Амилоидная кардиопатия: патоморфология, методы диагностики и лечения
Амилоидная кардиопатия: патоморфология, методы диагностики и лечения
Рамеева А.С., Рамеев В.В., Моисеев С.В. и др. Амилоидная кардиопатия: патоморфология, методы диагностики и лечения. Consilium Medicum. 2018; 20 (12): 15–22. DOI: 10.26442/20751753.2018.12.000020
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Rameeva A.S., Rameev V.V., Moiseev S.V. et al. Amyloid heart disease: pathomorphology, diagnostic approaches and treatment options. Consilium Medicum. 2018; 20 (12): 15–22. DOI: 10.26442/20751753.2018.12.000020
Амилоидная кардиопатия: патоморфология, методы диагностики и лечения
Рамеева А.С., Рамеев В.В., Моисеев С.В. и др. Амилоидная кардиопатия: патоморфология, методы диагностики и лечения. Consilium Medicum. 2018; 20 (12): 15–22. DOI: 10.26442/20751753.2018.12.000020
________________________________________________
Rameeva A.S., Rameev V.V., Moiseev S.V. et al. Amyloid heart disease: pathomorphology, diagnostic approaches and treatment options. Consilium Medicum. 2018; 20 (12): 15–22. DOI: 10.26442/20751753.2018.12.000020
Системный амилоидоз относится к группе редких системных заболеваний и характеризуется накоплением нерастворимых фибриллярных белков в различных органах и тканях. Амилоидные депозиты формируются из различных белков-предшественников, которые определяют тип амилоидоза, характер органного вовлечения и прогноз. Поражение сердца при системном амилоидозе в большинстве случаев является наиболее значимым фактором, определяющим клиническое течение и исход, особенно при AL- и ATTR-амилоидозе. В последнее десятилетие достигнут очевидный успех в понимании амилоидоза в целом и амилоидоза сердца в частности. В представленном обзоре внимание акцентировано на изменениях внутрисердечной гемодинамики при амилоидной кардиопатии, современных высокоинформативных методах диагностики этих изменений с использованием тканевой допплерометрии, strain- и speckle tracking-эхокардиографии, магнитно-резонансной томографии, определением сердечных биомаркеров. В статье рассмотрены новые методы типирования амилоида и современные схемы лечения амилоидной кардиопатии.
Systemic amyloidosis is a relatively rare multisystem disease caused by the deposition of misfolded protein in various tissues and organs. The deposits are derived from several amyloidogenic precursor proteins, and the prognosis of the disease is determined both by the organ(s) involved and the type of amyloid. Cardiac involvement is a leading cause of morbidity and mortality, especially in AL- and ATTR-amyloidosis. The last decade has seen considerable progress in understanding of cardiac amyloidosis. This review focuses on changes in intracardiac hemodynamics and their evaluation using novel diagnostic approaches (strain and speckle tracking imaging, cardiovascular magnetic resonance and cardiac biomarkers). New histological typing techniques and current treatments are discussed.
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1. Kozlovskaia L.V., Rameev V.V. AA- i AL-amiloidoz. Nefrologiia. Klinicheskie rekomendatsii. Pod red. E.M.Shilova, A.V.Smirnova, N.L.Kozlovskoi. M.: GEOTAR-Media, 2016: 299–319. [in Russian]
2. Sipe J, Benson M, Buxbaum J et al. Amyloid fibril proteins and amyloidosis: chemical identification and clinical classification International Society of Amyloidosis 2016 Nomenclature Guidelines. Amyloid 2016; 23 (4): 209–13.
3. Steiner I. The prevalence of isolated atrial amyloid. J Pathol 1987; 153 (4): 395–8.
4. Zhdanova E.A., Rameev V.V., Moiseev S.V. i dr. Amiloidoz serdtsa: klinika, lechenie, prognoz. Farmateka. 2012; 9: 10–6. [in Russian]
5. Kyle RA, Linos A, Beard CM et al. Incidence and natural history of primary systemic amyloidosis in Olmsted County, Minnesota, 1950 through 1989. Blood 1992; 79: 1817–22.
6. Pinney JH, Smith CJ, Taube JB et al. Systemic amyloidosis in England: an epidemiological study. Br J Haematol 2013; 161: 525–32.
7. Hemminki K, Li X, Försti A et al. Incidence and survival in nonhereditary amyloidosis in Sweden. BMC Public Health 2012; 12: 974.
8. Hemminki K, Li X, Försti A et al. Incidence of hereditary amyloidosis and autoinflammatory diseases in Sweden: endemic and imported diseases. BMC Med Genet 2013; 14: 88.
9. Rameev V.V., Kozlovskaia L.V. Amiloidoz: sovremennye metody diagnostiki i lecheniia. Effektivnaia farmakoterapiia. Urologiia i nefrologiia. Spetsvyp.: Aktual'nye voprosy nefrologii. 2012; s. 6–15. [in Russian]
10. Kyle R, Gertz M. Primary systemic amyloidosis: clinical and laboratory features in 474 cases. Semin Hematol 1995; 32 (1): 45–59.
