Table Info

Table 1.

Arrhythmia severity classification [23]

VA	Arrhythmia burden rate (bpm)	Risk of mortality (hazard ratio, 95% Confidence Interval)
Mild	PVC ≥ 5% VT runs 120	1.2 [0.63–2.14]
Moderate	VT runs 120–179
Severe	VT runs ≥ 180 or history of sustained VT	2.94 [1.36–6.36]

Arrhythmia burden rate (bpm)

Typical examples

Risk of mortality (hazard ratio, 95% Confidence Interval)

Mild

PVC ≥ 5%

VT runs 120

1.2 [0.63–2.14]

Moderate

VT runs 120–179

Severe

VT runs ≥ 180

or history of sustained VT

2.94 [1.36–6.36]

bpm: beats per minute; VT: ventricular tachycardia; nsVT: non-sustained VT

Declarations

Author contributions

Maria Możdżan: Writing—original draft, Investigation, Writing—review & editing. KWD: Investigation, Writing—review & editing, Supervision. Monika Możdżan: Conceptualization, Investigation, Writing—review & editing. MB, ID, ZM and MS: Writing—review & editing.

Conflicts of interest

The authors declare that they have no conflicts of interest.

Ethical approval

The study was conducted according to the guidelines of the Declaration of Helsinki and approved by the Ethics Committee of the Medical University of Lodz (approve number: RNN/73/22/KE).

Consent to participate

Informed consent to participate in the study was obtained from all participants.

Consent to publication

Informed consent to publication was obtained from all participants.

Availability of data and materials

Not applicable.

Funding

Not applicable.

Copyright

©The Author(s) 2023.

References

Nalliah CJ, Mahajan R, Elliott AD, Haqqani H, Lau DH, Vohra JK, et al. Mitral valve prolapse and sudden cardiac death: a systematic review and meta-analysis. Heart. 2019;105:144–51. [DOI] [PubMed]

Basso C, Perazzolo Marra M, Rizzo S, De Lazzari M, Giorgi B, Cipriani A, et al. Arrhythmic mitral valve prolapse and sudden cardiac death. Circulation. 2015;132:556–66. [DOI] [PubMed]

Maron BJ, Rowin EJ, Maron MS. Paradigm of sudden death prevention in hypertrophic cardiomyopathy. Circ Res. 2019;125:370–8. [DOI] [PubMed]

Perazzolo Marra M, Basso C, De Lazzari M, Rizzo S, Cipriani A, Giorgi B, et al. Morphofunctional abnormalities of mitral annulus and arrhythmic mitral valve prolapse. Circ Cardiovasc Imaging. 2016;9:e005030. [DOI] [PubMed] [PMC]

Delwarde C, Capoulade R, Mérot J, Le Scouarnec S, Bouatia-Naji N, Yu M, et al. Genetics and pathophysiology of mitral valve prolapse. Front Cardiovasc Med. 2023;10:1077788. [DOI] [PubMed] [PMC]

Levine HJ, Isner JM, Salem DN. Primary versus secondary mitral valve prolapse: clinical features and implications. Clin Cardiol. 1982;5:371–5. [DOI] [PubMed]

Devereux RB, Kramer-Fox R, Brown WT, Shear MK, Hartman N, Kligfield P, et al. Relation between clinical features of the mitral prolapse syndrome and echocardiographically documented mitral valve prolapse. J Am Coll Cardiol. 1986;8:763–72. [DOI] [PubMed]

Strahan NV, Murphy EA, Fortuin NJ, Come PC, Humphries JO. Inheritance of the mitral valve prolapse syndrome. Discussion of a three-dimensional penetrance model. Am J Med. 1983;74:967–72. [DOI] [PubMed]

Kyndt F, Gueffet JP, Probst V, Jaafar P, Legendre A, Le Bouffant F, et al. Mutations in the gene encoding filamin A as a cause for familial cardiac valvular dystrophy. Circulation. 2007;115:40–9. [DOI] [PubMed]

10.

Miyamoto R, Sekine A, Fujimaru T, Suwabe T, Mizuno H, Hasegawa E, et al. Echocardiographic findings and genotypes in autosomal dominant polycystic kidney disease. Kidney Dis (Basel). 2022;8:246–52. [DOI] [PubMed] [PMC]

11.

