Expert Center for cardiac amyloidosis: reality and perspectives
- 作者: Nasonova S.1, Zhirov I.1, Shoshina A.1, Osmolovskaya Y.1, Ansheles A.1, Tchaikovskaya O.1, Dobrovolskaya S.1, Sergienko V.1, Saidova M.1, Tereshchenko S.1, Boytsov S.1
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隶属关系:
- Chazov National Medical Research Center of Cardiology
- 期: 卷 96, 编号 4 (2024)
- 页面: 321-329
- 栏目: Editorial
- URL: https://journals.rcsi.science/0040-3660/article/view/255108
- DOI: https://doi.org/10.26442/00403660.2024.04.202677
- ID: 255108
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Aim. To evaluate the features of diagnosis of amyloid cardiomyopathy (ACMP), differential diagnosis of different types of amyloidosis and its clinical manifestations.
Materials and methods. Were analyzed 150 cases of patients who consulted at the Expert Center for Amyloidosis with suspicion of the presence of ACMP. 63 patients were diagnosed with ACMP: 25 (39.7%) – women, 38 (60.3%) – men, with an average age of 64.1±1.5. 36 (57.1%) patients had AL-amyloidosis (immunoglobulin amyloid light-chain amyloidosis), 25 (39.7%) – ATTR-amyloidosis (transthyretin amyloidosis), 2 (3.2%) – AA-amyloidosis with heart failure (reactive systemic amyloidosis caused by hypersecretion of á-globulin). The analysis of clinical manifestations depending on the type of amyloidosis, data of laboratory and instrumental methods of diagnosis is carried out.
Results. In most cases, 53 (84.1%) patients, amyloidosis manifested as signs of heart failure. Among cardiac manifestations, shortness of breath (95.2%), general weakness (93.7%), lower limb edema (76.2%) were the most common. To confirm the diagnosis, despite the high accuracy of the speckle-tracking echocardiography and magnetic resonance imaging of the heart with gadolinium, in rare cases a biopsy is required (e.g. there is a combination of clinical signs of several types of amyloidosis). Biopsy of the affected organ was performed in 31 (49.2%) patients. The strategy for further pathogenetic treatment depends on the determination of the type of amyloidosis. Free light chains of immunoglobulins were detected in 57.1% of cases, which allowed diagnosis of AL-amyloidosis. In 17 (38.6%) patients myocardial scintigraphy with 99mTc-pyrophosphate showed signs of ATTR-amyloidosis, which with a negative result of immunochemical studies allows non-invasive diagnosis of it.
Conclusion. ACMP is a disease with an extremely adverse prognosis. Raising the awareness of specialists about ACMP is an important goal. With timely diagnosis, pathogenetic therapy can be started early, which will improve the quality of life and prognosis of patients with ACMP.
作者简介
Svetlana Nasonova
Chazov National Medical Research Center of Cardiology
Email: izhirov@mail.ru
ORCID iD: 0000-0002-0920-7417
канд. мед. наук, ст. науч. сотр. отд. заболеваний миокарда и сердечной недостаточности
俄罗斯联邦, MoscowIgor Zhirov
Chazov National Medical Research Center of Cardiology
编辑信件的主要联系方式.
Email: izhirov@mail.ru
ORCID iD: 0000-0002-4066-2661
д-р мед. наук, вед. науч. сотр. отд. заболеваний миокарда и сердечной недостаточности
俄罗斯联邦, MoscowAnastasiya Shoshina
Chazov National Medical Research Center of Cardiology
Email: izhirov@mail.ru
ORCID iD: 0000-0002-9519-7373
аспирант отд. заболеваний миокарда и сердечной недостаточности
俄罗斯联邦, MoscowYulia Osmolovskaya
Chazov National Medical Research Center of Cardiology
Email: izhirov@mail.ru
ORCID iD: 0000-0002-7827-2618
канд. мед. наук, науч. сотр. отд. заболеваний миокарда и сердечной недостаточности
俄罗斯联邦, MoscowAlexey Ansheles
Chazov National Medical Research Center of Cardiology
Email: izhirov@mail.ru
ORCID iD: 0000-0002-2675-3276
д-р мед. наук, вед. науч. сотр. отд. радионуклидной диагностики и позитронно-эмиссионной томографии
俄罗斯联邦, MoscowOlga Tchaikovskaya
Chazov National Medical Research Center of Cardiology
Email: izhirov@mail.ru
ORCID iD: 0000-0002-2251-1675
врач функциональной диагностики отд-ния ультразвуковой диагностики
俄罗斯联邦, MoscowSvetlana Dobrovolskaya
Chazov National Medical Research Center of Cardiology
Email: izhirov@mail.ru
ORCID iD: 0000-0003-0580-393X
канд. мед. наук, мл. науч. сотр. отд. ультразвуковых методов исследования
俄罗斯联邦, MoscowVladimir Sergienko
Chazov National Medical Research Center of Cardiology
Email: izhirov@mail.ru
ORCID iD: 0000-0002-0487-6902
д-р мед. наук, проф., рук. отд. радионуклидной диагностики и позитронно-эмиссионной томографии
俄罗斯联邦, MoscowMarina Saidova
Chazov National Medical Research Center of Cardiology
Email: izhirov@mail.ru
ORCID iD: 0000-0002-3233-1862
д-р мед. наук, проф., рук. отд. ультразвуковых методов исследования
俄罗斯联邦, MoscowSergey Tereshchenko
Chazov National Medical Research Center of Cardiology
Email: izhirov@mail.ru
ORCID iD: 0000-0001-9234-6129
д-р мед. наук, проф., рук. отд. заболеваний миокарда и сердечной недостаточности
俄罗斯联邦, MoscowSergey Boytsov
Chazov National Medical Research Center of Cardiology
Email: izhirov@mail.ru
ORCID iD: 0000-0001-6998-8406
акад. РАН, д-р мед. наук, проф., ген. дир.
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