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Cardiac amyloidosis

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https://www.readbyqxmd.com/read/28447284/apical-sparing-pattern-of-left-ventricular-myocardial-99m-tc-hmdp-uptake-in-patients-with-transthyretin-cardiac-amyloidosis
#1
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
#2
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
#3
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
#4
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
#5
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
#6
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
#7
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
#8
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
#9
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
#10
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
https://www.readbyqxmd.com/read/28430919/non-coding-rnas-in-cardiovascular-diseases-diagnostic-and-therapeutic-perspectives
#11
Wolfgang Poller, Stefanie Dimmeler, Stephane Heymans, Tanja Zeller, Jan Haas, Mahir Karakas, David-Manuel Leistner, Philipp Jakob, Shinichi Nakagawa, Stefan Blankenberg, Stefan Engelhardt, Thomas Thum, Christian Weber, Benjamin Meder, Roger Hajjar, Ulf Landmesser
Recent research has demonstrated that the non-coding genome plays a key role in genetic programming and gene regulation during development as well as in health and cardiovascular disease. About 99% of the human genome do not encode proteins, but are transcriptionally active representing a broad spectrum of non-coding RNAs (ncRNAs) with important regulatory and structural functions. Non-coding RNAs have been identified as critical novel regulators of cardiovascular risk factors and cell functions and are thus important candidates to improve diagnostics and prognosis assessment...
April 18, 2017: European Heart Journal
https://www.readbyqxmd.com/read/28425041/familial-amyloid-cardiomyopathy-masquerading-as-chronic-guillain-barre-syndrome-things-are-not-always-what-they-seem
#12
Die Hu, Ling Liu, Shuguang Yuan, Yuhong Yi, Daoquan Peng
Familial amyloid cardiomyopathy is a challenging condition that mimics many other diseases, particularly in patients with pronounced neurological presentations and unexplained or equivocal cardiac abnormalities. In this case, a 57-year-old man was admitted for outpatient cardiological evaluation of progressive right heart failure and limb paraesthesias. The patient presented with hypertension, chronic Guillain-Barre syndrome, and sick sinus syndrome. Transthoracic echocardiograms showed a thickened ventricular wall and enlarged atrium...
April 19, 2017: Frontiers of Medicine
https://www.readbyqxmd.com/read/28413910/phenotypic-characterization-of-late-onset-cardiac-amyloidosis-caused-by-the-transthyretin-mutation-ttra45s-p-ala65ser
#13
Katrine M Müllertz, Steen Baerentzen, Henrik K Jensen, Henning Mølgaard
No abstract text is available yet for this article.
April 16, 2017: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/28405093/two-causes-in-one-patient-for-extremely-low-voltage-on-the-electrocardiogram
#14
William C Roberts, Melody Joy Sherwood, Paul A Grayburn
An 80-year-old woman is described with two different causes (pericardial effusion and cardiac amyloidosis) for low QRS voltage on the electrocardiogram. Total 12-lead QRS voltage (from the peak of the R wave to the nadir of either the Q or the S wave, whichever is deeper) was only 34 mm (10 mm standard in all leads), the lowest we have encountered among 331 previously reported patients with 10 different cardiac conditions.
April 2017: Proceedings of the Baylor University Medical Center
https://www.readbyqxmd.com/read/28395866/cardiac-amyloidosis-phenotype-associated-with-a-glu89lys-transthyretin-mutation
#15
Pierre R Bourque, Arleigh R McCurdy, Lisa M Mielniczuk, Carole Dennie, John P Veinot, Jodi Warman Chardon
We report a 45-year-old man with rapidly progressive cardiac amyloidosis, who required heart transplantation within 2 years of symptomatic onset. Hematologic testing and initial tissue biopsy results confirmed amyloid infiltration but were inconclusive for the amyloidogenic protein source. Mass spectroscopy and transthyretin (TTR) sequencing were required to reach a diagnosis of TTR amyloidosis resulting from a Glu89Lys mutation. Although a predominantly neuropathic phenotype has previously been described with this mutation, the present kinship documents a primarily cardiac presentation...
