State of the Art: Quantitative Cardiac MRI in Cardiac Amyloidosis
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Abstract:
Cardiac amyloidosis (CA) is characterized by amyloid infiltration in the myocardial extracellular space, causing heart failure. Patients with CA are currently underdiagnosed. Cardiac involvement is significantly associated with the prognosis and treatment decision‐making for CA. Early identification and accurate stratification are the crucial first step in patient management. Comprehensive cardiac MRI‐based evaluation of the cardiac structure, function, and myocardial tissue characterization assesses cardiac involvement by tracing disease processes. Emerging quantitative tissue characterization techniques have introduced new measures that can identify early staged CA and monitor disease progression or response after treatment. Quantitative cardiac MRI is becoming an instrumental tool in understanding CA, which leads to changes in individualized patient care. This review aimed to discuss the quantitative cardiac MRI‐based assessment of CA using established and emerging techniques. Evidence Level 5 Technical Efficacy Stage 3Keywords:
Cardiac Amyloidosis
Cardiac Imaging
Cardiac Amyloidosis
Amyloid (mycology)
Endomyocardial Biopsy
Nuclear imaging
Restrictive cardiomyopathy
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Cardiac amyloidosis is caused by the deposition of misfolded protein fibrils into the extracellular space of the heart. The diagnosis of cardiac amyloidosis remains challenging because of the heterogeneous manifestations of the disease. There are many different types of amyloidosis with light-chain (AL) amyloidosis and transthyretin (ATTR) amyloidosis being the most common types of cardiac amyloidosis. Endomyocardial biopsy is considered the gold standard for diagnosing cardiac amyloidosis and differentiating amyloid subtypes, but its use is limited because of the invasive nature of the procedure, with risks for complications and the need for specialized training and centers to perform the procedure. Radionuclide cardiac imaging has recently become the most commonly performed test for the diagnosis of ATTR amyloidosis but is of limited value for the diagnosis of AL amyloidosis. Positron emission tomography has been increasingly used for the diagnosis of cardiac amyloidosis and its applications are expected to expand in the future. Imaging protocols are under refinement to achieve better quantification of the disease burden and prediction of prognosis.
Cardiac Amyloidosis
Endomyocardial Biopsy
Amyloid (mycology)
AL amyloidosis
Restrictive cardiomyopathy
Nuclear imaging
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Cardiac Amyloidosis
AL amyloidosis
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Cardiac amyloidosis is expressed as a restrictive myocardiopathy. Echocardiography suggests the diagnosis. There is a great difference between the prognosis of senile cardiac amyloidosis and "AL" amyloidosis. We illustrate this point with two case reports.
Cardiac Amyloidosis
Restrictive cardiomyopathy
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Cardiac Amyloidosis
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Cardiac amyloidosis is accumulation in the heart of a pathologic fibrillar protein amyloid. It represents a heterogeneous group of states from clinically insignificant amyloid accumulation in isolated atrial amyloidosis to severe involvement of the heart in primary amyloidosis when mean duration of life equals to 6 months. Insufficient awareness of physicians of this pathology leads to erroneous and belated diagnosis of cardiac amyloidosis. This paper contains contemporary data of pathophysiology, clinical manifestation, diagnosis, treatment, and prognosis of various variants of cardiac amyloidosis.
Cardiac Amyloidosis
Amyloid (mycology)
Pathophysiology
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Cardiac Amyloidosis
Endomyocardial Biopsy
AL amyloidosis
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Cardiac Amyloidosis
Clinical Significance
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Cardiac Amyloidosis
Amyloid (mycology)
Standardized uptake value
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Cardiac amyloidosis occurs when misfolded protein fibrils are deposited in the extracellular space of the myocardium. The most common causes for cardiac amyloidosis are light chain (AL) amyloidosis and transthyretin (ATTR) amyloidosis. ATTR amyloidosis can occur with ageing due to the accumulation of wild-type transthyretin or can due to an inherited genetic mutation. One of the more common inherited forms in the United Kingdom (UK) is associated with the V122I mutation. I have described the clinical features, electrocardiogram and multimodality imaging findings in the most common forms of ATTR cardiac amyloidosis diagnosed in the UK. I found significant differences between ATTR amyloidosis types. In particular, those with V122I associated ATTR cardiac amyloidosis present at a younger age than those with wild-type disease but have a more advanced cardiac phenotype at diagnosis and a worse survival. I identified predictors of survival in transthyretin cardiac amyloidosis which were predominantly those associated with a more severe cardiac phenotype and found that the most common form of death in these patients is from heart failure. I examined the use of DPD in ATTR and AL cardiac amyloidosis and showed that higher DPD grades are associated with more severe cardiac disease in ATTR amyloidosis. However, DPD grade does not predict survival in ATTR amyloidosis and I demonstrated that this is also the case in AL amyloidosis. I conducted a retrospective study to examine the effect of the non-steroidal anti-inflammatory drug, diflunisal, in ATTR amyloidosis. I found that even in highly selected patients, diflunisal was poorly tolerated and I did not find any evidence for benefit. I described the UK experience of a phase 2 trial of a silencing RNA treatment, revusiran. I described challenges to recruitment and patient characteristics for those I recruited.
Cardiac Amyloidosis
Diflunisal
Amyloid (mycology)
AL amyloidosis
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