4.6 Review

Histopathological insights into mitral valve prolapse-induced fibrosis

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Dark-blood late gadolinium enhancement CMR improves detection of papillary muscle fibrosis in patients with mitral valve prolapse

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Summary: Dark-blood LGE CMR improves detection of LGE at the papillary muscles in patients with MVP compared to bright-blood LGE, with no difference in myocardial fibrosis detection. There is no association found between LGE at the papillary muscles and at the myocardium.

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Brian. P. P. Kelley et al.

Summary: Sudden cardiac death (SCD) can occur in mitral valve prolapse (MVP) without other comorbidities. Arrhythmic MVP (AMVP) is associated with ventricular ectopy, while malignant MVP (MMVP) has a high risk of SCD. The cause of AMVP is inflammation, fibrosis, and scarring in the left ventricle, while the exact risk of MMVP is unknown. Similar cardiac characteristics to AMVP can be seen in idiopathic SCD, suggesting a possible association. Further research is needed to identify MMVP and prevent SCD in MVP patients.

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Research Opportunities in the Treatment of Mitral Valve Prolapse

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Summary: In order to improve the outcomes for patients with mitral valve prolapse (MVP) who are at risk of severe mitral regurgitation, heart failure, or sudden cardiac death, it is crucial to identify those at higher risk. The use of advanced imaging techniques, novel omics methodologies, and web-based platforms for large-scale collaborations have provided researchers with the opportunity to make rapid advancements in the field of MVP. The National Heart, Lung, and Blood Institute has recently organized a webinar-based workshop to identify contemporary research opportunities in MVP treatment. This report highlights three specific areas of focus in the workshop: improving the management of degenerative mitral regurgitation and associated left ventricular systolic dysfunction; preventing sudden cardiac death in MVP; and understanding the mechanisms and progression of MVP through genetic studies and animal models, which may lead to the development of medical therapies.

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Malignant Mitral Valve Prolapse: Risk and Prevention of Sudden Cardiac Death

Yasufumi Nagata et al.

Summary: The purpose of this review is to study the malignant phenotype in mitral valve prolapse (MVP) characterized by life-threatening ventricular arrhythmias and sudden cardiac arrest and death (SCD). Recent findings suggest that mechanical forces induced by prolapse play a central role in the development of arrhythmogenic substrate and triggering life-threatening arrhythmias. More research is needed to establish effective therapies.

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Native and Post-Repair Residual Mitral Valve Prolapse Increases Forces Exerted on the Papillary Muscles: A Possible Mechanism for Localized Fibrosis?

Matthew H. Park et al.

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Wenyang Jiang et al.

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Systematic quantification of histologic ventricular fibrosis in isolated mitral valve prolapse and sudden cardiac death

Hui-Chen Han et al.

Summary: The study found that individuals with isolated MVP and SCD have left ventricular and interventricular septum fibrosis, with less right ventricular fibrosis. Additionally, in iMVP-SCD cases, left ventricular fibrosis is significantly higher in the lateral and posterior walls compared to the anterior wall and interventricular septum.

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Replacement Myocardial Fibrosis in Patients With Mitral Valve Prolapse Relation to Mitral Regurgitation, Ventricular Remodeling, and Arrhythmia

Anne-Laure Constant Dit Beaufils et al.

Summary: The study found that LV replacement myocardial fibrosis is common in patients with MVP, associated with mitral valve apparatus alteration, more dilated LV, MR grade, and ventricular arrhythmia, and independently associated with cardiovascular events. These findings suggest an MVP-related myocardial disease, with cardiac magnetic resonance providing additional information for MVP patients.

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Mitral Valve Prolapse Induces Regionalized Myocardial Fibrosis

Jordan E. Morningstar et al.

Summary: The study found prominent regional left ventricular fibrosis in patients and mice with MVP, supporting a relationship between MVP and progressive damaging effects on LV structure. These regionalized molecular and cellular changes suggest a reactive response of the papillary and inferobasal myocardium to increased chordal tension from a prolapsing valve.

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Multimodality Imaging Assessment of Myocardial Fibrosis

Sumit Gupta et al.

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