nmpЖурнал им. Н.В. Склифосовского «Неотложная медицинская помощь»Russian Sklifosovsky Journal "Emergency Medical Care"2223-90222541-8017“N.V. Sklifosovsky Research Institute for Emergency Medicine”10.23934/2223-9022-2025-14-2-387-397nmp-2184Research ArticleОБЗОР ЛИТЕРАТУРЫREVIEWSСовременные аспекты диагностики и лечения дилатационной кардиомиопатии с ремоделированием правых отделов сердцаModern Aspects of Diagnosis and Treatment of Dilated Cardiomyopathy With Remodeling of the Right Hearthttps://orcid.org/0000-0002-0746-1884ХубутияМ. Ш.KhubutiyaM. Sh.

Хубутия Могели Шалвович - академик РАН, профессор, доктор медицинских наук, президент ГБУЗ «НИИ СП им. Н.В. Склифосовского ДЗМ»; заведующий кафедрой трансплантологии и искусственных органов Научно-образовательного института «Высшая школа клинической медицины им. Н.А. Семашко» ФГБОУ ВО «Российский университет медицины» МЗ РФ.

129090, Москва, Большая Сухаревская пл., 3; 127473, Москва, ул. Долгоруковская, д. 4

Mogeli Sh. Khubutiya - Academician of the Russian Academy of Sciences, Full Professor, Doctor of Medical Sciences, President, N.V. Sklifosovsky Research Institute for Emergency Medicine; Head, Department of Transplantology and Artificial Organs, Scientific and Educational Institute “Higher School of Clinical Medicine named after N.A. Semashko”, Russian University of Medicine.

Bolshaya Sukharevskaya Sq. 3, Moscow, 129090; Dolgorukovskaya Str. 4, Moscow, 127473

khubutiyams@sklif.mos.ruhttps://orcid.org/0000-0002-6229-8629АлиджановаХ. Г.AlidzhanovaKh. G.

Алиджанова Хафиза Гафуровна - доктор медицинских наук, старший преподаватель учебного центра, старший научный сотрудник отделения неотложной кардиологии с методами неинвазивной диагностики ГБУЗ «НИИ СП им. Н.В. Склифосовского ДЗМ».

129090, Москва, Большая Сухаревская пл., 3

Khafiza G. Alidzhanova - Doctor of Medical Sciences, Senior Lecturer, Training Center; Senior Researcher, Scientific Department of Emergency Cardiology with Non-invasive Diagnostic Methods, N.V. Sklifosovsky Research Institute for Emergency Medicine.

Bolshaya Sukharevskaya Sq. 3, Moscow, 129090

alidzhanovahg@sklif.mos.ruhttps://orcid.org/0009-0004-5294-1352МолчановаЖ. В.MolchanovaZh. V.

Молчанова Жанна Владимировна - клинический ординатор по специальности «Кардиология» ГБУЗ «НИИ СП им. Н.В. Склифосовского ДЗМ».

129090, Москва, Большая Сухаревская пл., 3

Zhanna V. Molchanova - Cardiology Resident, N.V. Sklifosovsky Research Institute for Emergency Medicine.

Bolshaya Sukharevskaya Sq. 3, Moscow, 129090

zhanna1323@mail.ruhttps://orcid.org/0000-0002-2971-9188СагировМ. А.SagirovM. A.

Сагиров Марат Анварович - кандидат медицинских наук, заведующий научным отделом неотложной кардиохирургии ГБУЗ «НИИ СП им. Н.В. Склифосовского ДЗМ».

129090, Москва, Большая Сухаревская пл., 3

Marat A. Sagirov - Candidate of Medical Sciences, Head, Scientific Department of Emergency Cardiac Surgery, N.V. Sklifosovsky Research Institute for Emergency Medicine.

Bolshaya Sukharevskaya Sq. 3, Moscow, 129090

sagirovma@sklif.mos.ruhttps://orcid.org/0009-0007-7436-0681ИльинаЕ. В.IlyinaE. V.

Ильина Евгения Витальевна - клинический ординатор по специальности «Кардиология» ГБУЗ «НИИ СП им. Н.В. Склифосовского ДЗМ».

129090, Москва, Большая Сухаревская пл., 3

Evgeniya V. Ilyina - Cardiology Resident, N.V. Sklifosovsky Research Institute for Emergency Medicine.

