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HCM: Apical

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In this next case, we see a 64-year-old female who was sent

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to cardiac MRI to look for infiltrative cardiomyopathy.

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She had a known history of apical hypertrophic cardiomyopathy and presented

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with new chest pain and other problems that led them to believe that she might

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have additional infiltrative cardiomyopathy.

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That part of the study ended up being

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negative, but it's a nice example of apical HCM.

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So, on this first set of cine images, what we see here is that there's relative

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sparing of hypertrophy in the base of the left ventricle.

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You can see these areas of the wall are normal in thickness,

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whereas the mid cavity and the apex in particular are quite thick.

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Let me scroll down through this slower.

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You can see really marked thickening of the apex of the left ventricle.

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So the key teaching points here are, when

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you see apical hypertrophy that looks symmetric, that's oftentimes apical HCM.

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This is a subtype of HCM that's actually more common in Asian populations and can

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actually be associated with an apical aneurysm as well.

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In this case, we don't see an apical aneurysm.

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That's something you certainly need to rule out and comment on in your report.

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One thing to note about apical HCM, unlike regular HCM,

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is that the wall thickness requirements are a little bit different.

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In regular HCM, you want to have a wall

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thickness of approximately a 15mm or greater. In apical HCM,

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sometimes the wall thickness may not quite meet 15mm in the apex,

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but as long as the wall thickness of the apex is one and a half times

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greater than basal segments, that still qualifies for apical hypertrophic

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cardiomyopathy. So I just want to show you

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some more images of this typical case of apical HCM.

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You can see again, the basal segments are

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normal in thickness, whereas the apical segments are quite hypertrude.

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And you even have cavity obliteration at the end of systole.

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This is very common for these patients.

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When we go on to the short access images,

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you can see there's this marked thickening of the septum, anterior wall,

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lateral wall and inferior wall with the cavity obliteration.

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And then finally, when we go to the late enhancement images,

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what's common for people with apical HCM is they actually can have quite extensive

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enhancement at the apex where they're involved.

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And you see that in this patient,

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quite a lot of enhancement, very bright, often a lot more bright and kind of stands

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out a lot more than your typical HCM cases.

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I'm not sure it's really known why

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that is, but that is a very typical appearance for apical HCM.

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So the key findings here,

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apical hypertrophy, more so than the base, extensive enhancement in the apex,

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and this is a very typical appearance for apical hypertrophic 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

Congenital

Cardiac

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