Sudden cardiac death risk stratification in dilated cardiomyopathy: a state of the art review
https://doi.org/10.15829/1560-4071-2025-6114
EDN: BOSXIO
Abstract
Dilated cardiomyopathy is one of the most common types of cardiomyopathy, characterized by left ventricular dilation and systolic dysfunction. The disease is accompanied by a high risk of adverse outcomes, including heart failure decompensation and sudden cardiac death (SCD). Traditionally, only left ventricular ejection fraction ≤35% was used to identify candidates for cardioverter-defibrillator implantation for the primary prevention of SCD. However, with the advances in therapy for heart failure, cardiac resynchronization therapy and modern cardiac imaging techniques such as delayed contrast-enhanced magnetic resonance imaging and genetic tests, SCD risk stratification approaches should be revised. This paper reviews the state of the art on SCD risk stratification in patients with dilated cardiomyopathy. Particular attention is paid to the role of magnetic resonance imaging and genetic studies, which significantly expand the potential of risk assessment and allow more accurate identification of patients requiring cardioverter-defibrillator implantation for primary SCD prevention.
About the Authors
B. Sh. Berdibekov
Bakulev National Medical Research Center for Cardiovascular Surgery
Russian Federation
Competing Interests:
Russian Federation
Moscow
Competing Interests:
none
N. I. Bulaeva
Bakulev National Medical Research Center for Cardiovascular Surgery
Russian Federation
Competing Interests:
Russian Federation
Moscow
Competing Interests:
none
S. A. Alexandrova
Bakulev National Medical Research Center for Cardiovascular Surgery
Russian Federation
Competing Interests:
Russian Federation
Moscow
Competing Interests:
none
E. Z. Golukhova
Bakulev National Medical Research Center for Cardiovascular Surgery
Russian Federation
Competing Interests:
Russian Federation
Moscow
Competing Interests:
none
References
1. Arbelo E, Protonotarios A, Gimeno JR, et al. 2023 ESC Guidelines for the management of cardiomyopathies. Eur Heart J. 2023;44(37):3503-626. doi:10.1093/eurheartj/ehad194.
2. Reichart D, Magnussen C, Zeller T, Blankenberg S. Dilated cardiomyopathy: from epidemiologic to genetic phenotypes: A translational review of current literature. J Intern Med. 2019;286(4):362-72. doi:10.1111/joim.12944.
3. Goldberger JJ. Sudden cardiac death risk stratification in dilated cardiomyopathy: climbing the pyramid of knowledge. Circ Arrhythm Electrophysiol. 2014;7(6):1006-8. doi:10.1161/CIRCEP.114.002395.
4. Bokeria LA, Neminushchiy NM, Postol AS. Implantable Cardioverter-Defibrillators are the Main Link in the Modern Concept of Sudden Cardiac Death Prevention. Problems and Prospects of the Development of the Method. Kardiologiia. 2018;58(12):76-84. (In Russ.) doi:10.18087/cardio.2018.12.10197.
5. Priori SG, Blomström-Lundqvist C, Mazzanti A, et al. 2015 ESC Guidelines for the management of patients with ventricular arrhythmias and the prevention of sudden cardiac death: The Task Force for the Management of Patients with Ventricular Arrhythmias and the Prevention of Sudden Cardiac Death of the European Society of Cardiology (ESC). Endorsed by: Association for European Paediatric and Congenital Cardiology (AEPC). Eur Heart J. 2015;36(41):2793-867. doi:10.1093/eurheartj/ehv316.
6. Jia SQ, Lv SY, Jin YH, et al. Cardiac MRI for clinical dilated cardiomyopathy: Improved diagnostic power via combined T1, T2, and ECV. Radiography (Lond). 2024;30(3):926-31. doi:10.1016/j.radi.2024.04.005.
7. Vaykhanskaya TG, Sivitskaya LN, Kurushko TV, et al. Dilated cardiomyopathy: reconceptualization of the problem. Russian Journal of Cardiology. 2019;(4):35-47. (In Russ.) doi:10.15829/1560-4071-2019-4-35-47.
