Guinness Book of Echo II

A few weeks ago I promised to show you a few more spectacular echoes - so-called record breakers in respect of the severity or uniqueness of pathologies. Well, here are some more cases that are difficult to beat. This is the first one:
Pinhole stenosis
When you look at this patient's aortic valve you can see that aortic stenosis is already present. The valve is severely calcified. One can neither discern the leaflets nor see motion of the valve.

Short-axis view of the severely calcified aortic valve.

It was quite difficult to get a good CW Doppler spectrum of transvalvular flow. We finally achieved it by obtaining a right parasternal view. This is the maximum velocity we found:

Right parasternal CW Doppler signal of aortic stenosis.

7.2 m/s! This is very high. The maximal gradient is 208 mmHg while the mean gradient is 157 mmHg. Velocities exceeding 6 m/s would be very unlikely, even in severe stenosis. As the patient's systolic pressure was 140 mmHg, we concluded that pressure in the ventricle must be almost 300 mmHg! By the way, he was symptomatic and received an aortic valve prosthesis shortly after we established the diagnosis.
Big gap
Severe mitral regurgitation is no uncommon finding. But the following patient was unique in two ways: he had very severe regurgitation and one could actually see the coaptation defect on the 2D image. Here is a magnified view of the valve:

Zoom view – apical long-axis view.

A coaptation defect can be easily detected in the presence of a flail leaflet. It is also frequently discovered when assessing tricuspid regurgitation. However, I can't recall any patient with such a wide gap between the leaflets in the setting of mitral annular dilatation.
The color Doppler study shows the severity of mitral regurgitation:

Very severe mitral regurgitation on a four-chamber view.

The vena contracta was 1.2 cm! Usually we consider MR to be severe when it exceeds 0.7 cm. The patient had a large anterior infarct and a remodeled left ventricle. Also take a close look at the position of the leaflets towards the end of systole. There is a rather large “tenting area” beneath the valve. This is not pure annular dilatation but also leaflet restriction as an additional mechanism of mitral regurgitation. What did we offer the patient? As his left ventricular function was poor, we performed a MitraClip procedure to reduce mitral regurgitation.
Huge sack
Compared to the entire vascular bed the heart holds a rather small quantity of blood - about 200 ml. The following echo clearly contradicts this statement. This patient with anterior myocardial infarction. mitral regurgitation and a pacemaker has a grotesquely enlarged left atrium.

Huge left atrium; look how small the right atrium is in

The length of the LA was 147 mm (normal < 50mm) and we determined a left atrial volume of 721 ml. Considering the fact that his left ventricle and right atrium are also enlarged, the entire heart probably contains more than a liter of blood! Also note the “smoke” in the left atrium. With so much blood in the left atrium it is no wonder that his blood flow was slow.
Extreme backflow
One way to assess aortic regurgitation is by looking at flow reversal in the ascending aorta with the help of PW Doppler (suprasternal view). When aortic regurgitation is severe one finds holo-diastolic flow as well as a maximal retrograde flow velocity at end-diastole. It is about 0.3 to 1.0 m/s at most. Flow reversal is very prominent in the following patient. The velocity at end-diastole was 1.2 m/s, thus being just a little less than forward flow (1.5 m/s).


Flow reversal (retrograde flow) in the descending aorta in a
patient with very severe aortic regurgitation.

Here is the corresponding color Doppler study:

Color Doppler study (three-chamber view) showing very
severe aortic regurgitation.

The regurgitation jet fills the entire left ventricular outflow tract. This is undoubtedly very severe aortic regurgitation. What was its cause? The TEE study gives you the answer:

TEE study (short-axis view). The aortic valve is bicuspid and
shows vegetations. There is a hole in one of the cusps
and perivalvular abscess formation.


The patient has aortic valve endocarditis with leaflet destruction of the bicuspid valve, cusp perforation, and a large aortic root abscess. Since he was hemodynamically unstable we immediately sent him for aortic valve replacement and root reconstruction.
If you want to see more record-breaking echoes, here is the link to the first part of “Guinness Records”:

Do you have similar spectacular images or videos? If yes, we would be glad to post these. Not merely to amaze readers, but because these cases can be very instructional.
Please feel free to forward this post to friends you think would be interested. After all we want to spread the word that 123sonography is THE platform to learn and expand your skills in echocardiography.