Abstract
The morphological changes that occur in myxomatous mitral valve disease (MMVD) involve various components, ultimately leading to the impairment of mitral valve (MV) function. In this context, intrinsic mitral annular abnormalities are increasingly recognized, such as a mitral annular disjunction (MAD), a specific anatomical abnormality whereby there is a distinct separation between the mitral annulus and the left atrial wall and the basal portion of the posterolateral left ventricular myocardium. In recent years, several studies have suggested that MAD contributes to myxomatous degeneration of the mitral leaflets, and there is growing evidence that MAD is associated with ventricular arrhythmias and sudden cardiac death. In this review, the morphological characteristics of MAD and imaging tools for diagnosis will be described, and the clinical and functional aspects of the coincidence of MAD and myxomatous MVP will be discussed.
Highlights
Since the recognition that mitral valve (MV) leaflets can be affected by myxomatous degeneration (myxomatous mitral valve disease (MMVD)) [1,2], with the main functional feature being mitral valve prolapse (MVP) [3] and consequent mitral regurgitation (MR), our understanding of MMVD and MVP has deepened over the last decades—in particular, due to the finding that the MV annulus is a complex three-dimensional (3D) saddle-shaped structure [4]
We focus on mitral annular disjunction (MAD), a specific anatomical abnormality associated with MVP characterized by a distinct separation between the mitral annulus and the left atrial wall and the basal portion of the posterolateral left ventricular (LV) myocardium
It is evident that the presence of a MAD in patients with MMVD has both functional and clinical implications and contributes to an increased incidence of ventricular arrhythmia and sudden cardiac death (SCD)
Summary
Since the recognition that mitral valve (MV) leaflets can be affected by myxomatous degeneration (myxomatous mitral valve disease (MMVD)) [1,2], with the main functional feature being mitral valve prolapse (MVP) [3] and consequent mitral regurgitation (MR), our understanding of MMVD and MVP has deepened over the last decades—in particular, due to the finding that the MV annulus is a complex three-dimensional (3D) saddle-shaped structure [4]. MMVD is more complex than isolated MVP, as the morphological changes involve various components of the MV apparatus, leading to impairment of the MV function. In this context, intrinsic mitral annular abnormalities are increasingly recognized and have clinical ramifications [8,9,10]. The morphological characteristics of MAD and imaging tools for diagnosis will be described, and the clinical and functional aspects of the coincidence of MAD and myxomatous MVP will be discussed based on two case studies
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