Mitral valve and left ventricular features in malignant mitral valve prolapse

[en] Objective Mitral valve prolapse is a benign condition, however with occasional reports of sudden cardiac death or out-of-hospital cardiac arrest in the absence of severe mitral regurgitation or coronary artery disease, suggesting the existence of a malignant form. The objective of our study was to contribute to the characterisation of malignant mitral valve prolapse. Methods We performed a retrospective analysis of pathology findings in 68 consecutive cases of sudden cardiac death with mitral valve prolapse as lone abnormal finding, reported as cause of death. Results All mitral valve prolapse sudden death cases had mitral valve characteristics of Barlow disease, with extensive bileaflet multisegmental prolapse and dilatation of the annulus. The majority of cases (80.9%) had microscopic left ventricular fibrosis with associated hypertrophy and degenerative features of the myocytes, and some cases (10.9%) had right ventricular fibrosis as well. Conclusions Malignant mitral valve prolapse is Barlow disease. Sudden cardiac death in mitral valve prolapse is due to Barlow disease, which besides the typical mitral valve degeneration may comprise a distinct Barlow disease cardiomyopathy, as suggested by myocyte degeneration and bi-ventricular involvement. © Author(s) (or their employer(s)) 2018.

Disciplines :

Cardiovascular & respiratory systems

Author, co-author :

Garbi, Madalina; King's Health Partners, King's College Hospital NHS Foundation Trust, London, United Kingdom

Lancellotti, Patrizio ; Université de Liège - ULiège > Département des sciences cliniques > Cardiologie - Pathologie spéciale et réhabilitation

Sheppard, M. N.; Cardiovascular Pathology Unit, St Georges Hospital Medical School, London, United Kingdom

Language :

English

Title :

Mitral valve and left ventricular features in malignant mitral valve prolapse

Publication date :

2018

Journal title :

Open Heart

eISSN :

2053-3624

Publisher :

BMJ Publishing Group

Volume :

Issue :

Peer reviewed :

Peer Reviewed verified by ORBi

Available on ORBi :

since 21 December 2018

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Bibliography

Farb A, Tang AL, Atkinson JB, et al. Comparison of cardiac findings in patients with mitral valve prolapse who die suddenly to those who have congestive heart failure from mitral regurgitation and to those with fatal noncardiac conditions. Am J Cardiol 1992;70:234-9.
Anders S, Said S, Schulz F, et al. Mitral valve prolapse syndrome as cause of sudden death in young adults. Forensic Sci Int 2007;171(2-3):127-30.
Dollar AL, Roberts WC. Morphologic comparison of patients with mitral valve prolapse who died suddenly with patients who died from severe valvular dysfunction or other conditions. J Am Coll Cardiol 1991;17:921-31.
Chesler E, King RA, Edwards JE. The myxomatous mitral valve and sudden death. Circulation 1983;67:632-9.
Kligfield P, Levy D, Devereux RB, et al. Arrhythmias and sudden death in mitral valve prolapse. Am Heart J 1987;113:1298-307.
Sriram CS, Syed FF, Ferguson ME, et al. Malignant bileaflet mitral valve prolapse syndrome in patients with otherwise idiopathic outof-hospital cardiac arrest. J Am Coll Cardiol 2013;62:222-30.
Gulotta SJ, Gulco L, Padmanabhan V, et al. The syndrome of systolic click, murmur, and mitral valve prolapse-a cardiomyopathy? Circulation 1974;49:717-28.
Edwards NC, Moody WE, Yuan M, et al. Quantification of left ventricular interstitial fibrosis in asymptomatic chronic primary degenerative mitral regurgitation. Circ Cardiovasc Imaging 2014;7:946-53.
Avierinos JF, Gersh BJ, Melton LJ, et al. Natural history of asymptomatic mitral valve prolapse in the community. Circulation 2002;106:1355-61.
Basso C, Burke M, Fornes P. Association for European Cardiovascular Pathology. Guidelines for autopsy investigation of sudden cardiac death. Virchows Arch 2008;452:11-18.
Sheppard MN. Approach to the cardiac autopsy. J Clin Pathol 2012;65:484-95.
Barlow JB, Bosman CK. Aneurysmal protrusion of the posterior leaflet of the mitral valve. Am Heart J 1966;71:166-78.
Scampardonis G, Yang SS, Maranhão V, et al. Left ventricular abnormalities in prolapsed mitral leaflet syndrome. Review of eightyseven cases. Circulation 1973;48:287-97.
Bui AH, Roujol S, Foppa M, et al. Cardiac magnetic resonance evidence of diffuse myocardial fibrosis in patients with mitral valve prolapse. J Cardiovasc Magn Reson 2015;17(Suppl 1):337):P337.
Han Y, Peters DC, Salton CJ, et al. Cardiovascular magnetic resonance characterization of mitral valve prolapse. JACC Cardiovasc Imaging 2008;1:294-303.
Grigioni F, Enriquez-Sarano M, Ling LH, et al. Sudden death in mitral regurgitation due to flail leaflet. J Am Coll Cardiol 1999;34:2078-85.
Babuty D, Fauchier L, Neel C, et al. Arrhythmias and sudden death in patients with mitral valve prolapse. Card Electrophysiol Rev 1997;1(1/2):264-7.
Zouridakis EG, Parthenakis FI, Kochiadakis GE, et al. QT dispersion in patients with mitral valve prolapse is related to the echocardiographic degree of the prolapse and mitral leaflet thickness. Europace 2001;3:292-8.
Carabello BA. Sudden death in mitral regurgitation: why was I so surprised? J Am Coll Cardiol 1999;34:2086-7.
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.
Sanfilippo AJ, Harrigan P, Popovic AD, et al. Papillary muscle traction in mitral valve prolapse: quantitation by two-dimensional echocardiography. J Am Coll Cardiol 1992;19:564-71.
Basso C, Perazzolo Marra M, Rizzo S, et al. Arrhythmic mitral valve prolapse and sudden cardiac death. Circulation 2015;132:556-66.
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.
Lancellotti P, Garbi M. Malignant mitral valve prolapse: substrates to ventricular remodeling and arrhythmias. Circ Cardiovasc Imaging 2016;9:e005248.
Perazzolo Marra M, Basso C, De Lazzari M, et al. Morphofunctional abnormalities of mitral annulus and arrhythmic mitral valve prolapse. Circ Cardiovasc Imaging 2016;9:e005030.