Articles: amyloidosis.
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It is well recognized that amyloid protein can infiltrate many regions of the body. This can include the peripheral nerves, the liver, kidney, spleen, the gastrointestinal tract, and most importantly the myocardium. The amyloid proteins that cause cardiomyopathy may come from genetically altered liver genes (transthyretin amyloid, ATTR) or from the bone marrow with malignant plasma cells (light chain amyloid, AL) generating the aberrant protein. ⋯ In the operating room patients are exposed to dramatic hemodynamic changes and may have difficult airways, autonomic dysfunction, and conduction abnormalities. Although the topic of amyloidosis is well described in cardiology literature, it is underdiagnosed. The purpose of this review is to describe some of the pathophysiology behind the principle proteins that cause cardiac amyloidosis and to comprehensively describe perioperative considerations for anesthesia providers.
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Studies addressing the prevalence of cardiac amyloidosis (CA) among patients with spinal stenosis (SS) are lacking. The identification of the red flags (RF) of CA could lead to early detection of cases of CA. The primary objective of this study was to address the prevalence of RF of CA among patients with SS. ⋯ The prevalence of RF of CA is high among patients with SS and YLH. A high proportion of patients met the CA suspicion criteria.
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Randomized Controlled Trial
Efficacy and Safety of Acoramidis in Transthyretin Amyloid Cardiomyopathy.
Transthyretin amyloid cardiomyopathy is characterized by the deposition of misfolded monomeric transthyretin (TTR) in the heart. Acoramidis is a high-affinity TTR stabilizer that acts to inhibit dissociation of tetrameric TTR and leads to more than 90% stabilization across the dosing interval as measured ex vivo. ⋯ In patients with transthyretin amyloid cardiomyopathy, the receipt of acoramidis resulted in a significantly better four-step primary hierarchical outcome containing components of mortality, morbidity, and function than placebo. Adverse events were similar in the two groups. (Funded by BridgeBio Pharma; ATTRibute-CM ClinicalTrials.gov number, NCT03860935.).