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Mitral valve and left ventricular features in malignant mitral valve prolapse
Madalina Garbi1, Patrizio Lancellotti2,3, Mary N Sheppard4
1King's Health Partners, King's College Hospital NHS Foundation Trust, London, UK.
Open Heart
|October 27, 2018
Summary
Malignant mitral valve prolapse, identified as Barlow disease, can lead to sudden cardiac death. This condition involves distinct cardiomyopathy with myocyte degeneration and potential bi-ventricular fibrosis.
Area of Science:
- Cardiology
- Pathology
Background:
- Mitral valve prolapse (MVP) is typically benign, but rare sudden cardiac death (SCD) cases suggest a malignant form.
- The characteristics of malignant MVP require further investigation to understand associated cardiac risks.
Purpose of the Study:
- To characterize malignant mitral valve prolapse.
- To investigate the pathological findings in sudden cardiac death cases associated with mitral valve prolapse.
Main Methods:
- Retrospective analysis of pathology findings in 68 consecutive cases of SCD.
- Cases reviewed had mitral valve prolapse as the sole abnormal finding reported as the cause of death.
Main Results:
- All SCD cases with MVP exhibited Barlow disease characteristics, including bileaflet multisegmental prolapse and annular dilatation.
- Microscopic left ventricular fibrosis, hypertrophy, and myocyte degeneration were observed in 80.9% of cases.
- Right ventricular fibrosis was present in 10.9% of cases.
Conclusions:
- Malignant mitral valve prolapse is synonymous with Barlow disease.
- Sudden cardiac death in MVP is attributed to Barlow disease, potentially involving a distinct Barlow disease cardiomyopathy.
- Evidence suggests myocyte degeneration and bi-ventricular involvement in Barlow disease cardiomyopathy.
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