Arrhythmic Mitral Valve Prolapse: New Menaces of the Known Disease
https://doi.org/10.20996/1819-6446-2023-01-05
Abstract
Mitral valve prolapse (MVP) has long been the subject of intense discussions regarding the prognosis and follow-up tactics. In most cases, this condition has a benign prognosis. However, recent autopsy and follow-up studies have shown risks of developing sudden cardiac death (SCD) in some subgroups of patients who have this clinical phenomenon. The proposed literature review uses the population of patients with MVP with the highest probability of developing life-threatening ventricular tachyarrhythmias. Patients with the presence of a complex of changes, including bicuspid MVP, negative T waves in the inferior and lateral leads on a standard 12-lead electrocardiogram (ECG), and a special anatomical phenomenon called mitral annular disjunction (MAD), are at high risk of developing ventricular ectopias and VSS. A reflection of the high risk of SCD in such patients is the increase of ventricular ectopy according to Holter monitoring. The presence of a bicuspid MVP and the MAD phenomenon, which is a separation of the line of attachment of the posterior mitral leaflet from the basal inferior wall segment towards the atrial wall, determines the presence of a special form of MVP, the so-called arrhythmogenic MVP. Hence, in most cases MVP has a benign prognosis. However, patients with the aforementioned ar- rhythmic MVP signs must be given particular attention and annual follow-up including ECG control, Holter monitoring and echocardiographic examination of the heart to reduce the risk of ventricular tachyarrhythmias and SCD development.
About the Authors
D. A. KuzhelRussian Federation
Dmitriy A. Kuzhel.
Krasnoyarsk
G. V. Matyushin
Russian Federation
Gennadiy V. Matyushin.
Krasnoyarsk
E. A. Savchenko
Russian Federation
Elena A. Savchenko.
Krasnoyarsk
References
1. Barlow JB, Pocock WA. The significance of late systolic murmurs and mid-late systolic clicks. Md State Med J. 1963;12:76-7.
2. Criley JM, Lewis KB, Humphries JO, et al. Prolapse of the mitral valve: clinical and cine-angiocardiographic findings. Br Heart J. 1966;28(4):488-96. DOI:10.1136/hrt.28.4.488.
3. Feigenbaum H. Acquired valvular heart disease. In: Feigenbaum H. Echocardiography. 5th ed. Philadelphia: Lea & Febiger; 1995.
4. Levy D, Savage D. Prevalence and clinical features of mitral valve prolapse. Am Heart J. 1987;113(5):1281-90. DOI:10.1016/0002-8703(87)90956-2.
5. Mantegazza V, Volpato V, Gripari P, et al. Multimodality imaging assessment of mitral annular disjunction in mitral valve prolapse. Heart. 2020;107(1):25-32. DOI:10.1136/heartjnl-2020-317330.
6. Savage DD, Garrison RJ, Devereux RB, et al. Mitral valve prolapse in the general population. 1. epidemiologic features: the Framingham study. Am Heart J. 1983;106(3):571-6. DOI:10.1016/0002-8703(83)90704-4.
7. Freed LA, Levy D, Levine RA, et al. Prevalence and clinical outcome of mitral-valve prolapse. N Engl J Med. 1999;341(1):1-7. DOI:10.1056/NEJM199907013410101.
8. Martini B, Basso C, Thiene G. Sudden death in mitral valve prolapse with Holter monitoring-documented ventricular fibrillation: evidence of coexisting arrhythmogenic right ventricular cardiomyopathy. Int J Cardiol. 1995;49(3):274-8. DOI:10.1016/0167-5273(95)02294-7.
9. Nalliah CJ, Mahajan R, Elliott AD, et al. Mitral valve prolapse and sudden cardiac death: a systematic review and meta-analysis. Heart. 2019;105(2):144-51. DOI:10.1136/heartjnl-2017-312932.
10. Coutsoumbas GV, Di Pasquale G. Mitral valve prolapse with ventricular arrhythmias: does it carries a worse prognosis? Eur Heart J Suppl. 2021;23(Suppl):E77-E82. DOI:10.1093/eurheartj/suab096.
11. Sriram CS, Syed FF, Ferguson ME, et al. Malignant bileaflet mitral valve prolapse syndrome in patients with otherwise idiopathic out-of-hospital cardiac arrest. J Am Coll Cardiol. 2013;62(3):222-30. DOI:10.1016/j.jacc.2013.02.060.
12. Henle J. Handbuch der systematischen Anatomie des Menschen. v. 3 pt. 1. Germany: Vieweg; 1876.
13. Zimmerman J. The functional and surgical anatomy of the heart. Ann R Coll Surg. 1966;39(6):348-66.
14. McAlpine WA. Heart and Coronary Arteries: An Anatomical Atlas for Clinical Diagnosis, Radiological Investigation, and Surgical Treatment. New York, NY: Springer-Verlag; 1975.
15. Toh H, Mori S, Izawa Y, et al. Prevalence and extent of mitral annular disjunction in structurally normal hearts: comprehensive 3D analysis using cardiac computed tomography. Eur Heart J Cardiovasc Imag. 2021;22(6):614-22. DOI:10.1093/ehjci/jeab022.
16. Bharati S, Granston AS, Liebson PR, et al. The conduction system in mitral valve prolapse syndrome with sudden death. Am Heart J. 1981;101(5):667-70. DOI:10.1016/0002-8703(81)90235-0.
17. Hutchins GM, Moore GW, Skoog DK. The association of floppy mitral valve with disjunction of the mitral annulus fibrosis. N Engl J Med. 1986;314(9):535-40. DOI:10.1056/NEJM198602273140902.
