References

bmjour

Байкальский медицинский журнал

Baikal Medical Journal

2949-0715

Irkutsk State Medical University

10.57256/2949-0715-2023-4-11-23

bmjour-166

Research Article

Научные обзоры литературы

Scientific literature reviews

АМИЛОИДНАЯ КАРДИОМИОПАТИЯ: ДИАГНОСТИКА И ЛЕЧЕНИЕ

AMYLOID CARDIOMYOPATHY: DIAGNOSIS AND TREATMENT

https://orcid.org/0000-0002-9069-3570

Енисеева

Елена Сергеевна

Eniseeva

Elena S

eniseeva-irk@yandex.ru

ФГБОУ ВО Иркутский государственный медицинский университет Минздрава России; Иркутская государственная медицинская Академия последипломного образованияРоссияIrkutsk State Medical Academy of Postgraduate Education – Branch of Russian Medical Academy of Continuing Professional Education, Irkutsk 664049, Russian Federation Irkutsk State Medical University, Irkutsk 664003, Russian FederationRussian Federation

2023

25122023

241123

2023

Енисеева Е.С.

Eniseeva E.S.

Данная работа распространяется под лицензией Creative Commons Attribution 4.0.

This work is licensed under a Creative Commons Attribution 4.0 License.

https://www.bmjour.ru/jour/article/view/166

В обзоре представлены современные данные о поражении сердца при системном амилоидозе. Обсуждаются варианты амилоидоза, при которых поражение сердца является частым: AL амилоидоз, ATTRwt и ATTRv амилоидоз. Описаны клинические признаки амилоидной кардиомиопатии, экстракардиальные проявления, позволяющие подозревать амилоидоз. Представлены методы диагностики амилоидной кардиомиопатии.

Эхокардиографические диагностические признаки включают увеличение толщины стенки левого желудочка, атриовентрикулярного клапана, стенки правого желудочка, утолщение межпредсердной перегородки, диастолическую дисфункцию и снижение общей продольной деформации при относительном сохранении продольной деформации верхушки.

Магнитно-резонансная томография позволяет выявить характерные для амилоидоза признаки: увеличение внеклеточного объема и диффузное субэндокардиальное или трансмуральное отсроченное контрастирование после введения гадолиния.

Сцинтиграфия с использованием препаратов, меченных технецием-99m, может подтвердить диагноз ATTR-кардиомиопатии. Диагноз ATTR-кардиомиопатии возможен только в сочетании с анализами крови и мочи для исключения наличия моноклонального белка. Скрининг на моноклональный белок включает 3 лабораторных теста: определение свободных легких цепей в сыворотке крови, электрофорез сывороточного белка и белка мочи с иммунофиксацией.

Обсуждаются показания для биопсии миокарда и роль биопсии экстракардиальных тканей. Эндомиокардиальная биопсия должна быть выполнена: а) если имеется высокое клиническое подозрение на амилоидоз сердца у пациента с моноклональным белком; б) если имеется высокое клиническое подозрение на сердечный амилоидоз, несмотря на отрицательный или двусмысленный результат сцинтиграфии; в) если сцинтиграфия сердца недоступна.

Обсуждаются возможности патогенетической терапии при ATTR и AL амилоидозе, направленной на прекращение или замедление отложения амилоида в сердце, симптоматическое лечение.

The review presents current data on heart disease in systemic amyloidosis. The article discusses variants of amyloi-dosis with common heart disease: AL-, ATTRwt- and ATTRv amyloidoses. Clinical signs of amyloid cardiomyopathy and extracardiac manifestations that allow one to suggest amyloidosis are described. Methods for diagnosing amyloid cardiomyopathy are presented.Echocardiographic diagnostic characters include increased thickness of the left ventricular wall, atrioventricular valve, right ventricular wall, thickening of the interatrial septum, and also diastolic dysfunction, and decreased global longi-tudinal strain with relative preservation of apical longitudinal strain.Magnetic resonance imaging reveals signs specific for amyloidosis: increased extracellular volume and diffuse suben-docardial or transmural delayed enhancement after gadolinium administration.Scintigraphy using technetium-99m labeled compounds can confirm the diagnosis of ATTR cardiomyopathy. The diag-nosis of ATTR cardiomyopathy is only possible in combination with blood and urine tests to exclude the presence of a monoclonal protein. Screening for monoclonal protein includes three laboratory tests: determination of free light chains in blood serum, electrophoresis of serum protein and urine protein with immunofixation.Indications for myocardial biopsy and the role of extracardiac tissue biopsy are discussed. Endomyocardial biopsy should be performed: a) in case of strong clinical suggestion of cardiac amyloidosis in a patient with monoclonal pro-tein; b) in case of strong clinical suggestion of cardiac amyloidosis, despite negative or equivocal scintigraphy result; c) if cardiac scintigraphy is not available.The possibilities of pathogenetic therapy for ATTR- and AL amyloidosis, aimed at stopping or slowing amyloid deposi-tion in the heart, and symptomatic treatment are discussed.

амилоидозкардиомиопатиятранстиретинлегкие цепи иммуноглобулиноврестриктивная кардиомиопатиясердечная недостаточность.

amyloidosiscardiomyopathytransthyretinlight chains of immunoglobulinsrestrictive cardiomyopathyheart failure

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The authors declare that there are no conflicts of interest present.