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Cardiac amyloidosis is a subcategory of amyloidosis where there is depositing of the protein amyloid in the cardiac muscle and surrounding tissues. Amyloid, a misfolded and insoluble protein, can become a deposit in the heart's atria, valves, or ventricles .
Amyloidosis is a group of diseases in which abnormal proteins, known as amyloid fibrils, build up in tissue. [4] There are several non-specific and vague signs and symptoms associated with amyloidosis. [5] These include fatigue, peripheral edema, weight loss, shortness of breath, palpitations, and feeling faint with standing. [5]
Studies comparing synthetic to recombinant β amyloid peptide in assays measuring rate of fibrillation, fibril homogeneity, and cellular toxicity showed that recombinant β amyloid peptide has a faster fibrillation rate and greater toxicity than synthetic β amyloid peptide. [64] There are multiple classes of amyloid-forming polypeptide sequences.
Amyloid cardiomyopathy (stiff heart syndrome) [5] is a condition resulting in the death of part of the myocardium (heart muscle). It is associated with the systemic production and release of many amyloidogenic proteins , especially immunoglobulin light chain or transthyretin (TTR). [ 6 ]
Unlike the situation in AL amyloidosis, the ECG voltage is often normal, although low voltage may be seen (despite increased wall thickness on echocardiography). Marked axis deviation, bundle branch block, and AV block are common, as is atrial fibrillation. [citation needed]
Vascular dementia can sometimes be triggered by cerebral amyloid angiopathy, which involves accumulation of amyloid beta plaques in the walls of the cerebral arteries, leading to breakdown and rupture of the vessels. [2] [5] Since amyloid plaques are a characteristic feature of Alzheimer's disease, vascular dementia may occur as a consequence ...
AA amyloidosis is a form of amyloidosis, a disease characterized by the abnormal deposition of fibers of insoluble protein in the extracellular space of various tissues and organs. In AA amyloidosis, the deposited protein is serum amyloid A protein (SAA), an acute-phase protein which is normally soluble and whose plasma concentration is highest ...
Calcium channel blockers are generally contraindicated due to their negative inotropic effect, particularly in cardiomyopathy caused by amyloidosis. [ 18 ] [ 19 ] Digoxin, calcium channel blocking drugs and beta-adrenergic blocking agents provide little benefit, except in the subgroup of restrictive cardiomyopathy with atrial fibrillation. [ 20 ]
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