Modern Aspects of Diagnosis and Treatment of Dilated Cardiomyopathy With Remodeling of the Right Heart
https://doi.org/10.23934/2223-9022-2025-14-2-387-397
Abstract
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.
Keywords
About the Authors
M. Sh. KhubutiyaRussian Federation
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
Kh. G. Alidzhanova
Russian Federation
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
Zh. V. Molchanova
Russian Federation
Zhanna V. Molchanova - Cardiology Resident, N.V. Sklifosovsky Research Institute for Emergency Medicine.
Bolshaya Sukharevskaya Sq. 3, Moscow, 129090
M. A. Sagirov
Russian Federation
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
E. V. Ilyina
Russian Federation
Evgeniya V. Ilyina - Cardiology Resident, N.V. Sklifosovsky Research Institute for Emergency Medicine.
Bolshaya Sukharevskaya Sq. 3, Moscow, 129090
References
1. Sinagra G, Elliott PM, Merlo M. Dilated cardiomyopathy: so many cardiomyopathies! Eur Heart J. 2020;41(39):3784–3786. PMID: 31872205 https://doi.org/10.1093/eurheartj/ehz908
2. Schultheiss HP, Fairweather D, Caforio ALP, Escher F, Hershberger RE, Lipshultz SE, et al. Dilated cardiomyopathy. Nat Rev Dis Primers. 2019;5(1):32. PMID: 31073128 https://doi.org/10.1038/s41572-019-0084-1
3. Shumakov VI, Khubutiya MSh, Il’inskiy IM. Dilatatsionnaya kardiomiopatiya. Moscow: Triada Publ.; 2003. (In Russ.)
4. Ferreira A, Ferreira V, Antunes MM, Lousinha A, Pereira-da-Silva T, Antunes D, et al. Dilated Cardiomyopathy: A Comprehensive Approach to Diagnosis and Risk Stratification. Biomedicines. 2023;11(3):834. https://doi.org/10.3390/biomedicines11030834
5. Orphanou N, Papatheodorou E, Anastasakis A. Dilated cardiomyopathy in the era of precision medicine: latest concepts and developments. Heart Fail Rev. 2022;27(4):1173–1191. PMID: 34263412 https://doi.org/10.1007/s10741-021-10139-0
6. Hammersley DJ, Jones RE, Owen R, Mach L, Lota AS, Khalique Z, et al. Phenotype, outcomes and natural history of early-stage non-ischaemic cardiomyopathy. Eur J Heart Fail. 2023;25(11):2050–2059. PMID: 37728026 https://doi.org/10.1002/ejhf.3037
7. Golukhova EZ, Aleksandrova SA, Berdibekov BSh. Predictive role of quantification of myocardial fibrosis using delayed contrast-enhanced magnetic resonance imaging in nonischemic dilated cardiomyopathies: a systematic review and meta-analysis. Russian Journal of Cardiology. 2021;26(12):4776. (In Russ.) https://doi.org/10.15829/1560-4071-2021-4776
8. Weintraub RG, Semsarian C, Macdonald P. Dilated cardiomyopathy. Lancet. 2017;390(10092):400–414. PMID: 28190577 https://doi.org/10.1016/S0140-6736(16)31713-5
9. Xu XR, Han MM, Yang YZ, Wang X, Hou DY, Meng XC, et al. Fifteen-year mortality and prognostic factors in patients with dilated cardiomyopathy: persistent standardized application of drug therapy and strengthened management may bring about encouraging change in an aging society. J Geriatr Cardiol. 2022;19(5):335–342. PMID: 35722031 https://doi.org/10.11909/j.issn.1671-5411.2022.05.003
10. Merlo M, Stolfo D, Anzini M, Negri F, Pinamonti B, Barbati G, et al. Persistent recovery of normal left ventricular function and dimension in idiopathic dilated cardiomyopathy during long-term follow-up: does real healing exist? J Am Heart Assoc. 2015;4(1):e001504. PMID: 25587018 https://doi.org/10.1161/JAHA.114.000570
11. Leeper B. Right Ventricular Failure. AACN Adv Crit Care. 2020;31(1):49–56. PMID: 32168515 https://doi.org/10.4037/aacnacc2020172
12. Packer M. What causes sudden death in patients with chronic heart failure and a reduced ejection fraction? Eur Heart J. 2020;41(18):1757–1763. https://doi.org/10.1093/eurheartj/ehz553
