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Диагностика амилоидоза сердца с применением современных ультразвуковых технологий

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Амилоидоз является системным заболеванием, которое обусловлено отложением нерастворимых белковых фибрилл. При амилоидном поражении сердца развивается тяжелая сердечная недостаточность, зачастую рефрактерная к терапии. При этом степень тяжести сердечной недостаточности не соответствует степени снижения систолической функции левого желудочка, так как в большей степени нарушается диастолическая функция. В настоящее время существуют схемы лечения данного заболевания, поэтому важна его диагностика на ранней стадии. “Золотым стандартом” диагностики кардиального амилоидоза является биопсия миокарда. Однако на современном этапе весомое значение имеют и неинвазивные методы.Цель работы: обзор признаков амилоидоза сердца, выявляемых с помощью эхокардиографии, в том числе с применением современных ультразвуковых технологий, позволяющих заподозрить амилоидное поражение сердца и инициировать дальнейшее дообследование.

Ключевые слова:
амилоидоз сердца, амилоидная кардиомиопатия, эхокардиография, спекл-трекинг-эхокардиография, продольная деформация, скручивание левого желудочка, раскручивание левого желудочка, cardiac amyloidosis, amyloid cardiomyopathy, echocardiography, speckle tracking echocardiography, longitudinal deformation, left ventricular torsion, left ventricular untwisting

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Diagnosis of cardiac amyloidosis with the use of modern ultrasound techniques

Amyloidosis is a systemic disease caused by accumulation of insoluble amyloid proteins in the tissues. Cardiac amyloidosis (CA) lead to a severe heart failure, which is often therapy resistant. The severity of heart failure does not correspond to the degree of decrease in systolic function of the left ventricle, since diastolic function is more impairing. Early diagnosis of cardiac amyloidosis is important due to the presence of treatment regimens for this disease nowadays. The “gold standard” for cardiac amyloidosis diagnosis is myocardial biopsy, but non-invasive diagnostic methods are also of great importance. The aim of the article is a review of the ultrasound signs of cardiac amyloidosis, including the use of modern technologies, which helps to suspect CA to provide a further examination.

Keywords:
амилоидоз сердца, амилоидная кардиомиопатия, эхокардиография, спекл-трекинг-эхокардиография, продольная деформация, скручивание левого желудочка, раскручивание левого желудочка, cardiac amyloidosis, amyloid cardiomyopathy, echocardiography, speckle tracking echocardiography, longitudinal deformation, left ventricular torsion, left ventricular untwisting