The role of mitral annular disjunction and mitral valve prolapse in the arytmogenesis of ventricular arrhythmias

Authors: Ryzhova T.S., Khotsanyan C.V., Bulaeva N.I.

Company: Bakоulev National Medical Research Center for Cardiovascular Surgery, Moscow, Russian Federation

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Type:  Reviews


DOI: https://doi.org/10.24022/1997-3187-2024-18S-S34-S42

For citation: Ryzhova T.S., Khotsanyan C.V., Bulaeva N.I. The role of mitral annular disjunction and mitral valve prolapse |in the arytmogenesis of ventricular arrhythmias. Creative Cardiology. 2024; 18 (Special Issue): S34–S42 (in Russ.). DOI: 10.24022/1997-3187-2024-18S-S34-S42

Received / Accepted:  02.10.2024 / 23.10.2024

Keywords: mitral annular disjunction arrhythmic mitral valve prolapse arrhythmic risk sudden cardiac death

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Abstract

Mitral annular disjunction (MAD) is a systolic atrial displacement of the hinge point of the posterior mitral valve, characterized by a distinct separation of the mitral valve ring, the wall of the left atrium and the basal part of the left ventricular myocardium. This anomaly has a complex heterogeneous structure with significant individual variations and is characterized by paradoxical dynamics of the fibrous ring during the cardiac cycle, which leads to abnormal mechanical stress on valvular and subvalvular structures and creates prerequisites for the formation of myocardial fibrosis, acting as an arrhythmogenic substrate. Increased interest in disjunction is associated with confirmation of the association of MAD with ventricular arrhythmias, sudden cardiovascular death and arrhythmogenic mitral valve prolapse (MVP).

This review covers the main aspects related to MAD, concerning the peculiarities of its distribution in the population, structure and dynamics during the cardiac cycle, as well as the relationship with PMK and the role in the arimogenesis of ventricular arrhythmias.

