Abstract
Mitral valve prolapse (MVP) is the most common valvular disease with a prevalence of 2%. It has generally a benign course; however, recent findings suggested an association between MVP and complex arrhythmias and eventually cardiac arrest and for this reason, it is also called arrhythmogenic MVP. Subjects who experience this complication are in general young women, with thickened mitral leaflets or bileaflet prolapse not necessarily associated with severe mitral regurgitation (MR). The nature of the relation between MVP and cardiac arrest is not clearly understood. Actually, the challenging task is to find the cluster of prognostic factors including T-wave inversion, polymorphic premature ventricular contractions, bileaflet prolapse, MR severity, but most importantly, those parameters of hypercontractility, mitral annulus disjunction (MAD), and myocardial fibrosis using a multimodality approach. Transthoracic echocardiography is the first-line imaging modality for the diagnosis of MVP, but also for detecting MAD and hypercontractility, followed by cardiac magnetic resonance for tissue characterization and detection of myocardial and papillary muscle fibrosis, using either late gadolinium enhancement (at the basal segment of the inferolateral wall and papillary muscles) (macro-fibrosis), or diffuse fibrosis by T1 mapping (native and post contrast T1). Moreover, there are also preliminary data on positron emission tomography utilizing 18F-fluorodeoxyglucose as a tool for providing evidence of early myocardial inflammation. The objective of this review article is to provide the clinician with an overview and a practical clinical approach to MVP for risk stratification and treatment guidance.
Highlights
Mitral valve prolapse (MVP) is the displacement of one or both mitral leaflets into the left atrium in systole, described for the first time by Barlow in 1963 [1]
mitral annulus disjunction (MAD) is an anatomical entity that has been associated with MVP; it is an abnormal insertion of the hinge line of the posterior mitral leaflet (PML) on the atrial wall, characterized by distinct separation of the mitral valve annulus-left atrial wall and the basal region of the posterolateral left ventricular (LV) myocardium, defined as atrialization of the posterior leaflet base
Data derived from cardiac magnetic resonance (CMR) in MVP patients showed a basal to mid segment ratio >1.5, which was found to be higher in patients with positive Late gadolinium enhancement (LGE), without reaching statistical significance [14]
Summary
Mitral valve prolapse (MVP) is the displacement of one or both mitral leaflets into the left atrium in systole, described for the first time by Barlow in 1963 [1]. As elegantly described by Reinier and Chugh in their editorial [19] commenting Dilling’s et al paper [13], postmortem diagnosis alone might underestimate the prevalence of malignant MVP whereas cardiac magnetic resonance (CMR) can have the potential to identify those MVP patients at high risk. This is important because, beyond the classical definition of MVP, the challenge is to identify the subset of patients at risk of developing c-VA and SCD using multimodality imaging and to guide the best treatment (Figure 1)
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