0:00

And so the next Case Case five is going to be a 69

0:03

year old woman with a history of sudden cardiac death.

0:06

And so the station came in with this

0:09

contrast enhanced chest CT and we

0:12

can see several findings on this contrast CT so we

0:15

can see this moderate size left plural effusion. You can

0:18

see the entirety of the left lower lobe

0:21

is atic a little portion.

0:24

Of the lingulas also have some relaxation atelectasis

0:27

and then kind of focusing in on the

0:30

heart. You can see that the overall hard size

0:33

is big but there's quite a bit of wall thickening and

0:36

I'm often careful about calling wall thickening

0:39

on a non-gated CT scan just because

0:42

again, you want to look for wall thickening

0:45

at in diastole and it's very hard

0:48

to appreciate when is in diastole

0:51

on a non-gate CT scan. So you mean overall thickening

0:54

that actually isn't there because the image was obtained

0:57

at systole.

0:58

But in this case, you can pre-see pretty

1:01

extensive wall thickening and the wall thickening seems

1:04

to be distributed more towards the apex of

1:07

the ventricle as opposed to the base. The basal lateral wall

1:10

here seems less affected than the apex of

1:13

the ventricle.

1:15

So this patient went on to have a cardiac MRI,

1:18

so we have two cine steady-state 3

1:21

procession sequences here. So we have a vertical long

1:24

access or a two-chamber view on the left.

1:27

We have a four-chamber view on the right.

1:29

And so again, the whole purpose of this sequence is

1:32

to look at contraction globally regionally to

1:35

look at the valves as well. So look

1:38

at anatomy and morphology of the ventricles. And

1:41

so in this case, you can see that the contraction actually looks

1:44

pretty good. If anything that contraction is squeezing more

1:47

than typical. So the chamber is

1:50

essentially obliterated here at the Apex.

1:53

The other thing to look at is the valve seen normal. So this is

1:56

going to be the mitral valve the tricuspid valve. So I'm not

1:59

really seeing much in the way maybe a little bit of tricuspid insufficiency

2:02

here.

2:04

When we're looking at morphology of The ventricle.

2:07

You can see that wall thickening that really seems to be confined to

2:10

this apical region.

2:13

So you have this apical wall thickening that

2:16

relatively spares the basil portions

2:19

of the left ventricle.

2:21

You can see that same apical wall thickening on this two

2:24

chamber view with this obliteration of the cavity.

2:28

So we have three different slice levels

2:31

of the short axis images of the steady state

2:34

free procession kind of starting at the base going to

2:37

the mid midventricle and then more at the

2:40

Apex.

2:41

This really nicely shows how the wall thickening kind

2:44

of changes. So contraction wise this looks

2:47

you know hyperdynamic with obliteration of

2:50

the cavity both centricular cavity at insistly, especially

2:53

at the Apex.

2:55

I don't see any Regional wall motion abnormalities. Everything seems

2:58

to be contracting normally and then in terms

3:01

of wall thickness again, you want to measure that at in diastole.

3:05

And the best time to measure it is going to be on these short

3:08

axis images so you can see quite Exquisite wall

3:11

thickening mainly in involving these mid and

3:14

apical segments of the left ventricular. Myocardium.

3:20

now we've injected gadolinium contrast you pick that

3:23

inversion time so that the normal myocardium is black

3:26

and now we're looking to see do we see any Lake edelenium

3:29

enhancement images

3:31

And so these are short axis images and so

3:34

kind of starting out at the base of the left ventricle. You can

3:37

see most of this myocardium looks black.

3:40

There's quite a bit of artifact here these bands that are kind

3:43

of extending, you know through The myocardium you have to subtract that mentally

3:46

from your mind and then as we

3:49

get to these areas of thick and myocardium, we start seeing

3:52

quite a bit of enhancements notice how it isn't

3:55

really following a vascular territory.

3:58

Here you can see more enhancement kind of

4:01

towards the Apex again. Not really following a vascular

4:04

territory. It's involving kind of all portions, but it

4:07

really seems to be localized to those areas of all thickening.

4:11

And more enhancement here at the Apex.

4:17

This is a short axis image. These are

4:20

a few different Lake yellow and enhanced images to start

4:23

two chamber view of four chamber View and then I'll float

4:26

track view again showing that enhancement really not

4:29

following a vascular territory sparing the southern

4:32

accordion in some areas, but really primarily affecting

4:35

those areas of thick and myocardium

4:39

And so what is a distribution of hypertrophy in

4:42

this case? You think it's asymmetric septal concentric

4:45

apical midventricular or

4:48

mass-like?

4:55

And so this is characteristic of the apical variance

4:58

of hypertrophic cardiomyopathy where you

5:01

have the speed-like configuration. So it's

5:04

referred to as a spade-like configuration just refers

5:07

to one of the playing cards and you can see it with

5:10

contraction. It really resembles that Spade because the distribution

5:13

of hypertrophy is really at the Apex.

5:16

So which country has the highest prevalence of apical

5:19

hypertrophic cardiomyopathy the US

5:22

South Africa Brazil or Japan?

5:26

So one country in particularly really tends

5:29

a large percentage of

5:32

their hypertrophic cardiomyopathy patients are from this this

5:35

variance. And so it's actually Japan is a

5:38

correct answer. So about 25% of

5:41

cases of Holcomb in Japan or this apical variance.

5:45

And so this is the apical variant of hypertrophic cardiomyopathy.

5:48

I remember Holcomb is going to be an

5:51

autosomal dominant trait with variable penetrance. The apical

5:54

variant is more common in Japan and there are

5:57

several negative prognostic indicators that are helpful to report

6:00

with cardiac MRI this refers to all of hypertrophic

6:03

cardiomyopathy. So if you have any of these

6:06

different variant or different features, it's a

6:09

negative prognostic indicator. Meaning those patients will

6:12

do worse. And so

6:14

The measurement of wall thickness matters. So if you have

6:17

over 30 millimeters of wall thickness, that's a negative prognostic

6:20

variant indicator. If you have a big gradient across

6:23

that sub aortic stenosis

6:26

like we showed earlier

6:28

if your ejection fraction starts falling so most Holcomb

6:31

at least initially is going to have hyperdynamic ejection

6:34

fraction as a disease Progressive the EF may

6:37

start to fall and then if you have the more

6:40

Lake edelenium enhancement that you have so this patient

6:43

our last whole compassion actually had quite

6:46

a bit of Lake idling enhancement. Those would all be negative prognostic

6:49

indicators.

6:52

These are the different variants that you may see with Holcomb. So

6:55

people can be normal. The most common is

6:58

going to be this basal septal variance with also attractive

7:01

obstruction, which we saw earlier. You can have concentric Holcomb.

7:04

You can have mid ventricular hypertrophy.

7:08

You can have asymmetric septal variants without

7:11

also attractive obstruction. You can

7:14

have the apical variants Mass like and then non-contiguous. So

7:17

you've seen a couple different variants of this just realize

7:20

that there are many variants of Holcomb and we

7:23

tend to image a lot of the or atypical

7:26

variants at least with cardiac MRI.