18. Basso C, Marra MP, Rizzo S, et al. Arrhythmic Mitral Valve Prolapse and Sudden Cardiac Death. Circulation. 2015;132(7):556-66. DOI:10.1161/circulationaha.115.016291.
19. Dejgaard LA, Skjølsvik ET, Lie ØH, et al. The mitral annulus disjunction arrhythmic syndrome. J Am Coll Cardiol. 2018;72(14):1600-9. DOI:10.1016/j.jacc.2018.07.070.
20. Bennett S, Thamman R, Griffiths T, et al. Mitral annular disjunction: a systematic review of the literature. Echocardiography. 2019;36(8):1549-58. DOI:10.1111/echo.14437.
21. van Wijngaarden AL, de Riva M, Hiemstra YL, et al. Parameters associated with ventricular arrhythmias in mitral valve prolapse with significant regurgitation. Heart. 2021;107(5):411-8. DOI:10.1136/heartjnl-2020-317451.
22. Han HC, Ha FJ, Teh AW, et al. Mitral valve prolapse and sudden cardiac death: a systematic review. J Am Heart Assoc. 2018;7(23):e010584. DOI:10.1161/JAHA.118.010584.
23. Chivulescu M, Aabel E, Dejgaard L, et al. Cardiac Arrest in a Patient With Arrhythmic Mitral Valve Prolapse Syndrome. Multiple Possible Etiologies. J Am Coll Cardiol Case Rep. 2021;3(16):1769-73. DOI:10.1016/j.jaccas.2021.08.019.
24. Essayagh B, Iacuzio L, Civaia F, et al. Usefulness of 3-tesla cardiac magnetic resonance to detect mitral annular disjunction in patients with mitral valve prolapse. Am J Cardiol. 2019;124(11):1725-30. DOI:10.1016/j.amjcard.2019.08.047.
25. Shirobokikh OE, Bylova NA. Mitral valve prolapse and sudden cardiac death: who is in the risk group. Russ Arch Inter Med. 2016;3(29):25-9 (In Russ.) DOI:10.20514/2226-6704-2016-6-3-25-29.
26. Bains S, Tester DJ, Asirvatham SJ, et al. A novel truncating variant in FLNC-encoded filamin C may serve as a proarrhythmic genetic substrate for arrhythmogenic bileaflet mitral valve prolapse syndrome. Mayo Clin Proc. 2019;94(5):906-13. DOI:10.1016/j.mayocp.2018.11.028.
27. Muthukumar L, Rahman F, Jan M.F, et al. The Pickelhaube Sign: Novel Echocardiographic Risk Marker for Malignant Mitral Valve Prolapse Syndrome. JACC Cardiovasc Imaging. 2017;10(9):1078-80. DOI:10.1016/j.jcmg.2016.09.016.
28. Cipriani A, Bauce B. Ventricular arrhythmias in mitral valve prolapse: new explanations for an old problem. Heart. 2021;107(5):353-4. DOI:10.1136/heartjnl-2020-318086.
29. Haugaa K. Improving the imaging diagnosis of mitral annular disjunction. Heart. 2021;107(1):4-5. DOI:10.1136/heartjnl-2020-317667.
30. Villatore A, Sala S, Stella S, et al. Autoimmune Myocarditis and Arrhythmogenic Mitral Valve Prolapse: An Unexpected Overlap Syndrome. J Cardiovasc Dev Dis. 2021;8(11):151. DOI:10.3390/jcdd8110151.
31. Constant Dit Beaufils AL, Huttin O, Jobbe-Duval A, et al. Replacement myocardial fibrosis in patients with mitral valve prolapse: relation to mitral regurgitation, ventricular remodeling and arrhythmia. Circulation. 2021;143(18):1763-74. DOI:10.1161/CIRCULATIONAHA.120.050214.
32. Priori SG, Blomstrom-Lundqvist C, Mazzanti A, et al. ESC Scientific Document Group. 2015 ESC Guidelines for the management of patients with ventricular arrhythmias and the prevention of sudden cardiac death. Eur Heart J. 2015;36(41):2793-867. DOI:10.1093/eurheartj/ehv316.
33. Lebedev DS, Mikhailov EN, Neminuschiy NM, et al. Ventricular arrhythmias. Ventricular tachycardias and sudden cardiac death. 2020 Clinical guidelines. Russ J Cardiol. 2021;26(7):4600 (In Russ.) DOI:10.15829/1560-4071-2021-4600.
34. 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: A Report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guide-lines and the Heart Rhythm Society. J Am Coll Cardiol. 2018;72(14):e91-e220. DOI:10.1016/j.jacc.2017.10.054.
35. Pelliccia A, Sharma S, Gati S, et al. ESC Scientific Document Group. 2020 ESC Guidelines on sports cardiology and exercise in patients with cardiovascular disease. Eur Heart J. 2021;42(1):17-96. DOI:10.1093/eurheartj/ehaa605.
36. Higuchi K, Bhargava M. Management of premature ventricular complexes. Heart 2022;108(7):565-72. DOI:10.1136/heartjnl-2020-318628.
Review
For citations:
Kuzhel D.A., Matyushin G.V., Savchenko E.A. Arrhythmic Mitral Valve Prolapse: New Menaces of the Known Disease. Rational Pharmacotherapy in Cardiology. 2023;19(1):77-82. (In Russ.) https://doi.org/10.20996/1819-6446-2023-01-05