8. Amin RJ, Morris-Rosendahl D, Edwards M, et al. The addition of genetic testing and cardiovascular magnetic resonance to routine clinical data for stratification of etiology in dilated cardiomyopathy. Front Cardiovasc Med. 2022;9:1017119. doi:10.3389/fcvm.2022.1017119.
9. Rodionova YuV, Kim OT. What is a State of the Art (SotA) review? Cardiovascular Therapy and Prevention. 2024;23(4S):3946. (In Russ.) doi:10.15829/1728-8800-2024-3946.
10. Wyse DG, Friedman PL, Epstein AE. A Comparison of Antiarrhythmic-Drug Therapy with Implantable Defibrillators in Patients Resuscitated from Near-Fatal Ventricular Arrhythmias. N Engl J Med. 1997;337:1576-84. doi:10.1056/NEJM199711273372202.
11. Connolly SJ, Gent M, Roberts RS, et al. Canadian implantable defibrillator study (CIDS): a randomized trial of the implantable cardioverter defibrillator against amiodarone. Circulation. 2000;101(11):1297-302. doi:10.1161/01.cir.101.11.1297.
12. Kuck KH, Cappato R, Siebels J, Rüppel R. Randomized comparison of antiarrhythmic drug therapy with implantable defibrillators in patients resuscitated from cardiac arrest: the Cardiac Arrest Study Hamburg (CASH). Circulation. 2000;102(7):748-54. doi:10.1161/01.cir.102.7.748.
13. Zeppenfeld K, Tfelt-Hansen J, de Riva M, et al. 2022 ESC Guidelines for the management of patients with ventricular arrhythmias and the prevention of sudden cardiac death. Eur Heart J. 2022;43(40):3997-4126. doi:10.1093/eurheartj/ehac262.
14. Moss AJ, Hall WJ, Cannom DS, et al. Improved survival with an implanted defibrillator in patients with coronary disease at high risk for ventricular arrhythmia. Multicenter Automatic Defibrillator Implantation Trial Investigators. N Engl J Med. 1996;335(26): 1933-40. doi:10.1056/NEJM199612263352601.
15. Buxton AE, Fisher JD, Josephson ME, et al. Prevention of sudden death in patients with coronary artery disease: the Multicenter Unsustained Tachycardia Trial (MUSTT). Prog Cardiovasc Dis. 1993;36(3):215-26. doi:10.1016/0033-0620(93)90015-6.
16. Kadish A, Dyer A, Daubert JP, et al. Defibrillators in Non-Ischemic Cardiomyopathy Treatment Evaluation (DEFINITE) Investigators. Prophylactic defibrillator implantation in patients with nonischemic dilated cardiomyopathy. N Engl J Med. 2004;350(21):2151-8. doi:10.1056/NEJMoa033088.
17. Moss AJ, Zareba W, Hall WJ, et al. Multicenter Automatic Defibrillator Implantation Trial II Investigators. Prophylactic implantation of a defibrillator in patients with myocardial infarction and reduced ejection fraction. N Engl J Med. 2002;346(12):877-83. doi:10.1056/NEJMoa013474.
18. Bardy GH, Lee KL, Mark DB, et al. Sudden Cardiac Death in Heart Failure Trial (SCD-HeFT) Investigators. Amiodarone or an implantable cardioverter-defibrillator for congestive heart failure. N Engl J Med. 2005;352(3):225-37. doi:10.1056/NEJMoa043399.
19. Moss AJ. MADIT-I and MADIT-II. J Cardiovasc Electrophysiol. 2003;14(9 Suppl):S96-8. doi:10.1046/j.1540-8167.14.s9.5.x.
20. Strickberger SA, Hummel JD, Bartlett TG, et al. Amiodarone versus implantable cardioverter-defibrillator:randomized trial in patients with nonischemic dilated cardiomyopathy and asymptomatic nonsustained ventricular tachycardia—AMIOVIRT. J Am Coll Cardiol. 2003;41(10):1707-12. doi:10.1016/s0735-1097(03)00297-3.
