Cardiac Amyloidosis: Evolving Diagnosis and Management: A Scientific Statement From the American Heart Association.

Cardiac Amyloidosis: Evolving Diagnosis and Management: A Scientific Statement From the American Heart Association. Circulation. 2020 Jun 01;:CIR0000000000000792 Authors: Kittleson MM, Maurer MS, Ambardekar AV, Bullock-Palmer RP, Chang PP, Eisen HJ, Nair AP, Nativi-Nicolau J, Ruberg FL, American Heart Association Heart Failure and Transplantation Committee of the Council on Clinical Cardiology Abstract Transthyretin amyloid cardiomyopathy (ATTR-CM) results in a restrictive cardiomyopathy caused by extracellular deposition of transthyretin, normally involved in the transportation of the hormone thyroxine and retinol-binding protein, in the myocardium. Enthusiasm about ATTR-CM has grown as a result of 3 simultaneous areas of advancement: Imaging techniques allow accurate noninvasive diagnosis of ATTR-CM without the need for confirmatory endomyocardial biopsies; observational studies indicate that the diagnosis of ATTR-CM may be underrecognized in a significant proportion of patients with heart failure; and on the basis of elucidation of the mechanisms of amyloid formation, therapies are now approved for treatment of ATTR-CM. Because therapy for ATTR-CM may be most effective when administered before significant cardiac dysfunction, early identification of affected individuals with readily available noninvasive tests is essential. This scientific statement is intended to guide clinical practice and to facilitate management conformity by covering current diag...
Source: Circulation - Category: Cardiology Authors: Tags: Circulation Source Type: research

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CONCLUSION: Molecular imaging represent a sensitive tool for early assessment of both amyloid burden and cardiac innervation, to differentiate between subtypes and to monitor disease burden, disease progression, and potential response to therapy. PMID: 32525789 [PubMed - as supplied by publisher]
Source: Current Radiopharmaceuticals - Category: Radiology Tags: Curr Radiopharm Source Type: research
1366Objectives: CURRENT ROLE OF PET IMAGING IN CARDIAC AMYLOIDOSIS Cardiac amyloidosis (CA) is a relatively rare disease characterized by extracellular deposition of abnormal protein fibrils in the heart, resulting in restrictive cardiomyopathy and heart failure with preserved ejection fraction. Majority of CA occurs due to deposition of two types of amyloid protein: monoclonal immunoglobulin light chains (AL amyloidosis) or transthyretin (ATTR), either wild or mutant type. Differentiating the two most common types of amyloid deposition in the heart is of paramount importance in guiding therapy and prognostication. Diagnos...
Source: Journal of Nuclear Medicine - Category: Nuclear Medicine Authors: Tags: Cardiovascular Sciences (Poster Session) Source Type: research
AbstractPurpose of ReviewTransthyretin amyloidosis is an increasingly recognized cause of restrictive cardiomyopathy related to amyloid fibril deposition in cardiac tissues. As treatment therapies have emerged for transthyretin amyloidosis (ATTR), so has interest in using biomarkers to identify disease prior to advanced presentation.Recent FindingsLower levels of transthyretin and retinol binding protein-4 have been demonstrated in patients with pathogenic mutations of transthyretin either with or without clinical disease. Levels associate with the severity of mutations as well as response to treatment with transthyretin s...
Source: Current Heart Failure Reports - Category: Cardiology Source Type: research
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
We describe a rare case of concomitant moderately severe tricuspid and mitral valve stenosis because of ongoing amyloid deposition in a patient with progressive multiple myeloma and fat pad biopsy ‐proven light chain amyloidosis. Worsening infiltrative cardiomyopathy and valvulopathy despite evidence‐based chemotherapy and heart failure pharmacotherapy led to end‐stage disease and death. Valvular involvement in cardiac amyloidosis requires early recognition of the underlying disease con dition to guide directed medical therapy and prevent its progression. In this instance, valvuloplasty or valve replacement is not a viable option.
Source: ESC Heart Failure - Category: Cardiology Authors: Tags: Case Report Source Type: research
Cardiac Amyloidosis (CAm) is an infiltrative disease caused by abnormal extracellular protein deposits in the myocardium, leading to restrictive cardiomyopathy. The cardinal clinical manifestation is congestion resulting in symptomatic heart failure (HF). Relief of congestion is achieved with high dose loop diuretics and regularly requires hospitalization for intravenous (IV) diuresis. Additionally, CAm is a challenge to manage given sensitivity to preload and afterload, low systemic blood pressures, and inefficacy of advanced therapies typically used in HF.
Source: Heart and Lung - Category: Intensive Care Authors: Source Type: research
Abstract Cardiac amyloidosis (CA) is an infiltrative and restrictive cardiomyopathy that leads to heart failure, reduced quality of life, and death. The disease has two main subtypes, transthyretin cardiac amyloidosis (ATTR-CA) and immunoglobulin light chain cardiac amyloidosis (AL-CA), characterized by the nature of the infiltrating protein. ATTR-CA is further subdivided into wild-type (ATTRwt-CA) and variant (ATTRv-CA) based on the presence or absence of a mutation in the transthyretin gene. CA is significantly underdiagnosed and increasingly recognized as a cause of heart failure with preserved ejection fractio...
Source: Annual Review of Medicine - Category: General Medicine Authors: Tags: Annu Rev Med Source Type: research
Publication date: Available online 17 December 2019Source: Canadian Journal of CardiologyAuthor(s): Bennett Di Giovanni, Dakota Gustafson, Mitchell B. Adamson, Diego H. DelgadoAbstractAmyloidosis is a term used to describe a group of rare, heterogenous diseases which ultimately result in the deposition and accumulation of misfolded proteins. These misfolded proteins, known as amyloid, are associated with a variety of precursor proteins which have amyloidogenic potential. Ultimately, the specific type of amyloidosis is dependent on multiple factors including genetic variability of precursor proteins and the tissue/organ in ...
Source: Canadian Journal of Cardiology - Category: Cardiology Source Type: research
This article will review ATTR cardiac amyloidosis to provide physicians with the tools they need to establish a definitive diagnosis when there is a clinical suspicion of amyloidosis and provide the most appropriate care. Expert commentary: Increased awareness and improved diagnostic techniques will lead to earlier diagnosis and a greater understanding of the clinical presentation. The anticipated increases in the prevalence of this disease due to increased clinical awareness will require, and in-part, facilitate the development of new therapies to manage this patient population. PMID: 31478389 [PubMed - as supplied by publisher]
Source: Expert Review of Cardiovascular Therapy - Category: Cardiology Tags: Expert Rev Cardiovasc Ther Source Type: research
Rationale: Cardiac amyloidosis, considered for the last years to be a rare disease, is one of the determinants of HFpEF. The non-specific clinical presentation and the difficulties related to endomyocardial biopsy have made cardiac amyloidosis an underdiagnosed clinical entity. Improvement of non-invasive diagnostic techniques and the development of new therapies increased clinical awareness for this form of restrictive cardiomyopathy. We here summarize echocardiography and 99mTc-HDP scintigraphy findings in 6 cases of cardiac amyloidosis and review the literature data of this progressive and fatal cardiomyopathy. Patie...
Source: Medicine - Category: Internal Medicine Tags: Research Article: Clinical Case Report Source Type: research
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