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Mitral Valve Pathology Current Cardiology Reports (2019) 21: 61 https://doi.org/10.1007/s11886-019-1145-5 STRUCTURAL HEART DISEASE (RJ SIEGEL AND NC WUNDERLICH, SECTION EDITORS) Mitral Valve Pathology Gregory A. Fishbein1 & Michael C. Fishbein1 Published online: 23 May 2019 # Springer Science+Business Media, LLC, part of Springer Nature 2019 Abstract Purpose of Review This review describes numerous pathologic entities that cause structural abnormalities of the mitral valve. Different pathologic entities involve different components of the so-called mitral apparatus: atrial wall, annulus, leaflets, chordae, papillary muscles, and/or left ventricular free wall. These abnormalities can cause valvular stenosis, regurgitation, or both. Recent Findings Currently, in addition to open-chest surgery to replace or repair the damaged mitral valve, there are less invasive percutaneous approaches to address mitral valve dysfunction. These include narrowing the orifice, clipping the leaflets, and inserting bioprostheses percutaneously. Summary Understanding the structural abnormalities discussed in this review is essential for choosing the optimal therapeutic intervention for mitral valve disease. Keywords Mitral valve . Mitral regurgitation . Mitral stenosis . Endocarditis . Rheumatic heart disease . Valve repair . Myxomatous degeneration . Myxoid degeneration . Floppy valve disease . Mitral valve prolapse Introduction collagen-vascular diseases, ischemic heart disease, and heart failure to name the most common. Until recently, open-heart The structure of the mitral valve is unlike that of the other surgery with replacement of the valve was the only surgical cardiac valves. The mitral valve only has two rather than three option. More recently, open-chest and percutaneous repair of leaflets. The mitral valve has six components that affect its the valve can be accomplished. The pathology of the mitral function: the left atrial wall, an incomplete annulus, leaflets, valve discussed in this chapter will, to some extent, dictate the chordae tendineae, papillary muscles, and the left ventricular therapeutic approach. free wall. There are few causes of mitral stenosis, post-inflam- matory/rheumatic disease and congenital malformations being the most common. On the other hand, there are many struc- tural abnormalities of the components of the mitral valve that Mitral Valve Anatomy can result in mitral regurgitation. Dilation of the left atrium, annulus, or left ventricle; myxoid change of the leaflets and The structure responsible for modulating blood flow between chordae; and other changes in the leaflets or papillary muscles the left atrium and left ventricle is complex, and is not simply a can make the mitral valve leak. The causes of such changes “valve.” Indeed, the term “mitral apparatus” [1] has been aptly include congenital malformations, infection, numerous applied to this structure that actually has six components: the posterior wall of the left atrium, an annulus, two asymmetric This article is part of the Topical Collection on Structural Heart Disease leaflets, chordae tendineae, papillary muscles, and the left ventricular wall [Fig. 1]. Abnormalities in one or more of * Gregory A. Fishbein these structures may cause the valve to leak or become stenot- [email protected] ic. Left atrial dilatation can result in displacement of the pos- terior leaflet of the valve, resulting in regurgitation. Michael C. Fishbein The annulus, or valve ring, is an incomplete band of dense [email protected] collagen between the left atrium and ventricle that surrounds 1 Department of Pathology and Laboratory Medicine, David Geffen the posterior leaflet. There is no true annulus around the ante- School of Medicine at UCLA, 10833 Le Conte Ave, CHS 13-145, rior leaflet that is in fibrous continuity with the aortic valve Los Angeles, CA 90095, USA and aortic wall. The annulus is somewhat elastic and 61 Page 2 of 8 Curr Cardiol Rep (2019) 21: 61 Fig. 1 Mitral valve anatomy. a Gross photograph of the heart cut along the long axis of the left ventricle. The mitral apparatus consists of the posterior wall of the left atrium (LA), an annulus (blue dotted line), anterior (*) and posterior (**) leaflets, chordae tendineae (yellow arrows), posterior (P) and anterior (A) papillary muscles, and left ventricular wall (L). Other structures seen from this view include the right ventricular free wall (R), the aorta (Ao), and the interventricular septum (IVS). b View of the mitral valve from the left atrium (inflow surface). Conceptually, the anterior and posterior leaflets can be subdivided into three segments, A1 A2 A3 and P1 P2 P3, respectively. c View of the mitral valve from the left ventricle (outflow surface). The anterior mitral leaflet (A) is continuous with