Arrhythmic Mitral Valve Prolapse: Introducing an Era of Multimodality Imaging-Based Diagnosis and Risk Stratification

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.

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

“…It is also reported a correlation between MAD and SCD 19 but no cut‐off value for MAD, that could identify high risk patients, has been defined yet. Using TTE, some authors 26,28 defined MAD ≥2 mm, others 47 ≥5 mm or any length 27,29 . Carmo et al 27 .…”

Two Cases of Arrhythmic Mitral Valve Prolapse: Evil Has Many Faces

“…Conversely, fibroelastic deficiency is caused by impaired production of connective tissue, resulting in leaflet and chordae thinning. 4 …”

Hidden in Plain Sight: A Case of Arrhythmic Mitral Valve Prolapse Presenting as Cardiac Arrest

“…MAD has profound implications on the dynamic behavior of the MV shape, resulting in paradoxical annular flattening and increased diameter during systole. This alters the physiological saddle-shape of the annulus, which increases stress on the MV leaflets leading to their degeneration suggesting that MAD might be one of the most important precursors of VA [4]. In addition, altered shape of the MV annulus increases myocardial stretch in the inferobasal segments and papillary muscles.…”