References

  1. Sabbag А., Essayagh B., Barrera J., Basso C., Berni A., Cosyns B. et al. EHRA expert consensus statement on arrhythmic mitral valve prolapse and mitral annular disjunction complex in collaboration with the ESC Council on valvular heart disease and the European Association of Cardiovascular Imaging endorsed cby the Heart Rhythm Society, by the Asia Pacific Heart Rhythm Society, and by the Latin American Heart Rhythm Society. Europace. 2022; 24 (12): 1981–2003. DOI: 10.1093/europace/euac125
  2. Dejgaard L.A., Stokke M.K., Hegbom F., Scheirlynck E.S., Gjertsen E., Andresen K. et al. The mitral annulus disjunction arrhythmic syndrome. J. Am. Coll. Cardiol. 2018; 72 (14): 1600–1609. DOI: 10.1016/j.jacc.2018.07.070
  3. Gilbert B.W., Schatz R.A., VonRamm O.T., Behar V.S., Kisslo J.A. Mitral valve prolapse. Two-dimensional echocardiographic and angiographic correlation. Circulation. 1976; 4 (5): 716–723. DOI: 10.1161/01.cir.54.5.716
  4. Bharati S., Granston A.S., Liebson P.R., Loeb H.S., Rosen K.M., Lev M. The conduction system in mitral valve prolapse syndrome with sudden death. Am. Heart J. 1981; 101 (5): 667–670. DOI: 10.1016/0002-8703(81)90235-0
  5. Hutchins G.M., Moore G.W., Skoog D.K. The association of floppy mitral valve with disjunction of the mitral annulus fibrosus. N. Engl. J. Med. 1986; 314: 535–540. DOI: 10.1056/NEJM198602273140902
  6. Wunderlich N.C., Ho S.Y., Flint N., Siegel R.J. Myxomatous mitral valve disease with mitral valve prolapse and mitral annular disjunction: clinical and functional significance of the coincidence. J. Cardiovasc. Dev. Dis. 2021; 8 (2): 9. DOI: 10.3390/jcdd8020009
  7. Basso C., Perazzolo Marra M. Mitral annulus disjunction: emerging role of myocardial mechanical stretch in arrhythmogenesis. J. Am. Coll. Cardiol. 2018; 72 (14); 1610–1612. DOI: 10.1016/j.jacc.2018.07.069
  8. Gulati A., Gulati V., Hu R., Rajiah P.S., Stojanovska J., Febbo J. et al. Mitral annular disjunction: review of an increasingly recognized mitral valve entity. Radiology. Cardiothorac. Imag. 2023; 5 (6): e230131. DOI: 10.1148/ryct.230131
  9. Groeneveld S.A., Kirkels F.P., Cramer M.J., Evertz R., Haugaa K.H., Postema P.G. et al. Prevalence of mitral annulus disjunction and mitral valve prolapse in patients with idiopathic ventricular fibrillation. J. Am. Heart Assoc. 2022; 11 (16): e025364. DOI: 10.1161/JAHA.121.025364
  10. Zhu L., Chua Y.L. Mitral annular disjunction: clinical implications and surgical considerations. Cardiol. Research. 2023; 14 (6): 421–428. DOI: 10.14740/cr1584
  11. Nagata Y., Bertrand P.B., Levine R.A. Malignant mitral valve prolapse: risk and prevention of sudden cardiac death. Curr. Treat. Options Cardiovasc. Med. 2022; 24 (5): 61–86. DOI: 10.1007/s11936-022-00956-3
  12. Putnam A.J., Kebed K., Mor-Avi V., Rashedi N., Sun D., Patel B. et al. Prevalence of mitral annular disjunction in patients with mitral valve prolapse and severe regurgitation. Int. J. Cardiovasc. Imaging. 2020; 36 (7): 1363–1370. DOI: 10.1007/s10554-020-01818-4
  13. Essayagh B., Sabbag A., Antoine C., Benfari G., Yang L.T., Maalouf J. et al. Presentation and outcome of arrhythmic mitral valve prolapse. J. Am. Coll. Cardiol. 2020; 76: 637–649. DOI: 10.1016/j.jacc.2020.06.029
  14. Bennett S., Thamman R., Griffiths T., Oxley C., Khan J.N., Phan T. et al. Mitral annular disjunction: a systematic review of the literature. Echocardiography (Mount Kisco, N.Y.). 2019; 36 (8): 1549–1558. DOI: 10.1111/echo.14437
  15. Konda T., Tani T., Suganuma N. Mitral annular disjunction in patients with primary severe mitral regurgitation and mitral valve prolapse. Echocardiography. 2020; 37: 1716–1722. DOI: 10.1111/echo.14896
  16. Essayagh B., Sabbag A., Antoine C., Benfari G., Batista R., Yang L.T. et al. The mitral annular disjunction of mitral valve prolapse: presentation and outcome. JACC Cardiovasc. Imaging. 2021; 14: 2073–2087. DOI: 10.1016/j.jcmg.2021.04.029