13. Iovănescu ML, Florescu DR, Marcu AS, Donoiu I, Militaru S, Florescu C, et al. The Dysfunctional Right Ventricle in Dilated Cardiomyopathies: Looking from the Right Point of View. J Cardiovasc Dev Dis. 2022;9(10):359. PMID: 36286311 https://doi.org/10.3390/jcdd9100359
14. Becker MAJ, van der Lingen ALCJ, Wubben M, van de Ven PM, van Rossum AC, Cornel JH, et al. Characteristics and prognostic value of right ventricular (DYS)function in patients with non-ischaemic dilated cardiomyopathy assessed with cardiac magnetic resonance imaging. ESC Heart Fail. 2021;8(2):1055–1063. PMID: 33560582https://doi.org/10.1002/ehf2/13072
15. Vîjîiac A, Onciul S, Guzu C, Verinceanu V, Bătăilă V, Deaconu S, et al. The prognostic value of right ventricular longitudinal strain and 3D ejection fraction in patients with dilated cardiomyopathy. Int J Cardiovasc Imaging. 2021;37(11):3233–3244. PMID: 34165699 https://doi.org/10.1007/s10554-021-02322-z
16. Venner C, Selton-Suty C, Huttin O, Erpelding ML, Aliot E, Juillière Y. Right ventricular dysfunction in patients with idiopathic dilated cardiomyopathy: Prognostic value and predictive factors. Arch Cardiovasc Dis. 2016;109(4):231–241. PMID: 26782624 https://doi.org/10.1016/j.acvd.2015.10.006
17. Pathak RK, Sanders P, Deo R. Primary prevention implantable cardioverter-defibrillator and opportunities for sudden cardiac death risk assessment in non-ischaemic cardiomyopathy. Eur Heart J. 2018;39(31):2859–2866. PMID: 30020440 https://doi.org/10.1093/eurheartj/ehy344
18. Li Y., Guo J, Li W, Xu Y, Wan K, Xu Z, et al. Prognostic value of right atrial strain derived from cardiovascular magnetic resonance in non-ischemic dilated cardiomyopathy. J Cardiovasc Magn Reson. 2022;24(1):54. PMID: 36352424 https://doi.org/10.1186/s12968-022-00894-w
19. Sallach JA, Tang WH, Borowski AG, Tong W, Porter T, Martin MG, et al. Right Atrial Volume Index in Chronic Systolic Heart Failure and Prognosis. JACC Cardiovasc Imaging. 2009;2(5):527–534. PMID: 19442936 https://doi.org/10.1016/j.jcmg.2009.01.012
20. Marrow BA, Cook SA, Prasad SK, McCann GP. Emerging Techniques for Risk Stratification in Nonischemic Dilated Cardiomyopathy: JACC Review Topic of the Week. J Am Coll Cardiol. 2020;75(10):1196–1207. PMID: 32164893 https://doi.org/10.1016/j.jacc.2019.12.058
21. Boulet J, Mehra MR. Left Ventricular Reverse Remodeling in Heart Failure: Remission to Recovery. Struct Heart. 2021;5(5):466–481. https://doi.org/10.1080/24748706.2021.1954275
22. Mages C, Gampp H, Syren P, Rahm AK, André F, Frey N, et al. Electrical Ventricular Remodeling in Dilated Cardiomyopathy. Cells. 2021;10(10):2767. PMID: 34685747 https://doi.org/10.3390/cells10102767
23. Mazur VV, Kalinkin AM, Mazur ES. Heart Remodeling at Different Stages of Chronic Heart Failure in Patients With Postinfarction Cardiosclerosis and Dilated Cardiomyopathy. Rational Pharmacotherapy in Cardiology. 2010;6(6):818–822. (In Russ.). https://doi.org/10.20996/1819-6446-2010-6-6-818-822
24. Stecker EC, Vickers C, Waltz J, Socoteanu C, John BT, Mariani R, et al. Population-based analysis of sudden cardiac death with and without left ventricular systolic dysfunction: two-year findings from the Oregon Sudden Unexpected Death Study. J Am Coll Cardiol. 2006;47(6):1161–1166. PMID: 16545646 https://doi.org/10.1016/j.jacc.2005.11.045
25. Mikami Y, Jolly U, Heydari B, Peng M, Almehmadi F, Zahrani M, et al. Right Ventricular Ejection Fraction Is Incremental to Left Ventricular Ejection Fraction for the Prediction of Future Arrhythmic Events in Patients With Systolic Dysfunction. Circ Arrhythm Electrophysiol. 2017;10(1): e004067. PMID: 28087564 https://doi.org/10.1161/CIRCEP.116.004067
