AssessmentsMitral valve disease
Mitral valve disease
USMLE® Step 1 style questions USMLE
USMLE® Step 2 style questions USMLE
A 43-year-old man with a history of rheumatic fever comes to the primary care clinic for a check up. Cardiac examination reveals a late systolic crescendo murmur with midsystolic click best heard over the apex and loudest just before S2. Which of the following maneuvers will cause an earlier onset of the click/murmur?
The mitral valve has two leaflets: the anterior leaflet and the posterior leaflet. Together, they separate the left atrium from the left ventricle. During systole the valve closes, which means blood cannot do anything but be ejected out of the aortic valve and into circulation.
If the mitral valve doesn’t completely shut, blood can leak back into the left atrium; this is called mitral valve regurgitation. During diastole, the mitral valve opens and lets blood fill into the ventricle. If the mitral valve doesn’t open enough, it gets harder to fill the left ventricle; this is called mitral valve stenosis.
Let’s start with mitral valve regurgitation. The leading cause of mitral valve regurgitation, and the most common of all valvular conditions, is mitral valve prolapse. When the left ventricle contracts during systole, a ton of pressure is generated so that the blood can be pumped out of the aortic valve; therefore, a lot of pressure pushes on that closed mitral valve. Normally, the papillary muscles and connective tissue, called chordae tendineae or heart strings, keep the valve from prolapsing, or falling back into the atrium.
With mitral valve prolapse, the connective tissue of the leaflets and surrounding tissue are weakened; this is called myxomatous degeneration. Why this happens isn’t well understood, but it is sometimes associated with connective tissue disorders, such as Marfan syndrome and Ehlers-Danlos syndrome. Myxomatous degeneration results in a larger valve leaflet area and elongation of the chordae tendineae, which can sometimes rupture; this rupture typically happens to the chordae tendineae on the posterior leaflet, and can cause the posterior leaflet to fold up into the left atrium.
The click is a result of the leaflet folding into the atrium and being suddenly stopped by the chordae tendineae. Although mitral valve prolapse doesn’t always cause mitral regurgitation, it often does. If the leaflets don’t make a perfect seal, a little bit of blood leaks backward from the left ventricle into the left atrium, causing a murmur.
The mitral valve prolapse murmur is somewhat unique in that when patients squat down, the click comes later and the murmur is shorter, but when they stand or do a valsalva maneuver, the click comes sooner and the murmur lasts longer.
This happens because squatting increases venous return, which fills the left ventricle with slightly more blood; this means that the left ventricle gets just a little bit larger. Therefore, the larger leaflets have more space to hang out, and as the ventricle contracts and gets smaller, it takes just a little longer for the leaflet to get forced into the atrium. Standing, on the other hand, reduces venous return, meaning there’s a little less blood in the ventricle and, by extension, a little less room to hang out; thus, the leaflet gets forced out earlier during contraction. The other heart murmur that follows this pattern is the one present in hypertrophic cardiomyopathy.
So, in addition to mitral valve prolapse, another cause of mitral regurgitation is damage to the papillary muscles from a heart attack. If these papillary muscles die, they can’t anchor the chordae tendineae, which then cause the mitral valve to flop back and allow blood to go from the left ventricle to the left atrium.