11. Patel KS, Hawkins PN. Cardiac amyloidosis: where are we today? J Intern Med 2015; 278 (2): 126–44.
12. Rameev V.V. Sovremennye metody diagnostiki i lecheniia transtiretinovogo nasledstvennogo amiloidoza. Manage pain. 2018; 1: 20–4. [in Russian]
13. Zinov'eva O.E., Safiulina E.I. Transtiretinovaia amiloidnaia polineiropatiia: patogenez, klinicheskie osobennosti, perspektivy lecheniia. Manage pain. 2017; 4: 12–5. [in Russian]
14. Gertz MA, Merlini G. Definition of organ involvement and response to treatment in AL amyloidosis: an updated consensus opinion. Amyloid 2010; 17 (Suppl. 1): 48–9.
15. Falk R, Alexander K, Liao R, Dorbala S. AL (Light-Chain) Cardiac Amyloidosis: A Review of Diagnosis and Therapy. J Am Coll Cardiol 2016; 68 (12): 1323–41.
16. Rapezzi C, Merlini G, Quarta CC et al. Systemic cardiac amyloidoses: disease profiles and clinical courses of the 3 main types. Circulation 2009; 120: 1203–12.
17. Dubrey SW, Cha K, Anderson J et al. The clinical features of immunoglobulin light-chain (AL) amyloidosis with heart involvement. QJM 1998; 91: 141–57.
18. Larsen BT, Mereuta OM, Dasari S et al. Correlation of histomorphological pattern of cardiac amyloid deposition with amyloid type: a histological and proteomic analysis of 108 cases. Histopathology 2016; 68: 648–56.
19. Shi J, Guan J, Jiang B et al. Amyloidogenic light chains induce cardiomyocyte contractile dysfunction and apoptosis via a noncanonical p38 MAPK pathway. PNAS 2010; 107: 4188–93.
20. Alekhin M.N. Tkanevoi doppler v klinicheskoi ekhokardiografii. M., 2006. [in Russian]
21. Koyama J, Ray-Sequin PA, Falk RH. Longitudinal myocardial function assessed by tissue velocity, strain, and strain rate tissue Doppler echocardiography in patients with AL (primary) cardiac amyloidosis. Circulation 2003; 107: 2446–52.
22. Bellavia D, Pellikka PA, Abraham TP et al. Evidence of impaired left ventricular systolic function by Doppler myocardial imaging in patients with systemic amyloidosis and no evidence of cardiac involvement by standard twodimensional and Doppler echocardiography. Am J Cardiol 2008; 101: 1039–45.
23. Belkin RN, Kupersmith AC, Khalique O et al. A novel two-dimensional echocardiographic finding in cardiac amyloidosis. Echocardiography 2010; 27: 1171–6.
24. Phelan D, Collier P, Thavendiranathan P et al. Relative apical sparing of longitudinal strain using two-dimensional speckle-tracking echocardiography is both sensitive and specific for the diagnosis of cardiac amyloidosis. Heart 2012; 98: 1442–8.
25. Pandey T, Alapati S, Wadhwa V et al. Evaluation of myocardial strain in patients with amyloidosis using cardiac magnetic resonance feature tracking. Curr Probl Diagn Radiol 2017; 46: 288–94.
26. Ternacle J, Bodez D, Guellich A et al. Causes and consequences of longitudinal LV dysfunction assessed by 2D strain echocardiography in cardiac amyloidosis. JACC Cardiovasc Imaging 2016; 9: 126–38.
27. Liu D, Hu K, Nordbeck P et al. Longitudinal strain bull’s eye plot patterns in patients with cardiomyopathy and concentric left ventricular hypertrophy. Eur J Med Res. BioMed Central 2016; 21: 21.
28. Nikiforov V.S., Nikishchenkova Iu.V. Covremennye vozmozhnosti speckle tracking ekhokardiografiii v klinicheskoi praktike. Ratsional'naia farmakoterapiia v kardiologii. 2017; 13 (2): 248–55. [in Russian]
29. Phelan D, Thavendiranathan P, Popovic Z et al. Application of a parametric display of two-dimensional speckle-tracking longitudinal strain to improve the etiologic diagnosis of mild to moderate left ventricular hypertrophy. J Am Soc Echocardiog 2014; 27: 888–95.
30. Grossman W, Jones D, McLaurin LP. Wall stress and patterns of hypertrophy in the human left ventricle. J Clin Invest 1975; 56: 56–64.
31. Jiang L, Huang Y, Hunyor S, dos Remedios CG. Cardiomyocyte apoptosis is associated with increased wall stress in chronic failing left ventricle. Eur Heart J 2003; 24: 742–51.
32. Buchalter MB, Weiss JL, Rogers WJ et al. Noninvasive quantification of left ventricular rotation deformation in normal human using magnetic resonance imaging myocardial tagging. Circulation 1990; 81: 1236–44.
33. Torrent-Guasp F. Structure and function of the heart. Rev Esp Cardio 1998; 51: 91–102.
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1 ФГАОУ ВО «Первый Московский государственный медицинский университет им. И.М.Сеченова» Минздрава России. 119991, Россия, Москва, ул. Трубецкая, д. 8, стр. 2;
2 ФГБОУ ВО «Московский государственный университет им. М.В.Ломоносова». 119991, Россия, Москва, Ленинские горы, д. 1
*vvrameev@mail.ru
1 I.M.Sechenov First Moscow State Medical University of the Ministry of Health of the Russian Federation. 119991, Russian Federation, Moscow, ul. Trubetskaia, d. 8, str. 2;
2 M.V.Lomonosov Moscow State University. 119991, Russian Federation, Moscow, Leninskiye gory, d. 1