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

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