Abstract
Mitral valve prolapse is a common cardiac condition, with an estimated prevalence between 1% and 3%. Most patients have a benign course, but ever since its initial description mitral valve prolapse has been associated to sudden cardiac death. Although the causal relationship between mitral valve prolapse and sudden cardiac death has never been clearly demonstrated, different factors have been implicated in arrhythmogenesis in patients with mitral valve prolapse. In this work, we offer a comprehensive overview of the etiology and the genetic background, epidemiology, pathophysiology, and we focus on the state-of-the-art imaging-based diagnosis of mitral valve prolapse. Going beyond the classical, well-described clinical factors, such as young age, female gender and auscultatory findings, we investigate multimodality imaging features, such as alterations of anatomy and function of the mitral valve and its leaflets, the structural and contractile anomalies of the myocardium, all of which have been associated to sudden cardiac death.
Highlights
A mechanicistic insight into the molecular pathophysiology of mechanical stressinduced fibrosis has been offered by Blomme and colleagues in 2019 who showed a transient upregulation of transforming growth factor β2 (TGF-β2) and a longer lasting connective tissue growth factor (CTGF) expression, a known downstream effector of TGFβ [73]
Never demonstrated to be a risk factor for sudden cardiac death (SCD), this tissue Doppler imaging (TDI) signal has been found to be overrepresented in a group of patients with arrhythmic Mitral valve prolapse (MVP) [82], and it has been proposed that the sharp tugging of the papillary muscle and the left ventricular (LV) wall, which move apically, may be arrhythmogenic, in the absence of late gadolinium enhancement (LGE) at Cardiovascular magnetic resonance (CMR) [83]
Notwithstanding the fact that the causal relationship between MVP and SCD has never been clearly documented, the data we have presented suggest that there are different factors which may contribute to arrhythmogenesis in patients with MVP
Summary
Mitral valve (MV) is a complex three-dimensional (3D) structure composed of valvular (i.e., annulus, commissures and leaflets) and sub-valvular (i.e., papillary muscles and chordae tendineae) components (Figure 1). Lamofand anatomy of the sub-valvular ventricular surfacework of theof body the colleagues leaflet) anddefined tertiary the They found that chordae tendineae arise from the tips of the papillary muscles leaflet, attaching to the ventricular wall) [9]. The posteromedial papillary muscle gives rise to chords attaching to the medial half of both leaflets (i.e., posteromedial commissure, P3, tics 2021, 11, x FOR PEER REVIEW (anterolateral and posteromedial) and depending on the site of insertion can be classified (those that attach to the distal, free edge of the rough zone), secondary (those that ventricular surface of the body of the leaflet) and tertiary (those exclusive to the posterior leaflet, attaching to the ventricular wall) [9]. Diagnostics 2021, 11,as primary commissure, A1, P1, and lateral segments of P2 and A2) [7] (Figure 1)
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