  17. Chivulescu M., Krohg-Sorensen K., Scheirlynck E., Lindberg B.R., Dejgaard L.A., Lie O.H. et al. Mitral annulus disjunction is associated with adverse outcome in Marfan and Loeys–Dietz syndromes. Eur. Heart J. Cardiovasc. Imaging. 2021; 22 (9): 1035–1044. DOI:
  18. Zugwitz D., Fung K., Aung N., Rauseo E., McCracken C., Cooper J. et al. Mitral annular disjunction assessed using CMR imaging: insights from the UK biobank population study. JACC Cardiovasc. Imaging. 2022; 15 (11): 1856–1866. DOI: 10.1016/j.jcmg.2022.07.015
  19. Wu S., Siegel R.J. Mitral annular disjunction: a case series and review of the literature. Front. Cardiovasc. Med. 2022; 9: 976066. DOI: 10.3389/fcvm.2022.976066
  20. Apostolou F., Ioannides M., Mitsis A., Koutsofti C., Deltas C., Avraamides P. Case report: Aborted sudden cardiac death as a first presentation of severe mitral annulus disjunction – a case series and review of the literature. Front. Cardiovasc. Med. 2023; 10: 1171226. DOI:
  21. Faletra F., Leo L., Paiocchi V., Schlossbauer S., Pavon A., Ho S., Maisano F. Morphology of mitral annular disjunction in mitral valve prolapse. J. Am. Soc. Echocardiogr. 2022; 35 (2): 176–186. DOI: 10.1016/j.echo.2021.09.002
  22. Robinson S., Ring L., Augustine D.X., Rekhraj S., Oxborough D., Harkness A. et al. The assessment of mitral valve disease: a guideline from the British Society of Echocardiography. Echo Res. Pract. 2021; 8 (1): G87–136. DOI: 10.1530/ERP-20-0034
  23. Lee A.P., Jin C.N., Fan Y., Wong R.H.L., Underwood M.J., Wan S. Functional implication of mitral annular disjunction in mitral valve prolapse: a quantitative dynamic 3d echocardiographic study. JACC Cardiovasc. Imaging. 2017; 10 (12): 1424–1433. DOI: 10.1016/j. jcmg.2016.11.022
  24. Biondi R., Ribeyrolles S., Diakov C., Amabile N., Ricciardi G., Khelil N. et al. Mapping of the myxomatous mitral valve: The three-dimensional extension of mitral annular disjunction in surgically repaired mitral prolapse. Front. Cardiovasc. Med. 2022; 9: 1036400. DOI: 10.3389/fcvm.2022.1036400
  25. Deng Y., Liu J., Wu S., Li X., Yu H., Tang L. et al. Arrhythmic Mitral Valve Prolapse: A Comprehensive Review. Diagnostics (Basel). 2023; 13 (18): 2868. DOI: 10.3390/diagnostics13182868
  26. Bennett S., Tafuro J., Brumpton M., Bardolia C., Heatlie G., Duckett S. et al. Echocardiographic description and outcomes in a heterogeneous cohort of patients undergoing mitral valve surgery with and without mitral annular disjunction: a health service evaluation. Echo Res. Pract. 2022; 9 (1): 4. DOI: 10.1186/s44156-022-00004-7
  27. Torrent-Guasp F., Ballester M., Buckberg G.D., Carreras F., Flotats A., Carrió I. et al. Spatial orientation of the ventricular muscle band: physiologic contribution and surgical implications. J. Thorac. Cardiovasc. Surg. 2001; 122 (2): 389–392. DOI: 10.1067/mtc.2001.113745
  28. Karagodin I., Singh A., Lang R.M. Pathoanatomy of mitral regurgitation. Struct. Heart. 2020; 4 (4): 254–263. DOI: 10.1080/24748706.2020.1765055
  29. Morningstar J.E., Gensemer C., Moore R., Fulmer D., Beck T.C., Wang C. et al. Mitral valve prolapse induces regionalized myocardial fibrosis. J. Am. Heart Assoc. 2021; 10: e022332. DOI: 10.1161/JAHA.121.022332
  30. Lee J-H., Uhm J-S., Suh Y.J., Kim M., Kim I-S., Jin M-N. et al. Usefulness of cardiac magnetic resonance images for prediction of sudden cardiac arrest in patients with mitral valve prolapse: a multicenter retrospective cohort study. BMC Cardiovasc. Disord. 2021; 21: 546. DOI: 10.1186/s12872-021-02362-2
  31. Chakrabarti A.K., Bogun F., Liang J.J. Arrhythmic mitral valve prolapse and mitral annular disjunction: clinical features, pathophysiology, risk stratification, and management. J. Cardiovasc. Dev. Dis. 2022; 9 (2): 61. DOI: 10.3390/jcdd9020061
