Annals of the Russian academy of medical sciencesAnnals of the Russian academy of medical sciencesВестник Российской академии медицинских наук0869-60472414-3545"Paediatrician" Publishers LLC118410.15690/vramn1184CARDIOLOGY AND CARDIOVASCULAR SURGERY: CURRENT ISSUESАКТУАЛЬНЫЕ ВОПРОСЫ КАРДИОЛОГИИ И СЕРДЕЧНО-СОСУДИСТОЙ ХИРУРГИИResearch ArticleHeart involvement in AL-amyloidosis. Current state of the issueПоражение сердца при AL-амилоидозе. Состояние проблемыhttps://orcid.org/0000-0003-1092-65631911-9569TrukhinIvan V.ТрухинИван Викторович
Russian Federation

Department of Preventive and Emergency Cardiology, resident physician

Кафедра профилактической и неотложной кардиологии, врач-ординатор

ivan.trukhin@gmail.comhttps://orcid.org/0000-0002-8209-27913753-6915SchekochikhinDmitrij Yu.ЩехочихинДмитрий Юрьевич
Russian Federation

MD, PhD

к.м.н., доцент

agishm@list.ruhttps://orcid.org/0000-0002-1645-781X7876-8609NovikovaNina A.НовиковаНина Александровна
Russian Federation

MD, PhD, Professor

д.м.н., профессор

nanovikova@mail.ruhttps://orcid.org/0000-0003-3417-794X4566-7759AkselrodAnna S.АксельродАнна Сергеевна
Russian Federation

д.м.н., профессор

7402898@mail.ruhttps://orcid.org/0000-0002-9602-292X8884-8014SyrkinAbram L.СыркинАбрам Львович
Russian Federation

MD, PhD, Professor

д.м.н., профессор

previntenscardiology@yandex.ruhttps://orcid.org/0000-0002-8072-45137553-0371SyrkinaHelena A.СыркинаЕлена Абрамовна
Russian Federation

MD, PhD

к.м.н., доцент

e_syrkina@mail.ruhttps://orcid.org/0000-0001-7221-93924281-3923GrachevAlexander E.ГрачёвАлександр Евгеньевич
Russian Federation

MD, PhD

к.м.н.

Gra4al@yandex.ruhttps://orcid.org/0000-0002-4155-78203772-7386GribanovaHelena O.ГрибановаЕлена Олеговна
Russian Federation

MD, PhD

к.м.н.

el.gribanova@mail.ruhttps://orcid.org/0000-0002-2457-14282821-3160OkhotaValeria K.ОхотаВалерия Константиновна
Russian Federation
Sheveleva10@gmail.comhttps://orcid.org/0000-0003-1221-34951289-1850RyzhkoVyacheslav V.РыжкоВячеслав Владимирович
Russian Federation

MD, PhD

к.м.н., доцент

Gra4al@yandex.ruhttps://orcid.org/0000-0002-3952-68657311-9276PershinaEkaterina S.ПершинаЕкатерина Сергеевна
Russian Federation
pershina86@mail.ruI.M. Sechenov First Moscow State Medical University (Sechenov University)Первый Московский государственный медицинский университет имени И.М. Сеченова (Сеченовский Университет)National Research Center for HematologyНациональный медицинский исследовательский центр гематологииPirogov First Moscow Clinical HospitalГородская клиническая больница № 1 им. Н.И. Пирогова19112019041220197453073162008201905112019Copyright ©; 2019, "Paediatrician" Publishers LLCCopyright ©; 2019, Издательство "Педиатръ"2019"Paediatrician" Publishers LLCИздательство "Педиатръ"https://vestnikramn.spr-journal.ru/jour/article/view/1184

AL cardiac amyloidosis is a relatively rare disorder that belongs to the group of infiltrative cardiomyopathies. Diagnosis of primary amyloidosis is challenging due to many unspecific symptoms and sings, which often leads to late diagnosis when treatment options are limited. Primary amyloidosis particularly needs to be excluded in patients with heart failure with preserved ejection fraction. Therapy in cardiac amyloidosis has to main vectors: 1) chemotherapy to eliminate amyloidogenic plasmatic cells 2) heart failure treatment. The main challenge for cardiologists is to support hemodynamics until response to chemotherapy occurs. In the article the issue of diagnostics, risk stratification and treatment of primary cardiac amyloidosis is addressed.

Актуальность. AL-амилоидоз сердца относится к группе инфильтративных кардиомиопатий и является относительно редким заболеванием. Диагностика амилоидоза сердца представляет большие трудности ввиду множества неспецифичных симптомов системного характера, что зачастую приводит к поздней постановке диагноза, когда варианты лечения довольно ограничены. Диагноз амилоидоза обязательно должен быть исключен у пациентов с сердечной недостаточностью с сохранной фракцией выброса. Лечение пациентов с амилоидозом сердца включает два основных направления ― проведение химиотерапии с целью уничтожения амилоидогенных плазматических клеток и лечение сердечной недостаточности. Основной задачей кардиолога является поддержание гемодинамики до получения ответа на химиотерапию. В статье рассматриваются вопросы диагностики, стратификации риска и лечения больных с первичным амилоидозом сердца.

Заключение. Использование современной химиотерапии демонстрирует значительное улучшение выживаемости.

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