Baş VN, Yılmaz Agladioglu S, Özgür S, Karademir S, Aycan Z. Investigation of autoimmune diseases accompanying Hashimoto’s thyroiditis in children and adolescents and evaluation of cardiac signs. J Pediatr Endocrinol Metab. 2015;28:767–71. [DOI] [PubMed]

12.

van Wijngaarden AL, Kruithof BPT, Vinella T, Barge-Schaapveld DQCM, Ajmone Marsan N. Characterization of degenerative mitral valve disease: differences between fibroelastic deficiency and Barlow’s disease. J Cardiovasc Dev Dis. 2021;8:23. [DOI] [PubMed] [PMC]

13.

Viani GM, Leo LA, Borruso MG, Klersy C, Paiocchi VL, Schlossbauer SA, et al. Mitral annulus morphometry in degenerative mitral regurgitation phenotypes. Echocardiography. 2020;37:612–9. [DOI] [PubMed]

14.

Antoine C, Mantovani F, Benfari G, Mankad SV, Maalouf JF, Michelena HI, et al. Pathophysiology of degenerative mitral regurgitation. Circ Cardiovasc Imaging. 2018;11:e005971. [DOI] [PubMed]

15.

Adams DH, Rosenhek R, Falk V. Degenerative mitral valve regurgitation: best practice revolution. Eur Heart J. 2010;31:1958–66. [DOI] [PubMed] [PMC]

16.

Anyanwu AC, Adams DH. Etiologic classification of degenerative mitral valve disease: Barlow’s disease and fibroelastic deficiency. Semin Thorac Cardiovasc Surg. 2007;19:90–6. [DOI] [PubMed]

17.

Pype LL, Bertrand PB, Paelinck BP, Heidbuchel H, Van Craenenbroeck EM, Van De Heyning CM. Left ventricular remodeling in non-syndromic mitral valve prolapse: volume overload or concomitant cardiomyopathy? Front Cardiovasc Med. 2022;9:862044. [DOI] [PubMed] [PMC]

18.

Adams DH, Anyanwu AC, Sugeng L, Lang RM. Degenerative mitral valve regurgitation: surgical echocardiography. Curr Cardiol Rep. 2008;10:226–32. [DOI] [PubMed]

19.

Alenazy A, Eltayeb A, Alotaibi MK, Anwar MK, Mulafikh N, Aladmawi M, et al. Diagnosis of mitral valve prolapse: much more than simple prolapse. Multimodality approach to risk stratification and therapeutic management. J Clin Med. 2022;11:455. [DOI] [PubMed] [PMC]

20.

Carpentier A, Chauvaud S, Fabiani JN, Deloche A, Relland J, Lessana A, et al. Reconstructive surgery of mitral valve incompetence: ten-year appraisal. J Thorac Cardiovasc Surg. 1980;79:338–48. [DOI] [PubMed]

21.

Barlow JB, Bosman CK. Aneurysmal protrusion of the posterior leaflet of the mitral valve. Am Heart J. 1966;71:166–78. [DOI] [PubMed]

22.

Basso C, Iliceto S, Thiene G, Perazzolo Marra M. Mitral valve prolapse, ventricular arrhythmias, and sudden death. Circulation. 2019;140:952–64. [DOI] [PubMed]

23.

Essayagh B, Sabbag A, Antoine C, Benfari G, Yang LT, et al. Presentation and outcome of arrhythmic mitral valve prolapse. J Am Coll Cardiol. 2020;76:637–49. [DOI] [PubMed]

24.

Sabbag A, Essayagh B, Barrera JDR, 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. Europace. 2022;24:1981–2003. [DOI] [PubMed]

25.

Bhutto ZR, Barron JT, Liebson PR, Uretz EF, Parrillo JE. Electrocardiographic abnormalities in mitral valve prolapse. Am J Cardiol. 1992;70:265–6. [DOI] [PubMed]

26.

Peighambari MM, Alizadehasl A, Totonchi Z. Electrocardiographic changes in mitral valve prolapse syndrome. J Cardiovasc Thorac Res. 2014;6:21–3. [DOI] [PubMed] [PMC]

27.

Stevant D, Plessis M, Gourraud JB, Cueff C, Piriou N, Thollet A, et al. ECG modifications, life-threatening ventricular arrhythmias and relation to mitral valve apparatus phenotype in mitral valve prolapse. Eur Heart J. 2020;41:ehaa946.1885. [DOI]

28.

Alizadeh-Asl A. Reverse Δ-wave as a possible sign in electrocardiography to diagnose mitral valve prolapse. BMC Res Notes. 2011;4:16. [DOI] [PubMed] [PMC]

29.

Franchitto N, Bounes V, Telmon N, Rougé D. Mitral valve prolapse and out-of-hospital sudden death: a case report and literature review. Med Sci Law. 2010;50:164–7. [DOI] [PubMed]

30.

