Characterizing cardiac involvement in amyloidosis using cardiovascular magnetic resonance diffusion tensor imaging

In-vivo cardiovascular magnetic resonance (CMR) diffusion tensor imaging (DTI) allows imaging of alterations of cardiac fiber architecture in diseased hearts. Cardiac amyloidosis (CA) causes myocardial infiltr...
Source: Journal of Cardiovascular Magnetic Resonance - Category: Radiology Authors: Tags: Research Source Type: research

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​The wrist is not commonly aspirated in the emergency department, but emergent arthrocentesis may be indicated for extreme or concerning cases, and tapping the wrist to determine the underlying pathology or relieve pain may be of great value. The synovial fluid from the joint space can be analyzed for crystals, infection, and blood. This information may help determine the overall plan and aid in decision-making and consultation. The ultimate treatment plan may include admission, intravenous antibiotics, multiple aspirations, and even surgical washout.A swollen, painful wrist that is hot to the touch is concerning for sep...
Source: The Procedural Pause - Category: Emergency Medicine Tags: Blog Posts Source Type: blogs
Amyloid transthyretin (ATTR) cardiac amyloidosis (CA) is an increasingly recognized cause of restrictive cardiomyopathy and associated heart failure with preserved ejection fraction (HFpEF). Despite improved diagnostic techniques to identify this condition, many patients experience delayed diagnosis. Interpretation of routine cardiac biomarkers (i.e. troponins and brain natriuretic peptide (BNP)) and echocardiography in ATTR-CA is limited, particularly in relation to pyrophosphate (PYP) scintigraphy.
Source: The Journal of Heart and Lung Transplantation - Category: Transplant Surgery Authors: Tags: (64) Source Type: research
Medical treatment for cardiac amyloidosis (CA) is evolving rapidly. Heart transplantation can be a valid option when followed by transplantation of bone marrow or liver, dependent on the type and origin of the amyloid protein. Thus, accurate typing of amyloidosis has implications for treatment, prognosis, and genetic counseling. Although non-invasive diagnostic techniques can type CA, endomyocardial biopsy (EMB) may be needed in the case of equivocal imaging findings or discordant data. We aimed to define the role of mass spectrometry (MS) for diagnosis and subtyping of CA.
Source: The Journal of Heart and Lung Transplantation - Category: Transplant Surgery Authors: Tags: (566) Source Type: research
After initial efforts at heart transplantation (HT) in patients with light chain amyloidosis were fraught with poor outcomes, emerging experience with modern chemotherapy regimens has led to a major improvement in outcomes in select centers. Given the systemic nature of the disease and the added immunosuppression in the form of chemotherapy, this study sought to analyze if AL patients undergoing HT had a higher burden of readmissions and infections non-amyloid heart transplant patients.
Source: The Journal of Heart and Lung Transplantation - Category: Transplant Surgery Authors: Tags: (697) Source Type: research
Advanced amyloid cardiomyopathy (ACM) patients have high waitlist (WL) mortality. Given the greater emphasis and clarity for status exceptions for ACM patients in the new allocation system, we sought to assess whether this change in the allocation policy would affect the WL and post-transplant outcomes in ACM pts.
Source: The Journal of Heart and Lung Transplantation - Category: Transplant Surgery Authors: Tags: (660) Source Type: research
Limited data exists on the use continuous-flow left ventricular assist device (CF-LVAD) in patients with cardiac amyloidosis (CA). The largest single center analysis on CF-LVAD support in patients with CA reports a 50% mortality at 6-months. Our primary aim was to examine the survival and morbidity of our patients with diagnosed CA and supported by CF-LVAD.
Source: The Journal of Heart and Lung Transplantation - Category: Transplant Surgery Authors: Tags: (1086) Source Type: research
Advances in amyloidosis management have improved patient longevity. Coupling potential increased transplantation for amyloid patients with graft scarcity, we intend to assess the use of extended-donor criteria (EDC) hearts for amyloidosis.
Source: The Journal of Heart and Lung Transplantation - Category: Transplant Surgery Authors: Tags: (102) Source Type: research
AbstractTafamidis, a non ‐nonsteroidal anti‐inflammatory benzoxazole derivative, acts as a transthyretin (TTR) stabilizer to slow progression of TTR amyloidosis (ATTR). Tafamidis meglumine, available as 20‐mg capsules, is approved in more than 40 countries worldwide for the treatment of adults with early‐stage sympt omatic ATTR polyneuropathy. This agent, administered as an 80‐mg, once‐daily dose (4 × 20‐mg capsules), is approved in the United States, Japan, Canada, and Brazil for the treatment of hereditary and wild‐type ATTR cardiomyopathy in adults. An alternative single solid oral dosage formulati...
Source: Clinical Pharmacology in Drug Development - Category: Drugs & Pharmacology Authors: Tags: Original Manuscript Source Type: research
European Journal of Heart Failure, Volume 22, Issue 3, Page 389-390, March 2020.
Source: European Journal of Heart Failure - Category: Cardiology Authors: Tags: INTRODUCTION Source Type: research
ConclusionSurvival in patients with ATTR-PN is highly variable and affected by non-cardiac baseline characteristics, such as autonomic dysfunction, large fiber involvement, late-onset disease, and non-Val30Met mutation. Careful interpretation of these findings is warranted given that this synthesis did not control for differences between studies. Survival in patients with ATTR-PN remains poor among those who are untreated or with delayed diagnosis.
Source: Neurology and Therapy - Category: Neurology Source Type: research
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