26. Gulati A, Ismail TF, Jabbour A, Alpendurada F, Guha K, Ismail NA, et al. The prevalence and prognostic significance of right ventricular systolic dysfunction in nonischemic dilated cardiomyopathy. Circulation. 2013;128(15):1623–1633. PMID: 23965488 https://doi.org/10.1161/CIRCULATIONAHA.113.002518
27. Pueschner A, Chattranukulchai P, Heitner JF, Shah DJ, Hayes B, Rehwald W, et al. The Prevalence, Correlates, and Impact on Cardiac Mortality of Right Ventricular Dysfunction in Nonischemic Cardiomyopathy. JACC Cardiovasc Imaging. 2017;10(10 Pt B):1225–1236. PMID: 29025576 https://doi.org/10.1016/j.jcmg.2017.06.013
28. Elming MB, Hammer-Hansen S, Voges I, Nyktari E, Raja AA, Svendsen JH, et al. Right Ventricular Dysfunction and the Effect of Defibrillator Implantation in Patients With Nonischemic Systolic Heart Failure. Circ Arrhythm Electrophysiol. 2019;12(3):e007022. PMID: 30866666 https://doi.org/10.1161/CIRCEP.118.007022
29. Jimenez-Juan L, Ben-Dov N, Goncalves Frazao CV, Tan NS, Singh SM, Dorian P, et al. Right Ventricular Function at Cardiac MRI Predicts Cardiovascular Events in Patients with an Implantable Cardioverter-Defibrillator. Radiology. 2021;301(2):322–329. PMID: 34402663 https://doi.org/10.1148/radiol.2021210246
30. Di Marco A, Brown PF, Bradley J, Nucifora G, Claver E, de Frutos F, et al. Improved Risk Stratification for Ventricular Arrhythmias and Sudden Death in Patients With Nonischemic Dilated Cardiomyopathy. J Am Coll Cardiol. 2021;77(23):2890–2905. PMID: 34112317 https://doi.org/10.1016/j.jacc.2021.04.030
31. Alba AC, Gaztañaga J, Foroutan F, Thavendiranathan P, Merlo M, Alonso-Rodriguez D, et al. Prognostic Value of Late Gadolinium Enhancement for the Prediction of Cardiovascular Outcomes in Dilated Cardiomyopathy: An International, Multi-Institutional Study of the MINICOR Group. Circ Cardiovasc Imaging. 2020;13(4):e010105. PMID: 32312112 https://doi.org/10.1161/CIRCIMAGING.119.010105
32. Mandawat A, Chattranukulchai P, Mandawat A, Blood AJ, Ambati S, Hayes B, et al. Progression of Myocardial Fibrosis in Nonischemic DCM and Association With Mortality and Heart Failure Outcomes. JACC Cardiovasc Imaging. 2021;14(7):1338–1350. PMID: 33454264 https://doi.org/10.1016/j.jcmg.2020.11.006
33. Perone F, Dentamaro I, La Mura L, Alifragki A, Marketou M, Cavarretta E, et al. Current Insights and Novel Cardiovascular Magnetic Resonance-Based Techniques in the Prognosis of Non-Ischemic Dilated Cardiomyopathy. J Clin Med. 2024;13(4):1017. PMID: 38398330 https://doi.org/10.3390/jcm13041017
34. Tang HS, Kwan CT, He J, Ng PP, Hai SHJ, Kwok FYJ, et al. Prognostic Utility of Cardiac MRI Myocardial Strain Parameters in Patients With Ischemic and Nonischemic Dilated Cardiomyopathy: A Multicenter Study. AJR Am J Roentgenol. 2023;220(4):524–538. PMID: 36321987 https://doi.org/10.2214/AJR.22.28415
35. D’Andrea A, Scarafile R, Riegler L, Salerno G, Gravino R, Cocchia R, et al. Right atrial size and deformation in patients with dilated cardiomyopathy undergoing cardiac resynchronization therapy. Eur J Heart Fail. 2009;11(12):1169–1177. PMID: 19926601 https://doi.org/10.1093/eurjhf/hfp158
36. Venner C, Selton-Suty C, Huttin O, Erpelding ML, Aliot E, Juillière Y. Right ventricular dysfunction in patients with idiopathic dilated cardiomyopathy: Prognostic value and predictive factors. Arch Cardiovasc Dis. 2016;109(4):231–241. PMID: 26782624 https://doi.org/10.1016/j.acvd.2015.10.006
37. Liu T, Gao Y, Wang H, Zhou Z, Wang R, Chang S-S, et al. Association between right ventricular strain and outcomes in patients with dilated cardiomyopathy. Heart. 2021;107(15):1233–1239. PMID: 33139324 https://doi.org/10.1136/heartjnl-2020-317949