Anders S, Said S, Schulz F, Püschel K. Mitral valve prolapse syndrome as cause of sudden death in young adults. Forensic Sci Int. 2007;171:127–30. [DOI] [PubMed]

31.

Akcay M, Yuce M, Pala S, Akcakoyun M, Ergelen M, Kargin R, et al. Anterior mitral valve length is associated with ventricular tachycardia in patients with classical mitral valve prolapse. Pacing Clin Electrophysiol. 2010;33:1224–30. [DOI] [PubMed]

32.

Eltahlawi MA, Sanad AM, Ghazal KH, Abdelwahed AT. Can QT dispersion improve the accuracy of stress ECG TMT in detecting myocardial ischemia in chronic stable CAD patients? A stress myocardial perfusion imaging study. Egypt Heart J. 2021;73:5. [DOI] [PubMed] [PMC]

33.

Wolf PA, Abbott RD, Kannel WB. Atrial fibrillation as an independent risk factor for stroke: the Framingham Study. Stroke. 1991;22:983–8. [DOI] [PubMed]

34.

Berbarie RF, Roberts WC. Frequency of atrial fibrillation in patients having mitral valve repair or replacement for pure mitral regurgitation secondary to mitral valve prolapse. Am J Cardiol. 2006;97:1039–44. [DOI] [PubMed]

35.

Hinderer S, Schenke-Layland K. Cardiac fibrosis—a short review of causes and therapeutic strategies. Adv Drug Deliv Rev. 2019;146:77–82. [DOI] [PubMed]

36.

Pavon AG, Monney P, Schwitter J. Mitral valve prolapse, arrhythmias, and sudden cardiac death: the role of multimodality imaging to detect high-risk features. Diagnostics (Basel). 2021;11:683. [DOI] [PubMed] [PMC]

37.

Bui AH, Roujol S, Foppa M, Kissinger KV, Goddu B, Hauser TH, et al. Diffuse myocardial fibrosis in patients with mitral valve prolapse and ventricular arrhythmia. Heart. 2017;103:204–9. [DOI] [PubMed] [PMC]

38.

Kitkungvan D, Nabi F, Kim RJ, Bonow RO, Khan MA, Xu J, et al. Myocardial fibrosis in patients with primary mitral regurgitation with and without prolapse. J Am Coll Cardiol. 2018;72:823–34. [DOI] [PubMed]

39.

Bharati S, Granston AS, Liebson PR, Loeb HS, Rosen KM, Lev M. The conduction system in mitral valve prolapse syndrome with sudden death. Am Heart J. 1981;101:667–70. [DOI] [PubMed]

40.

Carmo P, Andrade MJ, Aguiar C, Rodrigues R, Gouveia R, Silva JA. Mitral annular disjunction in myxomatous mitral valve disease: a relevant abnormality recognizable by transthoracic echocardiography. Cardiovasc Ultrasound. 2010;8:53. [DOI] [PubMed] [PMC]

41.

Parwani P, Avierinos JF, Levine RA, Delling FN. Mitral valve prolapse: multimodality imaging and genetic insights. Prog Cardiovasc Dis. 2017;60:361–9. [DOI] [PubMed] [PMC]

42.

Adabifirouzjaei F, Hsiao A, DeMaria AN. Mitral valve prolapse—the role of cardiac imaging modalities. Struct Heart. 2022;6:100024. [DOI] [PubMed] [PMC]

43.

Bennett S, Thamman R, Griffiths T, Oxley C, Khan JN, Phan T, et al. Mitral annular disjunction: a systematic review of the literature. Echocardiography. 2019;36:1549–58. [DOI] [PubMed]

44.

Lee JH, Uhm JS, Suh YJ, Kim M, Kim IS, Jin MN, 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] [PubMed] [PMC]

45.

Hutchins GM, Moore GW, Skoog DK. The association of floppy mitral valve with disjunction of the mitral annulus fibrosus. N Engl J Med. 1986;314:535–40. [DOI] [PubMed]

46.

Dejgaard LA, Skjølsvik ET, Lie ØH, Ribe M, Stokke MK, Hegbom F, et al. The mitral annulus disjunction arrhythmic syndrome. J Am Coll Cardiol. 2018;72:1600–9. [DOI] [PubMed]

47.

Groeneveld SA, Kirkels FP, Cramer MJ, Evertz R, Haugaa KH, Postema PG, et al. Prevalence of mitral annulus disjunction and mitral valve prolapse in patients with idiopathic ventricular fibrillation. J Am Heart Assoc. 2022;11:e025364. [DOI] [PubMed] [PMC]

48.