  32. Aleksandrova S.A., Glazkova E.Yu., Zolotaykina O.C., Berdibekov B.Sh., Dorofeev A.V., Aslanidis I.P., Golukhova E.Z. Assessment of structural left heart abnormalities in mitral annular disjunction patients by magnetic resonance imaging. Creative Cardiology. 2023; 17 (3): 386–401 (in Russ.). DOI: 10.24022/1997-3187-2023-17-3-386-401
  33. Serguladze S.Yu., Pronicheva I.V. Results and limitations of catheter ablation of ventricular tachycardia in non-ischemic cardiomyopathies: endocardial, epicardial and intramural approaches. The Bulletin of Bakoulev Center. Cardiovascular Diseases. 2021; 22 (2): 139–150 (in Russ.). DOI: 10.24022/1810-0694-2021-22-2-139-150
  34. Serguladze S.Yu., Kotanova E.S. Non-invasive identification of non-ischemic scars and ventricular arrhythmia substrates: evidence and limitations. Annals of Arrhythmology. 2021; 18 (3): 176–186 (in Russ.). DOI: 10.15275/annaritmol.2021.3.7
  35. Serguladze S.Yu., Pronicheva I.V. Report on scientific and practical activities of the department of surgical treatment of tachyarhythmias of Bakoulev Center for 2022 and perspectives for further development. The Bulletin of Bakoulev Center. Cardiovascular Diseases. 2023; 24 (3): 264–275 (in Russ.). DOI: 10.24022/1810-0694-2023-24-3-264-275
  36. Hussain N., Bhagia G., Doyle M., Rayarao G., Williams R.B., Biederman R.W. Mitral annular disjunction; how accurate are we? A cardiovascular MRI study defining risk. Int. J. Cardiol. Heart Vasc. 2023; 49: 101298. DOI: 10.1016/j.ijcha.2023.101298
  37. Enriquez A., Shirai J.Y., Huang J., Liang J., Briceño D., Hayashi T. et al. Papillary muscle ventricular arrhythmias in patients with arrhythmic mitral valve prolapse: Electrophysiologic substrate and catheter ablation outcomes. J. Cardiovasc. Electrophysiol. 2019; 30 (6): 827–835. DOI: 10.1111/jce.13900
  38. Carmo P., Andrade M.J., Aguiar C., Rodrigues R., Gouveia R., Silva J.A. et al. Mitral annular disjunction in myxomatous mitral valve disease: a relevant abnormality recognizable by transthoracic echocardiography. Cardiovasc. Ultrasound. 2010; 8: 53.
  39. Miller M.A., Adams D.H., Pandis D., Robson P.M., Pawale A., Pyzik R. et al. Hybrid positron emission tomography/magnetic resonance imaging in arrhythmic mitral valve prolapse. JAMA Cardiol. 2020; 5: 1000–1005. DOI: 10.1001/jamacardio.2020.1555
  40. Chakrabarti A.K., Deshmukh A., Liang J.J., Madamanchi C., Ghannam M., Morady F., Bogun F. Mitral annular substrate and ventricular arrhythmias in arrhythmogenic mitral valve prolapse with mitral annular disjunction. JACC Clin. Electrophysiol. 2023; 9 (8): 1265–1275. DOI: 10.1016/j.jacep.2023.02.010
  41. Aabel E.W., Chivulescu M., Lie Ø.H., Hopp E., Gjertsen E., Ribe M. et al. Ventricular arrhythmias in arrhythmic mitral valve syndrome-a prospective continuous long-term cardiac monitoring study. Europace. 2023; 25 (2): 506–516. DOI: 10.1093/europace/euac182
  42. Basso C., Iliceto S., Thiene G., Perazzolo Marra M. Mitral valve prolapse, ventricular arrhythmias, and sudden death. Circulation. 2019; 140: 952–964. DOI: 10.1161/CIRCULATIONAHA.118.034075
  43. Hiemstra Y.L., Tomsic A., Gripari P., van Wijngaarden A.L., van der Pas S.L., Palmen M. et al. Evolution from mitral annular dysfunction to severe mitral regurgitation in Barlow’s disease. Interact. Cardiovasc. Thorac. Surg. 2021; 32 (4): 506–514. DOI: 10.1093/icvts/ivaa304
  44. Mantegazza V., Gripari P., Tamborini G., Muratori M., Fusini L., Ghulam Ali S. et al. 3D echocardiography in mitral valve prolapse. Front. Cardiovasc. Med. 2023; 9: 1050476. DOI: 10.3389/fcvm.2022.1050476

About Authors

  • Taisia S. Ryzhova, resident; ORCID
  • Chinara V. Khotsanyan, Cand. Med. Sci., Cardiologist; ORCID
  • Naida I. Bulaeva, Cand. Biol. Sci., Associate Professor, Head of the Department, Cardiologist; ORCID

Chief Editor

Elena Z. Golukhova, MD, PhD, DSc, Professor, Academician of Russian Academy of Sciences, Director of Bakoulev National Medical Research Center for Cardiovascular Surgery


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