21. Bänsch D, Antz M, Boczor S, et al. Primary prevention of sudden cardiac death in idiopathic dilated cardiomyopathy: the Cardiomyopathy Trial (CAT). Circulation. 2002;105(12): 1453-8. doi:10.1161/01.cir.0000012350.99718.ad.
22. Sabbag A, Suleiman M, Laish-Farkash A, et al. Contemporary rates of appropriate shock therapy in patients who receive implantable device therapy in a real-world setting: From the Israeli ICD Registry. Heart Rhythm. 2015;12(12):2426-33. doi:10.1016/j.hrthm.2015.08.020.
23. Lebedev DS, Mikhailov EN, Neminuschiy NM, et al. Ventricular arrhythmias. Ventricular tachycardias and sudden cardiac death. 2020 Clinical guidelines. Russian Journal of Cardiology. 2021;26(7):4600. (In Russ.) doi:10.15829/1560-4071-2021-4600.
24. Stecker EC, Vickers C, Waltz J, 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):1161-6. doi:10.1016/j.jacc.2005.11.045.
25. Køber L, Thune JJ, Nielsen JC, et al. Defibrillator Implantation in Patients with Nonischemic Systolic Heart Failure. N Engl J Med. 2016;375(13):1221-30. doi:10.1056/NEJMoa1608029.
26. McMurray JJ. The ICD in Heart Failure — Time for a Rethink? N Engl J Med. 2016; 375(13):1283-4. doi:10.1056/NEJMe1609826.
27. Ponikowski P, Voors AA, Anker SD, et al. 2016 ESC Guidelines for the Diagnosis and Treatment of Acute and Chronic Heart Failure. Rev Esp Cardiol (Engl Ed). 2016; 69(12):1167. English, Spanish. doi:10.1016/j.rec.2016.11.005.
28. Al-Khatib SM, Stevenson WG, Ackerman MJ, et al. 2017 AHA/ACC/HRS Guideline for Management of Patients With Ventricular Arrhythmias and the Prevention of Sudden Cardiac Death: Executive Summary: A Report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines and the Heart Rhythm Society. Circulation. 2018;138(13):e210-e271. doi:10.1161/CIR.0000000000000548.
29. McDonagh TA, Metra M, Adamo M, et al. 2021 ESC Guidelines for the diagnosis and treatment of acute and chronic heart failure. Eur Heart J. 2021;42(36):3599-726. doi:10.1093/eurheartj/ehab368.
30. Aquaro GD, De Gori C, Faggioni L, et al. Diagnostic and prognostic role of late gadolinium enhancement in cardiomyopathies. Eur Heart J Suppl. 2023;25(Suppl C):C130-C136. doi:10.1093/eurheartjsupp/suad015.
31. Yang W, Xu J, Zhu L, et al. Myocardial Strain Measurements Derived From MR Feature-Tracking: Influence of Sex, Age, Field Strength, and Vendor. JACC Cardiovasc Imaging. 2024;17(4):364-79. doi:10.1016/j.jcmg.2023.05.019.
32. Cojan-Minzat BO, Zlibut A, Agoston-Coldea L. Non-ischemic dilated cardiomyopathy and cardiac fibrosis. Heart Fail Rev. 2021;26(5):1081-101. doi:10.1007/s10741-020-09940-0.
33. Shulzhenko LV, Pershukov IV, Batyraliev TA, et al. The Clinical Evolution of Diffuse Myocardial Fibrosis in Patients With Arterial Hypertension and Heart Failure With Mildly Reduced Ejection Fraction Treated by Olmesartan or Sacubitril / Valsartan. Kardiologiia. 2023;63(12):31-8. (In Russ.) doi:10.18087/cardio.2023.12.n2557.
34. Gulati A, Jabbour A, Ismail TF, et al. Association of fibrosis with mortality and sudden cardiac death in patients with nonischemic dilated cardiomyopathy. JAMA. 2013;309(9): 896-908. doi:10.1001/jama.2013.1363.
35. Theerasuwipakorn N, Chokesuwattanaskul R, Phannajit J, 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. doi:10.1038/s41598-023-41087-4.
