Malignant mitral valve prolapse in sudden cardiac death cases was universally characterized by Barlow disease, with microscopic left ventricular fibrosis present in 80.9% of cases.
Observational (n=68)
Malignant mitral valve prolapse associated with sudden cardiac death is characterized by Barlow disease features and frequent bi-ventricular myocardial fibrosis, suggesting a distinct cardiomyopathy.
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.
Garbi et al. (2018) conducted an observational in Malignant mitral valve prolapse (n=68). Malignant mitral valve prolapse in sudden cardiac death cases was universally characterized by Barlow disease, with microscopic left ventricular fibrosis present in 80.9% of cases.