Cardiomyopathy associa­ ted with ventricular pre-excitation or dissynchronous cardiomyopathy in patients with asymptomatic and symptomatic Wolff-Parkinson-White syndrome: personal experience in patient management

Aim. To study the causal relationship between the functioning of accessory pathway and changes in intraventricular hemodynamics in patients with dissynchronous cardiomyopathy.

Methods. The study included 83 patients with registered preexitation according to ECG data. Patients were divided into study and control group. The study group included 33 patients with diagnosed echocardiographic signs of dissynchronous cardiomyopathy (reduced ejection fraction (EF), increased chamber volume and/or decreased global longitudinal strain (GLS) of the left ventricle (LV)). The control group included 50 patients with Wolff-Parkinson-White syndrome/ phenomenon without dissynchrony.

Results. After radiofrequency ablation (RFA), patients in the study group showed natural normalization of the QRS complex width and LV GLS. The median QRS width before RFA was 110 ms [100; 120] and after RFA 70 ms [60; 80] (p<0.0001). The median LV GLS before RFA was -18.2% [-19.1; -17] and after RFA -21.3% [-23; -19.2] (p<0.0001). Despite the absence of statistically significant differences in the QRS width in patients in the study and control groups, statistically significant differences in the size and LV EF were revealed. In the study group, the median of end-diastolic volume (EDV) of LV (as a percentage of the parameter from the individual predicted norm) was 112% [102; 123] and EF was 64% [55; 65], and in patients from the control group 102% [97; 112] and 65% [64; 66], respectively. The level of significance of the differences for EDV was p=0.0183, for EF it was p=0.0003.

Conclusion. Risk factors of dissynchronous cardiomyopathy (age of patients, right-sided localization of accessory pathway, severity of ventricular preexcitation) are probably of important clinical significance, but are not specific.

1. Emmel M, Balaji S, Sreeram N. Ventricular preexcitation associated with dilated cardiomyopathy: a causal relationship? Cardiol Young. 2004;14(6): 594-599. https://doi:10.1017/S1047951104006031

2. Mamchur SE, Sizova IN, Shmulevich SA. Evaluation of the intraventricular dyssynchrony in patients with manifesting ventricular preexitation using three-dimensional echocardiography. Annaly Aritmologii. 2014;11(1): 31-37 (In Russ.) https://doi.org/10.15275/annaritmol.2014.1.4.

3. Abdelmohsen G, El-Farargy N, Abdelaziz O, et al. Using 2D speckle-tracking echocardiography to localize the accessory pathway and evaluate cardiac function and dyssynchrony in pediatric patients with Wolf-Parkinson-White syndrome. Eur J Pediatr. 2023;182(8): 3659-3669. https://doi:10.1007/s00431-023-05040-x;

4. Cheng Z, Liu H, Chen Y, et al. Two-dimensional speckle tracking imaging to assess the hazards of left ventricular function and ventricular wall motion disorders in children with pre-excitation syndrome and the efficacy of radiofrequency ablation treatment. Eur Rev Med Pharmacol Sci. 2023;27(17):7882-7890. https://doi:10.26355/eurrev_202309_33545.

5. Mamedova AI, Prihod’ko NA, Lubimceva TA, et al Mechanical dyssynchrony in patients with chronic heart failure and left bundle branch block. Siberian Journal of Clinical and Experimental Medicine. 2024;39(3): 72-79 (In Russ.) https://doi.org/10.29001/2073-8552-2024-39-3-72-79.

6. Miyazaki A, Uemura H. Perspective of preexcitation induced cardiomyopathy; early septal contraction, and subsequent rebound stretch. J Cardiol. 2022;79(1):30-35. https://doi.10.1016/j.jjcc.2021.08.017.

7. Etheridge SP, Gakenheimer-Smith L, Asaki SY, et al. Asymptomatic Wolff-Parkinson-White Syndrome: An Ounce of Prevention Is Worth the Risk of Cure. Curr Cardiol Rep. 2023;25(6): 543-551. https://doi.org/10.1007/s11886-023-01879-6.

8. Savelev AA, Kamenev AV, Berman MV, Medvedev MM. Examination and treatment of a female patient with symptomatic manifesting WPW phenomenon: case report. Journal of Arrhythmology. 2022;29(4): e1-e8. (In Russ.). https://doi.org/10.35336/VA-2022-4-11.

9. Philip Saul J, Kanter RJ, et al. PACES/HRS expert consensus statement on the use of catheter ablation in children and patients with congenital heart disease: Developed in partnership with the Pediatric and Congenital Electrophysiology Society (PACES) and the Heart Rhythm Society (HRS). Endorsed by the governing bodies of PACES, HRS, the American Academy of Pediatrics (AAP), the American Heart Association (AHA), and the Association for European Pediatric and Congenital Cardiology (AEPC). Heart Rhythm. 2016;13(6): e251-e289. https://doi.10.1016/j.hrthm.2016.02.009.

10. Pasotti M, The MOGE(S) Classification for a Phenotype-Genotype Nomenclature of Cardiomyopathy: More Questions Than Answers? J Am Coll Cardiol. 2014;63(23): 2584. https://doi.org/10.1016/j.jacc.2014.01.078.

11. Lipshultz SE, Law YM, Asante-Korang A, et al. Cardiomyopathy in Children: Classification and Diagnosis: A Scientific Statement From the American Heart Association. Circulation. 2019;140(1): e9-e68. https://doi.10.1161/ CIR.0000000000000682.

12. Dai C, Guo B, Li WenXiu, et al. The effect of ventricular pre-excitation on ventricular wall motion and left ventricular systolic function. Europace. 2018;20(7): 1175- 1181. https://doi.10.1093/europace/eux242.

