Синдром Бругада: субэпикардиальная катетерная аблация как современный метод терапии - анализ клинического случая

Представлен клинический случай 27-летнего пациента с синдромом Бругада, у которого была выявлена патогенная укорачивающая мутация в гене SCN5A. После многократных неадекватных срабатываний имплантируемого кардиовертера-дефибриллятора и тяжёлых синкопальных эпизодов была успешно выполнена эпикардиальная радиочастотная аблация аритмогенного субстрата правого желудочка с применением трёхмерного картирования Ripple Mapping. Это привело к нормализации электрокардиографического паттерна и отсутствию срабатываний имплантируемого кардиовертера-дефибриллятора в течение 11 месяцев наблюдения. Полученные данные подтверждают перспективность катетерного вмешательства для контроля полиморфных желудочковых аритмий у больных с синдромом Бругада, включая случаи с вариативностью фенотипа при SCN5A-ассоциированной мутации.

1. Curio A, Santarpia G, Indolfi C, et al. The Brugada syndrome - from gene to therapy. Circ J. 2017;81: 290-297. https://doi.org/10.1253/circj.CJ-16-0529.

2. Zhang J, Sacher F, Hoffmayer K, et al. Cardiac electrophysiological substrate underlying the ECG phenotype and electrogram abnormalities in Brugada syndrome patients. Circulation. 2015;131(22): 1950-1959. https://doi.org/10.1161/CIRCULATIONAHA.114.014711.

3. Ревишвили АШ, Сергуладзе СЮ, Проничева ИВ, и др. Синдром Бругада: клинические рекомендации по ведению больных. Москва: ГЭОТАР-Медиа; 2020. 70 с.

4. Priori SG, Wilde AA, Horie M, et al. HRS/EHRA/ APHRS expert consensus statement on the diagnosis and management of patients with inherited primary arrhythmia syndromes. Heart Rhythm. 2013;10(12): 1932-1963. https://doi.org/10.1016/j.hrthm.2013.05.014.

5. Popa IP, Șerban DN, Mărănducă MA, et al. Brugada syndrome: from molecular mechanisms and genetics to risk stratification. Int J Mol Sci. 2023;24(4): 3328. https://doi.org/10.3390/ijms24043328.

6. Giustetto C, Cerrato N, Dusi V, et al. The Brugada syndrome: pharmacological therapy. Eur Heart J Suppl. 2023;25(Suppl C): C32-C37. https://doi.org/10.1093/eurheartjsupp/suad036.

7. 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. Heart Rhythm. 2018;15(10): e190-e252. https://doi.org/10.1016/j.hrthm.2017.10.035.

8. Krahn AD, Behr ER, Hamilton R, et al. Brugada syndrome. JACC Clin Electrophysiol. 2022;8(3): 386-405. https://doi.org/10.1016/j.jacep.2021.12.001.

9. Zeppenfeld K, Tfelt-Hansen J, de Riva M, et al. ESC Scientific Document Group. 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. https://doi.org/10.1093/eurheartj/ehac262.

10. Pappone C, Brugada J, Vicedomini G, et al. Electrical substrate elimination in 135 consecutive patients with Brugada syndrome. Circ Arrhythm Electrophysiol. 2017;10(5): e005053. https://doi.org/10.1161/CIRCEP.117.005053.

11. Kotake Y, Barua S, Kazi S, et al. Efficacy and safety of catheter ablation for Brugada syndrome: an updated systematic review. Clin Res Cardiol. 2023;112: 1715-1726. https://doi.org/10.1007/s00392-022-02020-3.

12. Luther V, Linton NW, Jamil-Copley S, et al. A prospective study of ripple mapping the post-infarct ventricular scar to guide substrate ablation for ventricular tachycardia. Circ Arrhythm Electrophysiol. 2016;9(6): e004072. https://doi.org/10.1161/CIRCEP.116.004072.

13. Providencia R, Cavaco D, Carmo P, et al. Ripple mapping for the detection of long-duration action potential areas in patients with Brugada syndrome. BioRxiv. 2018. Preprint. https://doi.org/10.1101/263145.

14. Monasky MM, Micaglio E, Locati ET, et al. Evaluating the use of genetics in Brugada syndrome risk stratification. Front Cardiovasc Med. 2021;8: 652027. https://doi.org/10.3389/fcvm.2021.652027.

15. Amin AS, Meregalli PG, Bardai A, et al. Fever increases the risk for cardiac arrest in Brugada syndrome. Ann Intern Med. 2008;149(3): 216-218. https://doi.org/10.7326/0003-4819-149-3-200808050-00020.

16. Roterberg G, El-Battrawy I, Veith M, et al. Arrhythmic events in Brugada syndrome patients induced by fever. Ann Noninvasive Electrocardiol. 2020;25(3): e12723. https://doi.org/10.1111/anec.12723.

17. Kawada S, Morita H, Antzelevitch C, et al. Shanghai Score System for Diagnosis of Brugada Syndrome: Validation of the Score System and System and Reclassification of the Patients. JACC Clin Electrophysiol. 2018 Jun;4(6): 724-730. https://doi.org/10.1016/j.jacep.2018.02.009.

18. G Ribouleau, J B Gourraud, N Behar, et al. Phenotype heterogeneity of SCN5A mutations in Brugada syndrome. European Heart Journal. 2024;45(1): ehae666.320. https://doi.org/10.1093/eurheartj/ehae666.320.

19. Milman A, Andorin A, Gourraud JB, et al. Genotype-Phenotype Correlation of SCN5A Mutation for the Clinical and Electrocardiographic Characteristics of Probands With Brugada Syndrome. Circulation. 2018;138(7): 629-643. https://doi.org/10.1161/CIRCULATIONAHA.117.027983.

20. Milman A, Behr ER, Gray B, et al. Genotype-Phenotype Correlation of SCN5A Genotype in Patients With Brugada Syndrome and Arrhythmic Events: Insights From the SABRUS in 392 Probands. Circulation: Genomic and Precision Medicine. 2021;14(6) :e003442. https://doi.org/10.1161/CIRCGEN.120.003442.

21. Deica AV, Paduraru LF, Paduraru DN, et al. The SCN5A Gene Is a Predictor of Phenotype Severity in Brugada Syndrome: A Comprehensive Literature Review. Med Princ Pract. 2023; 32(1): 1-8. https://doi.org/10.1159/000528375.

22. Meregalli PG, Tan HL, Probst V, et al. Type of SCN5A mutation determines clinical severity and degree of conduction slowing in loss-of-function sodium channelopathies. Heart Rhythm. 2009 Mar;6(3): 341-8. https://doi.org/10.1016/j.hrthm.2008.11.009.

23. Brugada P, Brugada J. Right bundle branch block, persistent ST segment elevation and sudden cardiac death: a distinct clinical and electrocardiographic syndrome. Journal of the American College of Cardiology. 1992;20(6): 1391-1396. https://doi.org/10.1016/0735-1097(92)90253-J.