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Restrictive Cardiomyopathy: Idiopathic

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This next case is an 83 year old female with dyspnea on exertion

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who ended up getting an echocardiogram that showed normal left ventricular

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function but really large atrial. And she eventually got sent to a cardiac

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MRI to look for an infiltrated cardiomyopathy as an explanation for her

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heart failure symptoms. So what we see on this initial set of static

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images on the cardiac MRI is that this patient has really,

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really dilated atrial, out of proportion to the left ventricular and right

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ventricular dilation. This is a pattern highly suggestive of restrictive

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cardiomyopathy. And certainly we know from her history, that fits her clinical

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diagnosis as well. So when we look at cine images of this same

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patient, we see that there's preserved left and right ventricular systolic

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function. The left ventricle here, there's a little bit of blurring at end

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systole. But I would say it's normal to low normal systolic function.

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And then you see it, there's both mitral and tricuspid regurgitation based

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on these regurgitant jets through the valves. And then you see that these

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atrial are massively dilated. And that there's also

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blood swirling in the atrial. And whenever you see this blood swirling on

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cardiac MRI, that really tells you that it's just not moving very fast.

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So there's a lot of stasis. If the blood starts swirling,

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that means that there's basically saturation of the blood that's happening

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because it's moving so slowly through the image.

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And so anytime you see that, you know that this patient has poor

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function in whatever chamber it's that you're looking at. This patient also

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had atrial fibrillation that accounts for some of the fuzziness. And one

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way you can often tell if there's atrial fibrillation on cardiac MRI is

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you don't see that nice atrial kick. You should see a little squeeze

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of the left atrium right before systole. And in this case,

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you really just don't see that. It's sort of just static,

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the left atrial wall throughout the cardiac cycle.

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Now when we go to the short axis cine images, I'll just show

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a representative slice, you're gonna find that the left ventricular function

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here actually looks pretty good. And right ventricular function as well.

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I think if you had only this image, you might not really say

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there's a whole lot wrong with this patient. And this is again really

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typical of restrictive cardiomyopathy. You have preserved left ventricular

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function with really abnormal atrial morphologically. Now when we move on

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to the late gadolinium enhancement images, what we're really looking for

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here is some sort of enhancement that might explain why this patient has

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a restrictive cardiomyopathy. And remember, restrictive cardiomyopathies

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are caused by increased stiffness of the ventricles. And that stiffness

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leads to the atrial having to work harder to push blood into the

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ventricles. So although the ventricles have normal systolic function, they

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squeeze fine. They don't relax normally. So the diastolic function is abnormal.

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And so we're looking for diseases that could be causing that diastolic dysfunction.

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And unfortunately in this case, we really didn't find anything. You see

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that there's really no late enhancement in the ventricles on this view,

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the four chamber view. And then if we go to a two chamber

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view, similar, nice dark uniform nulling of the myocardium. And then finally

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when we go to the stack of short axis views, you can see

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that again, there's really nice uniform nulling of the myocardium, no late

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gadolinium enhancement identified that would explain the restrictive cardiomyopathy.

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Now in some later cases, we're gonna go through other etiologies that can

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explain restrictive cardiomyopathy and show their enhancement pattern. But

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in this case, if you were reading this, you would have to say

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this is a patient who has restrictive cardiomyopathy features,

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but with no late gadolinium enhancement to suggest any scarring or infiltrated

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disease to explain her restrictive cardiomyopathy.

Report

Faculty

Stefan Loy Zimmerman, MD

Associate Professor of Radiology and Radiological Science

Johns Hopkins Medicine Department of Radiology and Radiological Science

Tags

Myocardium

MRI

Idiopathic

Cardiac

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