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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 .
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 ]
Amyloidosis of the central nervous system can have more severe and systemic presentations that may include life-threatening arrhythmias, cardiac failure, malnutrition, infection, or death. [14] Neuropathic presentation can depend on the etiology of amyloidosis. [14]
Pages in category "Deaths from amyloidosis" The following 26 pages are in this category, out of 26 total. This list may not reflect recent changes. B. Bernie Boston;
Mutant proteins can disturb cardiac function in the contractile apparatus (or mechanosensitive complexes). Cardiomyocyte alterations and their persistent responses at the cellular level cause changes that are correlated with sudden cardiac death and other cardiac problems. [27] Cardiomyopathies are generally varied individually.
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 ]
Wild-type transthyretin amyloid (WTTA), also known as senile systemic amyloidosis (SSA), [1] is a disease that typically affects the heart and tendons of elderly people. It is caused by the accumulation of a wild-type (that is to say a normal ) protein called transthyretin .
70–80% of those with AL amyloidosis have heart involvement, and heart involvement is the leading cause of death. [4] Heart complications, include heart failure and irregular heart beat . Early heart involvement in AL amyloidosis may present as low voltage electrical rhythms on an electrocardiograph , concentric left ventricular hypertrophy ...
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