the aortic valve (AV), whereas the posterior mitral leaflet (P) attaches to the left ventricular wall distensible that allows contraction during systole. of endothelial cells on all surfaces. The fibrous continuity Unfortunately, this compliance also allows the ring to stretch between the anterior leaflet and the aortic root may be altered if the atrium or ventricle is dilated, contributing to regurgita- in a number of congenital malformations of the heart, partic- tion [2]. ularly transposition of the great vessels. The valve leaflets are The ring may also calcify, most often in patients with hy- victim to a variety of pathologic changes to be discussed. The pertension, renal failure, or certain connective tissue diseases. valve leaflets are tethered to the papillary muscles by string- Calcifications are more common in women, the elderly, and in like fibrous strands, the chordae tendineae. Most chordae at- association with obesity in women [3, 4]. This calcification tach to the edges of the leaflets, but other chordae attach to the can limit the contraction of the annulus resulting in mild mitral undersurface, or ventricular surface, of the leaflets. The regurgitation (MR), or the calcific nodules may rarely extend chordae subdivide and anastomose as they approach the valve onto the valve leaflets and cause valvular stenosis [5]. Atrial leaflets. Approximately half the chordae from each leaflet at- fibrillation is also more common in individuals with a calci- tach to each of the two papillary muscles. With age, the valve fied mitral annulus [4]. leaflets and chordae may be thickened by fibrous tissue and The two quite different valve leaflets are responsible for show some degenerative changes. anatomic closure of the valve orifice. The posterior leaflet is The two papillary muscles and underlying left ventricular longer and narrower than the anterior leaflet, which is more free wall are the muscular portions of the mitral apparatus. The “fan-shaped.” Accordingly, the anterior leaflet is more mobile papillary muscles arise from the apical to mid-third of the and swings between the septum during diastole, and the left ventricular wall. The posteromedial papillary muscle, as its ventricular free wall during systole. The posterior, longer leaf- name implies, takes origin adjacent to the posteroseptum, let, surrounds 2/3 of the annulus [6] while the anterior leaflet is while the anterolateral papillary muscle is not in close appo- responsible for the other 1/3. The leaflets have a layered struc- sition to the interventricular septum. There is considerable ture: a prominent layer of collagen, the fibrosa, a thinner layer variation in the structure of the papillary muscles: the anterior of basophilic extracellular matrix, the spongiosa, a layer with usually has one large “head,” while the posterior more often elastic fibers on the atrial side, the atrialis, and a covering layer has multiple “heads” [Table 1][7]. Curr Cardiol Rep (2019) 21: 61 Page 3 of 8 61 Table 1 Causes of mitral regurgitation Myxomatous degeneration Rheumatic heart disease Infective endocarditis Connective tissue disease Congestive heart failure Cardiomyopathy Papillary muscle dysfunction/rupture Drug-induced valvular disease Congenital malformations Myxomatous Degeneration Myxomatous disease of the mitral valve is reported to have an Fig. 3 Histologic section showing myxomatous degeneration of the incidence of 2.4–5%, and is the most common cause of mitral mitral valve. There is an increase in proteoglycans and subsequent regurgitation in older patients [8]. The gross abnormality con- fibrosis (*) in the valve tissue, as highlighted in blue by the Masson sists of elongation and stretching of the leaflets and chordae trichrome stain. This section also shows the muscle, staining red,ofthe left atrial (LA) and ventricular (LV) myocardium. Chordae tendineae tendineae. The valve may have a thickened, gelatinous, (CT) can also be seen scalloped appearance [Fig. 2].Theaffectedchordaeare stretched and attenuated, and are prone to rupture. syndrome and pseudoxanthoma elasticum [Tables 2 and 3] Myxomatous degeneration may cause superior displacement [15, 16]. of one or both mitral leaflets into the left atrium, referred to as mitral valve prolapse. Other names for this clinical entity in- clude Barlow syndrome, floppy valve syndrome, ballooning degeneration, billowing leaflet syndrome, and mucinous de- Post-inflammatory Mitral Valve Disease generation [9]. The histopathology is characterized by archi- tectural changes in the leaflets consisting of fragmentation of While there are many causes of mitral valve regurgitation, collagen with an increase in extracellular proteoglycans and there are relatively few causes of mitral stenosis. Very large subsequent fibrosis
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