13. Cameli M, Mandoli GE, Sciaccaluga C, Mondillo S. More than 10 years of speckle tracking echocardiography: Still a novel technique or a definite tool for clinical practice? Echocardiography. 2019; 36(5): 958-970. https://doi.10.1111/echo.14339.

14. Hayashi T, Ono H, Kaneko Y. Echocardiographic assessment of ventricular contraction and synchrony in children with isolated complete atrioventricular block and epicardial pacing: Implications of interventricular mechanical delay. Echocardiography. 2018;35(9):1370-1377. https://doi.10.1111/echo.14035.

15. Suzuki S, Hokosaki T, Iwamoto M. Pharmacologic therapy with flecainide for asymptomatic Wolff-Parkinson-White syndrome in an infant with severe left ventricular dyssynchrony. Cardiol Young. 2018;28(7): 970-973. https://doi.10.1017/S1047951118000252.

16. Sekine M, Masutani S, Imamura T, et al. Improvement in Dyssynchrony with Pharmacological Ablation of Right-Sided Accessory Pathway-Induced Cardiomyopathy in Infants. Int Heart J. 2019;60(5): 1201-1205. https://doi.org/10.1536/ihj.18-723.

17. Sumitomo NF, Fukushima N, Miura M. Flecainide improves cardiac synchronization in an early infant with Wolff-Parkinson-White syndrome with left ventricular dyssynchrony. J Cardiol Cases. 2020;22(1):1-4. https://doi.10.1016/j.jccase.2020.03.004.

18. Chencheng D, Min Z, Wanming S, et al. Case Report: Ventricular preexcitation-induced dilated cardiomyopathy improved by the pharmacologic suppression of ventricular preexcitation in three infants, and literature review. Front Pediatr. 2024; 12. https://doi.10.3389/fped.2024.1302534.

19. Popov SV, Svintsova LI, Kovalev IA, et al. Effectiveness of endocardial radiofrequency ablation of tachyarrhythmias in patients of the first year of life. Journal of Arrhythmology. 2012;67(67): 5-10. (In Russ.).

20. Makarov LM, Komolyatov VN, Kiseleva II, et al. Standard ECG parameters in children. Guidelines. PH “Medpractika-M” - Moscow, 2018. (In Russ.).

21. Makarov LM. Holter monitoring. 2 nd edition. PH “Medpractika-M” - Moscow. ISBN: 5-901654-55-2. (In Russ.).

22. Lai WW, Geva T, Shirali GS, et al. Guidelines and standards for performance of a pediatric echocardiogram: a report from the Task Force of the Pediatric Council of the American Society of Echocardiography. J Am Soc Echocardiogr. 2006;19(12): 1413-1430. https://doi.org/10.1016/j.echo.2006.09.001.

23. Martsinkevich GI, Sokolov АА. Echocardiography in children: anthropometric and developmental norms. Rossijskij pediatricheskij zhurnal. 2012;2: 17-21. (In Russ.)

24. Martsinkevich GI, Sokolov AA. The Child Heart program application for automation of a workplace of the doctor of an echocardiography: the certificate on the state registration of the computer program No. 20096105560, is registered in the Register of the computer programs on January 23, 2009 Moscow 2009; 14.

25. Lang RM, Badano LP, Mor-Avi V, 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. Eur Heart J Cardiovasc Imaging. 2015;16(3): 233-270. https://doi.org/10.1093/ehjci/jev014.

26. Dai CC, Guo BJ, Li WX, et al. Dyssynchronous ventricular contraction in Wolff-Parkinson-White syndrome: a risk factor for the development of dilated cardiomyopathy. Eur J Pediatr. 2013;172(11): 1491-1500. https://doi.org/10.1007/s00431-013-2070-z.

27. Fukunaga H, Akimoto K, Furukawa T, et al. Improvement in non-tachycardia-induced cardiac failure after radiofrequency catheter ablation in a child with a right-sided accessory pathway. Heart Vessels. 2013;28(6): 802-807. https://doi.org/10.1007/s00380-013-0322-5.

28. Zimmerman FJ, Pahl E, Rocchini AP, et al. High incidence of incessant supraventricular tachycardia in pediatric patients referred for cardiac transplantation. Pacing clin Electrophysiol. 1996;19: 663.

29. Kohli U, Pumphrey KL, Ahmed A, Das S. Pre-excitation induced ventricular dysfunction and successful berlin heart explantation after accessory pathway ablation. J Electrocardiol. 2018;51(6): 1067-1070. https://doi.10.1016/j.jelectrocard.2018.09.008.

30. Dai C., Guo B., Li WenXiu, et al. The effect of ventricular pre-excitation on ventricular wall motion and left ventricular systolic function. Europace. 2018;20(7): 1175- 1181.https://doi.org/10.1093/europace/eux242.

31. Kwon EN, Carter KA, Kanter RJ. Radiofrequency catheter ablation for dyssynchrony-induced dilated cardiomyopathy in an infant. Congenit Heart Dis. 2014;9(6): E179-E184. https://doi.org/10.1111/chd.12124.

32. Kartofeleva EO, Svintsova LI, Dzhaffarova OYu, Smorgon AV, Krivolapov SN. Effects of accessory pathway catheter ablation on left ventricular function in patients with dyssynchrony-induced cardiomyopathy associated with pre-excitation syndrome. Journal of Arrhythmology. 2024;31(2): 5-12. (In Russ.)https://doi.org/10.35336/VA-1314.

33. Plotnikova IV, Svintsova LI, Dzhaffarova OYu, et al. Primary cardiomyopathies in childhood: clinical and diagnostic features (literature review). Siberian Journal of Clinical and Experimental Medicine. 2022;37(3): 65-74. (In Russ.) https://doi.org/10.29001/2073-8552-2022-37-3-65-74.