38. Liu S, Li Y, Lian J, Wang X, Li Y, Wang D, et al. Prognostic Significance of Biventricular and Biatrial Strain in Dilated Cardiomyopathy: Strain Analysis Derived from Cardiovascular Magnetic Resonance. Rev Cardiovasc Med. 2023;24(12):347. https://doi.org/10.31083/j.rcm2412347
39. Houard L, Benaets MB, de Meester de Ravenstein C, Rousseau MF, Ahn SA, Amzulescu M-S, et al. Additional Prognostic Value of 2D Right Ventricular Speckle-Tracking Strain for Prediction of Survival in Heart Failure and Reduced Ejection Fraction: A Comparative Study With Cardiac Magnetic Resonance. JACC Cardiovasc Imaging. 2019;12(12):2373–2385. PMID: 30772232 https://doi.org/10.1016/j.jcmg.2018.11.028
40. Muraru D, Badano LP, Nagata Y, Surkova E, Nabeshima Y, Genovese D, et al. Development and prognostic validation of partition values to grade right ventricular dysfunction severity using 3D echocardiography. Eur Heart J Cardiovasc Imaging. 2020;21:10–21. PMID: 31539046 https://doi.org/10.1093/ehjci/jez233
41. Lang RM, Badano LP, Mor-Avi V, Afilalo J, Armstrong A, Ernandeet L, et al. Recommendations for Cardiac Chamber Quantification by Echocardiography in Adults: An Update from the American Society of Echocardiography and the European Association of Cardiovascular Imaging. J Am Soc Echocardiogr. 2015;28(1):1–39.e14. PMID: 25559473 https://doi.org/10.1016/j.echo.2014.10.003
42. Vîjîiac A, Onciul S, Guzu C, Verinceanu V, Bătăilă V, Deaconu S, et al. The prognostic value of right ventricular longitudinal strain and 3D ejection fraction in patients with dilated cardiomyopathy. Int J Cardiovasc Imaging. 2021;37(11):3233–3244. PMID: 34165699 https://doi.org/10.1007/s10554-021-02322-z
43. Kübler J, Burgstahler C, Brendel JM, Gassenmaier S, Hagen F, Klingel K, et al. Cardiac MRI findings to differentiate athlete’s heart from hypertrophic (HCM), arrhythmogenic right ventricular (ARVC) and dilated (DCM) cardiomyopathy. Int J Cardiovasc Imaging. 2021;37(8):2501–2515. PMID: 34019206 https://doi.org/10.1007/s10554-021-02280-6
44. Monzo L, Tupy M, Borlaug BA, Reichenbach A, Jurcova I, Benes J, et al. Pressure overload is associated with right ventricular dyssynchrony in heart failure with reduced ejection fraction. ESC Heart Fail. 2024;11(2):1097–1109. PMID: 38263857 https://doi.org/10.1002/ehf2.14682
45. Golwala H, Bajaj NS, Arora G, Arora P. Implantable cardioverter-defibrillator for nonischemic cardiomyopathy: An updated meta-analysis. Circulation. 2017;135(2):201–203. PMID: 27993908 https://doi.org/10.1161/CIRCULATIONAHA.116.026056
46. O’Keefe JH Jr, Magalski A, Stevens TL, Bresnahan DR Jr, Alaswad K, Krueger SK, et al. Predictors of improvement in left ventricular ejection fraction with carvedilol for congestive heart failure. J Nucl Cardiol. 2000;7(1):3–7. PMID: 10698228 https://doi.org/10.1067/mnc.2000.102678
47. Theerasuwipakorn N, Chokesuwattanaskul R, Phannajit J, Marsukjai A, Thapanasuta M, Klem I, et al. Impact of late gadolinium-enhanced cardiac MRI on arrhythmic and mortality outcomes in nonischemic dilated cardiomyopathy: updated systematic review and meta-analysis. Sci Rep. 2023;13(1):13775. PMID: 37612359 https://doi.org/10.1038/s41598-023-41087-4
48. Tong X, Shen L, Zhou X, Wang Y, Chang S, Lu S. Comparative Efficacy of Different Drugs for the Treatment of Dilated Cardiomyopathy: A Systematic Review and Network Meta-analysis. Drugs RD. 2023;23(3):197–210. PMID: 37556093 https://doi.org/10.1007/s40268-023-00435-5
Review
For citations:
Khubutiya M.Sh., Alidzhanova Kh.G., Molchanova Zh.V., Sagirov M.A., Ilyina E.V. Modern Aspects of Diagnosis and Treatment of Dilated Cardiomyopathy With Remodeling of the Right Heart. Russian Sklifosovsky Journal "Emergency Medical Care". 2025;14(2):387-397. (In Russ.) https://doi.org/10.23934/2223-9022-2025-14-2-387-397