Cardiac amyloidosis is an underdiagnosed condition associated with a poor prognosis, likely leading to heart failure, malignant arrhythmias and death. A high index of suspicion should be maintained in regards to clinical, electrocardiographic and echocardiographic changes, as the "g...
Systemic amyloidosis (SA) is a group of rare diseases in which abnormal misfolded proteins, amyloid fibrils, accumulate in tissue. The gold standard for diagnosis is tissue biopsy of the affected organ, but it is invasive and needs specific proteomic or immunohistochemistry analyses to characterize ...
“Cardiac amyloidosis” is the term commonly used to reflect the deposition of abnormal protein amyloid in the heart. This process can result from several different forms, most commonly from light-chain (AL) amyloidosis and transthyretin (ATTR) amyloidosis, which in turn can represent wild-type (...
et al. Endomyocardial biopsy characterization of heart failure with preserved ejection fraction and prevalence of cardiac amyloidosis. JACC Heart Fail. 8, 712–724 (2020). Article PubMed PubMed Central Google Scholar Burke, M. A. et al. Prognostic importance of pathophysiologic markers in ...
Heart failure (HF) involves ventricular dysfunction that ultimately leads to the heart not providing tissues with adequate blood for metabolic needs. In heart failure with reduced ejection fraction (HFrEF), the ventricle contracts poorly and empties inadequately; ejection fraction is low. ...
and endocardial biopsy for giant cell myocarditis. Performing right heart catheterization and cardiopulmonary exercise testing frequently provides vital information for managing heart failure. Serum and urine immunofixation with a technetium 99 pyrophosphate scan for cardiac amyloidosis. Additionally, urine or...
ventricular (red arrow) myocardia. Endomyocardial biopsy (EMB) from the right ventricle and gene mutation test confirmed the patient had a wild-type transthyretin amyloidosis with cardiomyopathy. Treatment with tafamidis meglumine ...
Pellikka PA, Holmes DR Jr., Edwards WD, Nishimura RA, Tajik AJ, Kyle RA. Endomyocardial biopsy in 30 patients with primary amyloidosis and suspected cardiac involvement. Arch Intern Med. 1988;148(3):662–6. ArticlePubMedCASGoogle Scholar ...
SPECT for cardiac amyloidosis [140],18F-FDG PET in inflammatory diseases, and nuclear MPI,123I-MIBG SPECT, and18F-FDG PET in TTC [147]. In women with dyspnea and preserved systolic LVEF, an in-depth screening for HFpEF must be considered, based on the four-step algorithm previously ...
Abdominal fat biopsy was negative for Congo red stain, nevertheless a bone marrow biopsy was performed, revealing lambda protein monoclonal plasmocytosis, confirming the diagnosis of primary systemic amyloidosis. This case represents a rare cause of heart failure in a young adult. Low-voltage QRS ...