Bolshaya Sukharevskaya Sq. 3, Moscow, 129090

osmakosma98@yandex.ruГБУЗ «Научно-исследовательский институт скорой помощи им. Н.В. Склифосовского ДЗМ», Научное отделение неотложной кардиологии с методами неинвазивной диагностики; ФГБОУ ВО «Российский университет медицины» МЗ РФРоссияN.V. Sklifosovsky Research Institute for Emergency Medicine, Scientific Department of Emergency Cardiology with Non-invasive Diagnostic Methods; Russian University of MedicineRussian FederationГБУЗ «Научно-исследовательский институт скорой помощи им. Н.В. Склифосовского ДЗМ», Научное отделение неотложной кардиологии с методами неинвазивной диагностикиРоссияN.V. Sklifosovsky Research Institute for Emergency Medicine, Scientific Department of Emergency Cardiology with Non-invasive Diagnostic MethodsRussian Federation202506092025142387397Copyright © Хубутия М.Ш., Алиджанова Х.Г., Молчанова Ж.В., Сагиров М.А., Ильина Е.В., 20252025Хубутия М.Ш., Алиджанова Х.Г., Молчанова Ж.В., Сагиров М.А., Ильина Е.В.Khubutiya M.S., Alidzhanova K.G., Molchanova Z.V., Sagirov M.A., Ilyina E.V.Данная работа распространяется под лицензией Creative Commons Attribution 4.0.This work is licensed under a Creative Commons Attribution 4.0 License.https://www.jnmp.ru/jour/article/view/2184

Дилатационная кардиомиопатия (ДКМП) — вторая по частоте причина хронической сердечной недостаточности и ведущее показание к трансплантации сердца. На ранних стадиях болезнь протекает бессимптомно, однако сохраняется высокий риск развития опасных для жизни желудочковых аритмий и внезапной сердечной смерти (ВСС). Современные методы диагностики ДКМП, такие как магнитно-резонансная томография (МРТ) сердца, спекл-трекинг и 3D-эхокардиография, убедительно доказали развитие структурно-функциональных изменений правых отделов сердца (ПОС) и их прогностическое значение. Методом МРТ сердца диагностируют фиброз миокарда (ФМ), возникающий на ранних стадиях ДКМП. Прогрессирующий фиброз наблюдается у одной пятой пациентов и связан с более чем 3-кратным увеличением риска смерти и осложнений хронической сердечной недостаточности. При адекватном фармакологическом и аппаратном лечении у части пациентов происходит обратное ремоделирование сердца. Однако оптимальная и длительная медикаментозная терапия не приводит к регрессии ФМ даже у лиц с улучшенной функцией левого желудочка (ЛЖ). Ремоделирование ПОС независимо от фракции выброса ЛЖ является предиктором ВСС, указывает на прогрессирование заболевания, предвещая неблагоприятный исход. Распространённость ремоделирования ПОС достоверно не определена, однако систолическая дисфункция правого желудочка зарегистрирована у 34–65% пациентов с ДКМП. Годовая и последующая смертность пациентов с ДКМП остается высокой. МРТ сердца с контрастным усилением стала золотым стандартом диагностики ДКМП и решающим для стратификации риска и прогноза заболевания. Лечение пациентов с ДКМП является сложным процессом. Адекватная медикаментозная и инструментальная терапия в 40% случаев приводит к обратному ремоделированию сердца, которое может быть неустойчивым.

Dilated cardiomyopathy (DCM) is the second most common cause of chronic heart failure, and a leading indication for heart transplantation. In the early stages of the disease, many patients are asymptomatic, but they have a high risk of developing life-threatening ventricular arrhythmias (VA) and sudden cardiac death (SCD). Modern methods of DCM diagnosis, such as MRI of the heart, speckle tracking and 3D echocardiography, have convincingly proved the development of structural and functional changes of the right heart (RH) in patients with DCM, their prognostic and therapeutic significance. By MRI of the heart, myocardial fibrosis (MF) is diagnosed, which occurs in the early stages of DCM. Progressive fibrosis is observed in one fifth of patients and is associated with a more than a 3-fold increase in the risk of death and complications of chronic heart failure (CHF). With adequate pharmacological and hardware treatment, reverse heart remodeling occurs in some patients. However, optimal and long-term drug therapy does not lead to MF regression even in individuals with improved left ventricular (LV) function. Remodeling of the RH, regardless of LV ejection fraction (EF), is a predictor of SCD, indicates the progression of the disease, foreshadowing an unfavorable outcome. The prevalence of RH remodeling has not been reliably determined; however, systolic dysfunction of the right ventricle was registered in 34-65% of patients with DCM. The annual and subsequent mortality of patients with DCM remains high.MRI of the heart with contrast enhancement has become the gold standard for the diagnosis of DCM and crucial for the stratification of risk and prognosis of the disease. The treatment of patients with DCM is a complex process. Adequate drug and instrumental therapy in 40% of cases leads to reverse remodeling of the heart, which may be unstable.

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