Eriksson MJ, Bitkover CY, Omran AS, David TE, Ivanov J, Ali MJ, et al. 44 Mitral annular disjunction in advanced myxomatous mitral valve disease: echocardiographic detection and surgical correction. J Am Soc Echocardiogr. 2005;18:1014–22. [DOI] [PubMed]

49.

Lee APW, Jin CN, Fan Y, Wong RHL, Underwood MJ, Wan S. Functional implication of mitral annular disjunction in mitral valve prolapse. JACC Cardiovasc Imaging. 2017;10:1424–33. [DOI] [PubMed]

50.

Sriram CS, Syed FF, Ferguson ME, Johnson JN, Enriquez-Sarano M, Cetta F, et al. Malignant bileaflet mitral valve prolapse syndrome in patients with otherwise idiopathic out-of-hospital cardiac arrest. J Am Coll Cardiol. 2013;62:222–30. [DOI] [PubMed]

51.

De Silva K, Kumar S. Catheter ablation for atrial fibrillation: Are we isolating our most vulnerable patients? Heart Lung Circ. 2022;31:1315–7. [DOI] [PubMed]

52.

Aabel EW, Chivulescu M, Dejgaard LA, Ribe M, Gjertsen E, Hopp E, et al. Tricuspid annulus disjunction: novel findings by cardiac magnetic resonance in patients with mitral annulus disjunction. JACC Cardiovasc Imaging. 2021;14:1535–43. [DOI] [PubMed]

53.

Garbi M, Lancellotti P, Sheppard MN. Mitral valve and left ventricular features in malignant mitral valve prolapse. Open Heart. 2018;5:e000925. [DOI] [PubMed] [PMC]

54.

Han HC, Parsons SA, Teh AW, Sanders P, Neil C, Leong T, et al. Characteristic histopathological findings and cardiac arrest rhythm in isolated mitral valve prolapse and sudden cardiac death. J Am Heart Assoc. 2020;9:e015587. [DOI] [PubMed] [PMC]

55.

Nordhues BD, Siontis KC, Scott CG, Nkomo VT, Ackerman MJ, Asirvatham SJ, et al. Bileaflet mitral valve prolapse and risk of ventricular dysrhythmias and death. J Cardiovasc Electrophysiol. 2016;27:463–8. [DOI] [PubMed]

56.

Vairo A, Desalvo P, Rinaudo A, Piroli F, Tribuzio A, Ballatore A, et al. Echocardiographic parameters to predict malignant events in arrhythmic mitral valve prolapse population. J Clin Med. 2023;12:1232. [DOI] [PubMed] [PMC]

57.

Ermakov S, Gulhar R, Lim L, Bibby D, Fang Q, Nah G, et al. Left ventricular mechanical dispersion predicts arrhythmic risk in mitral valve prolapse. Heart. 2019;105:1063–9. [DOI] [PubMed] [PMC]

58.

Muthukumar L, Rahman F, Jan MF, Shaikh A, Kalvin L, Dhala A, et al. The pickelhaube sign: novel echocardiographic risk marker for malignant mitral valve prolapse syndrome. JACC Cardiovasc Imaging. 2017;10:1078–80. [DOI] [PubMed]

59.

Zienciuk-Krajka A, Daniłowicz-Szymanowicz L, Dorniak K, Owczuk R, Kaufmann D, Zacharek D, et al. Clinical characteristics of patients with arrhythmic mitral valve prolapse in a single tertiary center: prevalence of electrocardiographic and myocardial abnormalities. Kardiol Pol. 2021;79:693–6. [DOI] [PubMed]

60.

Turker Y, Ozaydin M, Acar G, Ozgul M, Hoscan Y, Varol E, et al. Predictors of ventricular arrhythmias in patients with mitral valve prolapse. Int J Cardiovasc Imaging. 2010;26:139–45. [DOI] [PubMed]

61.

Narayanan K, Uy-Evanado A, Teodorescu C, Reinier K, Nichols GA, Gunson K, et al. Mitral valve prolapse and sudden cardiac arrest in the community. Heart Rhythm. 2016;13:498–503. [DOI] [PubMed] [PMC]

62.

Constant Dit Beaufils AL, Huttin O, Jobbe-Duval A, Senage T, Filippetti L, Piriou N, et al. Replacement myocardial fibrosis in patients with mitral valve prolapse. Circulation. 2021;143:1763–74. [DOI] [PubMed]