Diagnosis of Mitral Valve Prolapse: Much More than Simple Prolapse. Multimodality Approach to Risk Stratification and Therapeutic Management

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.

Transthoracic echocardiography for arrhythmic mitral valve prolapse: Phenotypic characterization as first step

“…The cause of fibrosis is the mechanical stretching of the myxomatous bileaflet disease, mitral annulus disjunction (MAD) and systolic curling on the adjacent myocardium. As a consequence, the diagnosis of MVP and its phenotypic characterization with a multimodality imaging approach are crucial, particularly in patients at high risk for SCD 17 . Notwithstanding this, TTE is the first imaging tool in MVP work‐up.…”

“…As a consequence, the diagnosis of MVP and its phenotypic characterization with a multimodality imaging approach are crucial, particularly in patients at high risk for SCD. 17 Notwithstanding this, TTE is the first imaging tool in MVP work-up. In experienced hands it can provide detailed information on morphofunctional abnormalities of the valve, mitral annulus, focal myocardial contractility/deformation providing parameters useful to identify patients at risk for major arrhythmias.…”

Mitral valve prolapse: From new mechanisms to diagnostic challenges

“…Importantly, MVP has recently been recognized as a common cause of arrhythmias, including sudden cardiac death (SCD). This life-threatening phenotype is referred to as "arrhythmogenic" or "malignant" MVP [23], and its exact prevalence is unclear [24,25]. In our preliminary results, 9.4% of the MVP families had a history of SCD (unpublished data).…”

“…For arrhythmic events prevention and treatment, beta-blockers are the first-choice treatment for symptomatic or asymptomatic patients with non-sustained or sustained ventricular arrhythmias. However, high-risk features such as ventricular arrhythmias, hypercontractility, and fibrosis may prompt further electrophysiologic study investigations [23]. Some authors have considered ablation protocols to relieve the arrhythmia burden with high procedural success, although recurrence of ventricular arrhythmia was not uncommon [35].…”

Special Issue: State of the Art of Cardiac Multimodality Imaging

“…This annulus disjunction is defined as an abnormal insertion of the hinge line of the posterior mitral leaflet on the atrial wall-in order words, an atrialization of the posterior leaflet base. Alenazy, A. et al [1] describes, in an excellent review, the recent advances of multimodality imaging in the assessment of morphologic characteristics of mitral valvular prolapse and the role of CMR in the evaluation and risk stratification of this disease. Furthermore, multimodality imaging can guide treatment, allowing the identification of different, mitral valve prolapse phenotypes.…”