European guidelines for the management of patients with ventricular arrhythmias and the prevention of sudden cardiac death 2022: cardiomyopathy. What’s new?

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Abstract

The review provides information on new indications that should be guiden the diagnosis and treatment of ventricular arrhythmias in patients with cardiomyopathy. The analysis of modern definitions and classifications of cardiomyopathy is given. The issues of ventricular arrhythmias in different cardiomyopathy phenotypes, risk stratification of sudden cardiac death and its prevention are considered in detail.

About the authors

Tatiana N. Novikova

North-Western State Medical University named after I.I. Mechnikov

Author for correspondence.
Email: novikova-tn@mail.ru
ORCID iD: 0000-0003-4655-0297
SPIN-code: 3401-0329

Cand. Sci. (Med.), associate professor of the Department of Hospital Therapy and Cardiology named after M.S. Kushakovskiy. North-Western State Medical University named after I.I. Mechnikov, Saint Petersburg

Russian Federation, Saint Petersburg

Fatima I. Bitakova

North-Western State Medical University named after I.I. Mechnikov

Email: Fatima.Bitakova@szgmu.ru
ORCID iD: 0000-0001-6637-8266
SPIN-code: 8624-7193

Cand. Sci. (Med.), associate professor

Russian Federation, Saint Petersburg

Valeria S. Ignateva

North-Western State Medical University named after I.I. Mechnikov

Email: valeria2311@bk.ru
ORCID iD: 0009-0008-9446-9374

6th year student

Russian Federation, Saint Petersburg

Vladimir I. Novikov

North-Western State Medical University named after I.I. Mechnikov

Email: novikov-vi@mail.ru
ORCID iD: 0000-0002-2493-6300
SPIN-code: 6909-3377

Dr. Sci. (Med.), professor

Russian Federation, Saint Petersburg

Sergey A. Sayganov

North-Western State Medical University named after I.I. Mechnikov

Email: ssayganov@gmail.com
ORCID iD: 0000-0001-8325-1937
SPIN-code: 2174-6400

Dr. Sci. (Med.), professor, rector

Russian Federation, Saint Petersburg

Vladislava A. Shcherbakova

North-Western State Medical University named after I.I. Mechnikov

Email: shcher.vl@yandex.ru

6th year student

Russian Federation, Saint Petersburg

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2. Fig. 1. Algorithm for risk stratification and primary prevention of sudden cardiac death in patients with dilated cardiomyopathy/hypokinetic non-dilated cardiomyopathy [2] AV — atrioventricular; CMR — cardiac magnetic resonance; DCM — dilated cardiomyopathy; HNDCM — hypokinetic non-dilated cardiomyopathy; ICD — implantable cardioverter defibrillator; ILR — implantable loop recorder; LMNA — nuclear lamin gene; LVEF — left ventricular ejection fraction; N — no; NSVT — non-sustained ventricular tachycardia; PES, programmed electrical stimulation; SCD — sudden cardiac death; SMVT — sustained monomorphic ventricular tachycardia; VA — ventricular arrhythmias; Y — yes a Risk factors: unexplained syncope, pathogenic variants in PLN, FLNC, or RBM20, LGE on CMR, inducible SMVT at PES. b The 2018 ESC Guidelines for the diagnosis and management of syncope

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3. Fig. 2. Genetic risk for VA/SCD, typical triggers for VA/SCD, age at presentation with VA/SCD, sex predominance, and typical VA in different diseases associated with VA/SCD — presence of genetic risks; — trigger — physical and emotional stress; — trigger — swimming, diving; — syncope during sleep; —trigger — a sharp sound; — trigger – physical stress ACS — acute coronary syndrome; ARVC — arrhythmogenic right ventricular cardiomyopathy; BrS — Brugada syndrome; CAD — coronary artery disease; CPVT — catecholaminergic polymorphic ventricular tachycardia; DCM — dilated cardiomyopathy; HCM — hypertrophic cardiomyopathy; LQT — long QT syndrome; MVT — monomorphic ventricular tachycardia; PVT — polymorphic ventricular tachycardia; rTOF — repaired tetralogy of Fallot; SCD — sudden cardiac death; VA — ventricular arrhythmia

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4. Fig. 3. Electrocardiogram of a patient suffering from biventricular arrhythmogenic cardiomyopathy (explanation in the text)

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5. Fig. 4. Electrocardiogram of a patient suffering from right ventricular arrhythmogenic cardiomyopathy, at the time of tipical ventricular tachycardia (explanation in text)

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6. Fig. 5. Echocardiogram of a patient with left ventricular hypertrabeculation. Apical 4-chamber view. Noteworthy is the dilatation of the left ventricle and pronounced trabecularity in the area at the apex

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7. Table 1. Recommendations for genetic testing for dilated/hypokinetic nondilated cardiomyopath

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8. Table 2. Recommendations for the examination of patients suffering from dilated/hypokinetic non-dilated cardiomyopathy and their relatives

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9. Table 3. Recommendations for the primary prevention of sudden cardiac death in patients suffering from dilated/hypokinetic nondilated cardiomyopathy

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10. Table 4. Recommendations for secondary prevention of sudden cardiac death in patients suffering from dilated/hypokinetic nondilated cardiomyopathy

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11. Table 5. General recommendations for patients with mutations in genes encoding lamin

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12. Table 6. Recommendations for the diagnosis and management of patients suffering from arrhythmogenic cardiomyopathy

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13. Table 7. Recommendations for risk stratification and primary prevention of sudden death in arrhythmogenic cardiomyopathy

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14. Table 8. Recommendations for secondary prevention of sudden death and treatment of ventricular arrhythmias in arrhythmogenic cardiomyopathy

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15. Table 9. Recommendations for the management of relatives of a patient with arrhythmogenic cardiomyopathy

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16. Table 10. Recommendations for risk stratification, prevention of sudden cardiac death, and treatment of ventricular arrhythmias in hypertrophic cardiomyopathy

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17. Table 11. Recommendations for cardioverter defibrillator implantation in left ventricular non-compaction

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18. Table 12. Recommendations for implantation of a cardioverter-defibrillator in patients with cardiac amyloidosis

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19. Rice. 2 application 1

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20. Rice. 2 application 2

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21. Rice. 2 application 3

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22. Rice. 2 application 4

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23. Rice. 2 application 5

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24. Rice. 2 appendix 6

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