36. Halliday BP, Baksi AJ, Gulati A, et al. Outcome in Dilated Cardiomyopathy Related to the Extent, Location, and Pattern of Late Gadolinium Enhancement. JACC Cardiovasc Imaging. 2019;12(8 Pt 2):1645-55. doi:10.1016/j.jcmg.2018.07.015.
37. 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.) doi:10.15829/1560-4071-2021-4776.
38. Golukhova EZ, Bulaeva NI, Alexandrova SA, et al. The extent of late gadolinium enhancement predicts mortality, sudden death and major adverse cardiovascular events in patients with nonischaemic cardiomyopathy: a systematic review and meta-analysis. Clin Radiol. 2023;78(4):e342-e349. doi:10.1016/j.crad.2022.12.015.
39. Berdibekov BSh, Aleksandrova SA, Golukhova EZ. New evaluation features of ventricular arrhythmia substrate: the role of magnetic resonance imaging. Creative Cardiology. 2019;13(3):217-28. (In Russ.) doi:10.24022/1997-3187-2019-13-3-217-228.
40. Cadour F, Quemeneur M, Biere L, et al. Prognostic value of cardiovascular magnetic resonance T1 mapping and extracellular volume fraction in nonischemic dilated cardiomyopathy. J Cardiovasc Magn Reson. 2023;25(1):7. doi:10.1186/s12968-023-00919-y.
41. Azuma M, Kato S, Kodama S, et al. Relationship between the cardiac magnetic resonance derived extracellular volume fraction and feature tracking myocardial strain in patients with non-ischemic dilated cardiomyopathy. Magn Reson Imaging. 2020;74:14-20. doi:10.1016/j.mri.2020.09.004.
42. Golukhova EZ, Alexandrova SA, Bulaeva NI, et al. Prognostic value of myocardial strain by magnetic resonance imaging in nonischemic dilated cardiomyopathy: a systematic review and meta-analysis. Kardiologiia. 2022;62(10):35-41. (In Russ.) doi:10.18087/cardio.2022.10.n2034.
43. Wang S, Zhang Z, He J, et al. Comprehensive review on gene mutations contributing to dilated cardiomyopathy. Front Cardiovasc Med. 2023;10:1296389. doi:10.3389/fcvm.2023.1296389.
44. Vaikhanskaya TG, Sivitskaya LN, Levdansky OD, et al. Genetic risk factors for dilated cardiomyopathy. Russian Journal of Cardiology. 2021;26(10):4628. (In Russ.) doi:10.15829/1560-4071-2021-4628.
45. van Rijsingen IA, van der Zwaag PA, Groeneweg JA, et al. Outcome in phospholamban R14del carriers: results of a large multicentre cohort study. Circ Cardiovasc Genet. 2014;7(4):455-65. doi:10.1161/CIRCGENETICS.113.000374.
46. Celeghin R, Cipriani A, Bariani R, et al. Filamin-C variant-associated cardiomyopathy: A pooled analysis of individual patient data to evaluate the clinical profile and risk of sudden cardiac death. Heart Rhythm. 2022;19(2):235-43. doi:10.1016/j.hrthm.2021.09.029.
47. Parikh VN, Caleshu C, Reuter C, et al. Regional Variation in RBM20 Causes a Highly Penetrant Arrhythmogenic Cardiomyopathy. Circ Heart Fail. 2019;12(3):e005371. doi:10.1161/CIRCHEARTFAILURE.118.005371.
48. Zaklyazminskaya E, Dzemeshkevich S. The role of mutations in the SCN5A gene in cardiomyopathies. Biochim Biophys Acta. 2016;1863(7 Pt B):1799-805. doi:10.1016/j.bbamcr.2016.02.014.
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Berdibekov B.Sh., Bulaeva N.I., Alexandrova S.A., Golukhova E.Z. Sudden cardiac death risk stratification in dilated cardiomyopathy: a state of the art review. Russian Journal of Cardiology. 2025;30(6S):6114. (In Russ.) https://doi.org/10.15829/1560-4071-2025-6114. EDN: BOSXIO
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