Ronald H. Wharton, MD

Disclosures

October 30, 2018

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Hello and greetings from Bronx, New York. This is Ronald Wharton. I am a cardiologist at Montefiore Medical Center and the Albert Einstein College of Medicine. I thought I'd share with you something that one does not see very often. I call this case "four of a kind."

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Here is the history. This is a 23-year-old man who was admitted because of chronic, progressive shortness of breath. Now, being that he's only 23 years old, it's reasonable to look at the right side of the heart, because when you have young people who have shortness of breath from a cardiac cause, one common place that one goes to is the realm of congenital heart disease. Perhaps there's something going on there. So let's look at the right side of the heart.

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This is a parasternal short axis at the level of the aortic valve. We're focusing right now on the color Doppler through the pulmonic valve. Doctors who treat congenital heart disease like the pulmonic valve much more than those of us who treat adults. You'll notice first of all that the rhythm is atrial flutter; the right ventricular (RV) systolic function looks pretty good. There is a fairly dense color jet of pulmonic regurgitation present, which is not common in a young person.

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In this slide, you can see the pulse-wave Doppler through the pulmonic valve. You'll notice that even though there's a lot of pulmonic regurgitation, the velocity through the pulmonic valve isn't that high. We're going to come back to that, but take a good look at that spectral tracing for a second; note that the peak velocity (PV) is somewhere a little more than 0.6 m/s. Take a look at the acceleration time through the valve.

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Here is the continuous Doppler, which demonstrates a pulmonic regurgitation jet that is probably moderate in severity. Take a look at the velocities as well. Any time you look at a Doppler signal, you need to look at the contour as well as the timing and the velocities.

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What has been shown so far? First, as previously mentioned, the patient has atrial flutter, which is a little unusual in a 23-year-old. We also see moderate pulmonic regurgitation; the end diastolic velocity of the pulmonic jet (or pulmonic regurgitation or pulmonic insufficiency jet) is about 2.5 m/s. That means, in end diastole, that there is a gradient between the pulmonary artery and the RV of about 25 mm Hg, or 4 x 2.52. So there's diastolic pulmonary hypertension, which means there is pulmonary artery systolic hypertension. There's a little pulmonic stenosis, but it's not significant. The RVOT [right ventricular outflow tract] velocity was only about 0.6 m/s. The velocity through the pulmonic valve is about 1.2 m/s, so a little bit of a step up in velocity but nothing to write home about. And despite the pulmonic insufficiency jet, the velocity flow through the pulmonic valve remains low. Why would that be?

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Well, if we look at this slide, which is a 2D color Doppler in the RV inflow view, you can see a very dense signal of tricuspid regurgitation (TR). If you look closely at the tricuspid leaflets, they do look a little thickened. In a young person, that's rather thick.

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You can see a dense TR signal in this continuous-wave Doppler through the tricuspid valve. There's a little diastolic TR at the end of diastole in each beat—that's from atrial relaxation because the underlying rhythm is atrial flutter.

So, what are we dealing with?

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Well, there's severe TR. What would cause that in a young person? He's young for a carcinoid tumor, and the leaflets are a little thickened, but they're certainly not frozen stiff, which is what you'd expect in a carcinoid valve. In addition, we have atrial flutter, which is very, very unusual. So there are a lot of things going on here with the pulmonic and tricuspid valves that are not quite normal for a 23-year-old person.

While we're in the parasternal short axis, let's take a look a few other images.

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Here is the zoomed view of the aortic valve. The aortic valve looks to be trileaflet; however, it is very thickened for a young person.

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This slide shows the continuous-wave Doppler through the aortic valve. You can see here that the PV of the flow through the aortic valve approaches 4 m/s.

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In this slide, you can also see that there is a moderate jet of aortic regurgitation. I'm saying "moderate" because I'm comparing the density of the regurgitation flow to the density of the systolic flow.

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What do we see so far? We see a thickened trileaflet aortic valve with moderate aortic stenosis and moderate aortic regurgitation. What could be causing that? If we were to entertain something like carcinoid (which I don't think we would), we could always see if there's a PFO [patent foramen ovale]; this would account for a carcinoid tumor if there were an intra-cardiac shunt that allowed flow from the right side of the heart to the left side of the heart.

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But as you can see here in this subcoastal view, there is no color-flow across the inter-atrial septum.

Here's a hint: We have stayed pretty much in the parasternal short-axis view. I'm going to show you a couple of other views in the parasternal short axis, looking at the mitral valve both in the 2D color and M-mode.

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In this slide, we can see a 2D and 2D color view of the mitral valve. You will notice that there is a posteriorly directed jet of mitral regurgitation. You can see the aortic regurgitation jet impinging upon the interior mitral leaflet during diastole. If you look at the mitral valve itself, what do you see there? You can see that the movement of the anterior mitral leaflet is somewhat restricted, and movement of the posterior mitral leaflet seems to be nonexistent. In the next slide, there are some additional findings which one doesn't often hear about nowadays because very few people focus as much on M-modes as they used to way back when. But I still like them.

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Here is an M-mode through the left ventricle (LV) in the parasternal long axis. You'll notice that in early diastole, there is what is referred to as an exaggerated early diastolic dip. What is that about? Well, that means that the filling of the LV is occurring a little later than the filling of the RV, and therefore the septum has an exaggerated dip into the LV in early diastole.

There are two things that can cause this. One cause is significant aortic regurgitation if the aortic regurgitation jet is impinging on the anterior mitral leaflet and preventing it from opening. But there is another thing that causes it.

Here's the punchline.

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Here is the M-mode through the mitral valve. If you've had any basic education in M-mode echocardiography, you will recognize that this is a famous M-mode image.

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This is an M-mode of rheumatic mitral stenosis. You can see here the "hockey stick" appearance of the anterior mitral leaflet and the immobility of the posterior mitral leaflet. You can also notice how thick the aortic valve is, although we touched on that in the parasternal short axis before.

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In this apical four-chamber view, you can see again that the posterior mitral leaflet is frozen, and the anterior mitral leaflet is thickened and doming.

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In this slide, you can see both the color jet of mitral stenosis and posteriorly directed mitral regurgitation, which is exactly where rheumatic mitral regurgitation is supposed to go.

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Here you can see the continuous-wave Doppler through the mitral valve that demonstrates a very significant mitral stenosis. The mean gradient here is almost 15 mm Hg at a heart rate of 75 bpm. Some of that gradient is from the mitral stenosis, and some of that gradient is from the regurgitant volume coming back through the mitral valve during diastole. Either way, the gradient is the gradient, and that's going to make someone feel very, very short of breath.

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In this slide, you can see the TR as well. Looking very closely at the 2D image on the left, you can see that the tricuspid leaflets do have some thickening and dome a little bit; the tricuspid valve has some rheumatic involvement as well.

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In summary, I thought this was a really interesting case because this is a young person who seems to have rheumatic involvement of all four valves. We often see rheumatic mitral valve disease. We often see rheumatic mitral valve disease in combination with rheumatic aortic valve disease. Sometimes, we see rheumatic tricuspid involvement, but involvement of the pulmonic valve is pretty unusual, and so I thought this was fun.

I hope you enjoyed this. This is Dr Ron Wharton from Bronx, New York, at the Albert Einstein College of Medicine, for the heart.org at Medscape Cardiology. Thanks for listening.

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