February 3, 2017: Canadian Journal of Cardiology
https://www.readbyqxmd.com/read/28384285/impact-of-genotype-and-phenotype-on-cardiac-biomarkers-in-patients-with-transthyretin-amyloidosis-report-from-the-transthyretin-amyloidosis-outcome-survey-thaos
#16
Arnt V Kristen, Mathew S Maurer, Claudio Rapezzi, Rajiv Mundayat, Ole B Suhr, Thibaud Damy
AIM: Cardiac troponins and natriuretic peptides are established for risk stratification in light-chain amyloidosis. Data on cardiac biomarkers in transthyretin amyloidosis (ATTR) are lacking. METHODS AND RESULTS: Patients (n = 1617) with any of the following cardiac biomarkers, BNP (n = 1079), NT-proBNP (n = 550), troponin T (n = 274), and troponin I (n = 108), available at baseline in the Transthyretin Amyloidosis Outcomes Survey (THAOS) were analyzed for differences between genotypes and phenotypes and their association with survival...
2017: PloS One
https://www.readbyqxmd.com/read/28373528/addressing-common-questions-encountered-in-the-diagnosis-and-management-of-cardiac-amyloidosis
#17
REVIEW
Mathew S Maurer, Perry Elliott, Raymond Comenzo, Marc Semigran, Claudio Rapezzi
Advances in cardiac imaging have resulted in greater recognition of cardiac amyloidosis in everyday clinical practice, but the diagnosis continues to be made in patients with late-stage disease, suggesting that more needs to be done to improve awareness of its clinical manifestations and the potential of therapeutic intervention to improve prognosis. Light chain cardiac amyloidosis, in particular, if recognized early and treated with targeted plasma cell therapy, can be managed very effectively. For patients with transthyretin amyloidosis, there are numerous therapies that are currently in late-phase clinical trials...
April 4, 2017: Circulation
https://www.readbyqxmd.com/read/28367859/identification-and-assessment-of-cardiac-amyloidosis-by-myocardial-strain-analysis-of-cardiac-magnetic-resonance-imaging
#18
Seitaro Oda, Daisuke Utsunomiya, Takeshi Nakaura, Hideaki Yuki, Masafumi Kidoh, Kosuke Morita, Seiji Takashio, Megumi Yamamuro, Yasuhiro Izumiya, Kyoko Hirakawa, Toshifumi Ishida, Kenichi Tsujita, Mitsuharu Ueda, Taro Yamashita, Yukio Ando, Hiroyuki Hata, Yasuyuki Yamashita
BACKGROUND: We explored the usefulness of myocardial strain analysis on cardiac magnetic resonance imaging (CMR) scans for the identification of cardiac amyloidosis.Methods and Results:The 61 patients with systemic amyloidosis underwent 3.0-T CMR, including CMR tagging and late-gadolinium enhanced (LGE) imaging. The circumferential strain (CS) of LGE-positive and LGE-negative patients was measured on midventricular short-axis images and compared. Logistic regression modeling of CMR parameters was performed to detect patients with LGE-positive cardiac amyloidosis...
March 31, 2017: Circulation Journal: Official Journal of the Japanese Circulation Society
https://www.readbyqxmd.com/read/28366333/high-prevalence-of-recurrent-nocturnal-desaturations-in-systemic-al-amyloidosis-a-cross-sectional-pilot-study
#19
Shameem Mahmood, Milind Sovani, Peter Smith, Leena George, Christina C Quarta, Sajitha Sachchithanantham, Marianna Fontana, Carol J Whelan, Helen J Lachmann, Julian D Gillmore, Philip N Hawkins, Ashutosh D Wechalekar
OBJECTIVES: Cardiac involvement and/or macroglossia with soft tissue deposits are risk factors for central sleep apnoea (CSA) and obstructive sleep apnoea (OSA), and common features of systemic AL amyloidosis. Little data exist on the occurrence of sleep-disordered breathing (SDB) or recurrent nocturnal hypoxia in amyloidosis, which this study sought to investigate. METHODS: A total of 72 consecutive patients with systemic amyloidosis (mean age 69 years and mean BMI 25) were evaluated for occurrence of SDB, by overnight continuous pulse oximetry, and completed Epworth Sleepiness Score (ESS) and STOPBANG questionnaires...
April 2017: Sleep Medicine
https://www.readbyqxmd.com/read/28364177/magnetic-resonance-imaging-for-characterizing-myocardial-diseases
#20
REVIEW
Maythem Saeed, Hui Liu, Chang-Hong Liang, Mark W Wilson
The National Institute of Health defined cardiomyopathy as diseases of the heart muscle. These myocardial diseases have different etiology, structure and treatment. This review highlights the key imaging features of different myocardial diseases. It provides information on myocardial structure/orientation, perfusion, function and viability in diseases related to cardiomyopathy. The standard cardiac magnetic resonance imaging (MRI) sequences can reveal insight on left ventricular (LV) mass, volumes and regional contractile function in all types of cardiomyopathy diseases...
March 31, 2017: International Journal of Cardiovascular Imaging
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