphy acquired at 10-min post-injection (early-phase) followed by a thorax SPECT/CT...
April 26, 2017: Journal of Nuclear Cardiology: Official Publication of the American Society of Nuclear Cardiology
https://www.readbyqxmd.com/read/28444296/challenging-the-myths-of-cardiac-amyloidosis
#12
Omar K Siddiqi, Frederick L Ruberg
No abstract text is available yet for this article.
April 24, 2017: European Heart Journal
https://www.readbyqxmd.com/read/28435766/a-heart-too-stiff-to-beat-a-case-of-familial-transthyretin-amyloidosis-cardiomyopathy
#13
Yan Zhou, Sameen Khalid, Aamer Abbass, Laura Hughes, Marcos Hazday
Heart failure is a common clinical syndrome caused by a variety of cardiac diseases. We report a rare case of familial transthyretin amyloidosis cardiomyopathy to heighten the awareness of this rare but lethal cause of heart failure, as therapeutic interventions such as liver or heart transplant could be curative in selected patients.
March 20, 2017: Curēus
https://www.readbyqxmd.com/read/28434361/feasibility-study-of-cardiac-magnetic-resonance-elastography-in-cardiac-amyloidosis
#14
Ian C Y Chang, Arvin Arani, Shivaram Poigai Arunachalam, Martha Grogan, Angela Dispenzieri, Philip A Araoz
No abstract text is available yet for this article.
March 2017: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/28434357/retinol-binding-protein-4-rbp4-concentration-identifies-v122i-transthyretin-cardiac-amyloidosis
#15
Marios Arvanitis, Steven Simon, Gloria Chan, Denise Fine, Paula Beardsley, Michael LaValley, Daniel Jacobson, Clarissa Koch, John L Berk, Lawreen H Connors, Frederick L Ruberg
No abstract text is available yet for this article.
March 2017: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/28434295/standard-heart-failure-medication-in-cardiac-transthyretin-amyloidosis-useful-or-harmful
#16
Fabian Aus dem Siepen, Selina Hein, Ralf Bauer, Hugo A Katus, Arnt V Kristen
No abstract text is available yet for this article.
March 2017: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/28434293/severity-and-reversibility-of-cardiac-dysfunction-and-residual-concentration-of-amyloidogenic-light-chain-predict-overall-survival-of-patients-with-al-amyloidosis-who-attain-complete-response
#17
Giovanni Palladini, Paolo Milani, Marco Basset, Francesca Russo, Francesca Lavatelli, Mario Nuvolone, Giovanni Ferraro, Margherita Bozzola, Andrea Foli, Stefano Perlini, Giampaolo Merlini
No abstract text is available yet for this article.
March 2017: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/28434267/amyloid-in-bone-marrow-smears-in-systemic-light-chain-amyloidosis
#18
Christoph Kimmich, Stefan Schönland, Sandra Kräker, Mindaugas Andrulis, Anthony D Ho, Gudrun Mayer, Tobias Dittrich, Michael Hundemer, Ute Hegenbart
We performed a prospective sensitivity analysis to detect amyloid in bone marrow (BM) smears stained with Congo red (CR) and according to Pappenheim of patients with systemic light-chain (AL) amyloidosis. Results were directly compared to routine BM histology and fat aspiration. We analysed 198 BM smears from patients with the diagnosis or suspicion of systemic AL amyloidosis. Ultimately, the diagnosis could be established for 168 patients. Amyloid was detected on BM smears with CR in 33% (56/168). All patients suspicious for amyloid on Pappenheim staining (n = 39) showed substantial amyloid infiltration on CR...
April 23, 2017: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/28433439/cardiac-amyloidosis-shows-decreased-diastolic-function-as-assessed-by-echocardiographic-parameterized-diastolic-filling
#19
Katrin Salman, Peter A Cain, Benjamin T Fitzgerald, Martin G Sundqvist, Martin Ugander
Cardiac amyloidosis is a rare but serious condition with poor survival. One of the early findings by echocardiography is impaired diastolic function, even before the development of cardiac symptoms. Early diagnosis is important, permitting initiation of treatment aimed at improving survival. The parameterized diastolic filling (PDF) formalism entails describing the left ventricular filling pattern during early diastole using the mathematical equation for the motion of a damped harmonic oscillator. We hypothesized that echocardiographic PDF analysis could detect differences in diastolic function between patients with amyloidosis and controls...
April 19, 2017: Ultrasound in Medicine & Biology
https://www.readbyqxmd.com/read/28432670/the-complementary-nature-of-tissue-doppler-to-99m-tc-pyp-imaging-in-diagnosis-of-right-ventricular-cardiac-amyloidosis
#20
Mirza Mujadil Ahmad, Jaswant Basraon, Imaad Razzaque, Steven C Port, Khawaja Afzal Ammar
No abstract text is available yet for this article.
April 21, 2017: Journal of Nuclear Cardiology: Official Publication of the American Society of Nuclear Cardiology
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