Leading Factors of Progression in Patients with Cardiac Amyloidosis

Rameeva, A.S.; Рамеев, В.В.; Bobkova, Irina; Сафарова, А. Ф.; Kobalava, Zh. D.; Moiseev, Sergey

doi:10.20996/1819-6446-2022-04-02

Cited by 2 publications

(1 citation statement)

References 16 publications

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“…Был предложен коэффициент деформации ФВ ЛЖ с пороговым значением 4,1, позволяющим с точностью 91% отличить амилоидоз сердца от ГКМП или нормы [38]. Обычно наблюдается тяжелое нарушение базальной продольной деформации с относительным сохранением апикальной деформации [39]. Это "апикальное сохранение" одновременно обладает высокой чувствительностью и специфичностью для диагностики амилоидоза сердца [6].…”

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Red flags to diagnose infiltrative cardiomyopathies

et al. 2023

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Infiltrative cardiomyopathies are a group of diseases characterized by the deposition of abnormal substances in heart tissues, which leads to thickening of the walls or dilation of chambers with a secondary decrease in wall thickness and the development of diastolic, less often systolic, ventricular dysfunction. Most often, these are progressive diseases that, in the absence of adequate therapy, have an unfavorable prognosis. Clinical manifestations of infiltrative cardiac diseases are variable, which often leads to diagnostic difficulties and errors. In most cases, specific laboratory and morphological tests are required to confirm or clarify the diagnosis. Early diagnosis is critical to initiating therapy and improving patient prognosis. This article provides characteristic signs and symptoms, the so-called "red flags", making it possible to suspect infiltrative cardiomyopathies, diagnose them at an early stage and start life-saving therapy.

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Section: эхокгunclassified

Red flags to diagnose infiltrative cardiomyopathies

et al. 2023

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History of the study of amyloidosis: from the Rokitansky’s theory to the present day

Rameev,

Lysenko

2024

Terapevticheskii arkhiv

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In the history of amyloidosis studying the concept of liquids dyscrasia has been predominated and finally it is resulted in accepting a serum protein-precursor as a leading amyloidogenic factor in the disease pathogenesis. Consequently basic diagnostic and treatment strategy was aimed to find and eliminate this protein from the blood and this approach evidenced high effectiveness in most frequent AA and AL-amyloidosis characterized with anomaly high levels of precursors in the blood. At the same time there are less frequent and slower progressing inheritant forms of systemic amyloidosis including transthyretin induced, which are less depending on amyloidogenecity of amyloid precursor and because of that, in example, the effectiveness of transthyretin stabilizers or blockers of its synthesis is limited comparing with the precursor elimination in AA or AL. Developed in the middle of XX century a theory of local synthesis by macrophages is more preferable to describe the pathogenesis of these forms. And modern proteome analysis using give rise to confirm the key meaning of macrophage in the amyloidogenesis and proves necessity to know deeply mechanisms of macrophagial autophagia – basic tool of maintaining intracellular protein homeostasis. That is why it is difficult to hope on high effectiveness of chemical amyloid solvents in vivo, which being under macrophages regulation never could realize its chemical activities.

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Leading Factors of Progression in Patients with Cardiac Amyloidosis

Cited by 2 publications

References 16 publications

Red flags to diagnose infiltrative cardiomyopathies

Red flags to diagnose infiltrative cardiomyopathies

History of the study of amyloidosis: from the Rokitansky’s theory to the present day

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