0:00

This is a case. This is a few years

0:03

old. This was a little child at

0:06

the time and she had a

0:09

valve sparing ascending aortic

0:12

replacement for a prior aortic aneurysm.

0:16

She also has a familiar erotopathy. So

0:19

I'm going to give you that.

0:20

And she came for routine.

0:24

Follow up as they get to look at

0:27

the post-surgical as to

0:30

give us Post surgical assessment and also to make sure that her

0:33

remaining vasculature looked normal. We

0:36

try to put her in the Mr. Scanner, but she has

0:39

a PDA coil because she also had a

0:42

PDA.

0:43

And that little coil.

0:46

Costs so much artifact that we could

0:49

not put her in the MRI scanner so we scan her what CT?

0:57

I'm going to show it to you a couple more times.

1:02

And here's another run through that maybe

1:05

too fast. I'm going to do it by hand. Let me

1:09

stop it here. So we're coming here.

1:12

Um, you can see she has priority Branch

1:15

vessels.

1:16

We're going down.

1:20

There's one stitch in the Stitch is always this

1:23

hyperdense ring-like structure

1:26

around the order.

1:28

And if you do not contrast Imaging that

1:31

that Stitch quote unquote will be hyper dense

1:34

because it will have almost calcification because

1:37

it's a felt like structure so it will be hypertension run

1:40

contrast images, which is important because some

1:43

people may mistake that for Asura aneurysm

1:47

or something else and then going for

1:50

the down.

1:52

This is the takeoff of the white corn

1:55

area artery.

1:57

And here is the takeoff of

2:00

the left corn area artery and obviously here Anatomy is

2:03

a little bit challenging because she has a severe Pectus deformity

2:06

or hard is soft over to the left Hemi thorax.

2:09

This is done in left heart

2:12

enhancement. So the right

2:15

heart is not enhancing. This is

2:18

the right atrium. This is the right ventricle again. It's all distorted

2:21

due to her pictures deformity.

2:24

This is the left ventricle.

2:27

And then we have the right left atrium. Well in hands.

2:31

and

2:33

and that's it.

2:34

I'm going to run it one more time so to for you

2:37

guys to

2:38

look at it.

2:40

And have an idea of what's going on.

2:45

And then I would like to ask Ashley to bring

2:48

up the pool.

2:51

What is the most likely diagnosis?

2:55

is this

2:56

an aortic root aneurysm that this child

2:59

has

3:01

is this a normal expected post-operative appearance on

3:04

a valve sparing procedure?

3:08

Is this a pseudo-anneurysm?

3:11

or a mycotic aneurysm

3:13

Yes, wonderful. So now I have a few people who

3:16

actually think it's a normal appearance. I have a few people who think

3:19

it's a Surah aneurysm and

3:22

and one person thinks it's mycotic and Arizona. I

3:25

like the spread. I like that. This is that there's

3:28

sort of a spread of normal versus

3:31

pseudo aneurysm versus mycotic aneurysm

3:35

and I have to tell you so I read this case on a

3:38

Friday afternoon at 4 o'clock, and I don't

3:41

know how about you guys but you know, Friday afternoon 4

3:44

o'clock is not my best time,

3:47

but I looked at

3:50

it and I thought this is all weird and I was about to sign it

3:53

off as normal expected post-operative findings when

3:56

I stopped and I looked at it and I'm

3:59

like, wait a second. There are too many left side of Chambers.

4:02

because

4:04

remember I said that the white heart is not enhancing so you can disregard

4:07

all the right-hearted structure right atrium right

4:10

ventricle just forget about it because they're not enhancing.

4:13

But if you go to this image, there

4:16

are too many enhancing structures

4:19

on this image if we go from anterior this

4:24

This is the pulmonary. This is the beginning

4:27

of the pulmonary artery, right so we can even go

4:30

here because then we can forget this because that's not enhancing this.

4:35

is the aortic valve we see a little bit of the right coronary artery

4:38

here this

4:41

Is the left atrium this is the descending order.

4:44

But what are we going to do with this thing here?

4:47

So now the question is what is this thing?

4:50

So it sits between the left atrium and the ascending

4:53

era.

4:54

What is it? It has a weird shape.

4:59

Here it almost looks like a you don't double mushroom.

5:02

And where does it come from and what is it?

5:05

Where does it belong?

5:07

And if I if you look really closely.

5:12

There is a tiny connection a

5:15

tiny communication.

5:19

between that structure

5:21

And the left ventricular outflow track, so

5:24

this is the mitral valve.

5:26

Again, it's a little bit distorted due to the Pectus deformity.

5:29

Again. This is the left atrium mitral valve

5:32

left ventricle. You see the left ventricular myocardium and

5:35

you have this communication here to this mushroom like structure.

5:39

And I'm going to show you the corona because on the Corona it's

5:42

much more obvious.

5:43

There you go.

5:45

so left ventricle

5:47

aortic valve

5:50

open

5:52

and this additional quote unquote chamber contrast

5:55

to pacified just like the left ventricular

5:58

left aortic.

6:00

Left atrial and aortic structures now you

6:03

would say but why is it here? And why is it?

6:06

Well, why is it happening? Well, remember this

6:09

kid had a valve sparing ascending aortic

6:12

replacement and the valve sparing

6:15

surgery.

6:16

Spares the native aortic valve. So what the surgeon does

6:19

is they take a two graft.

6:21

And they imaginate the native valve and

6:24

do the Stitch at the

6:27

bottom off the native valve pretty much in the region of

6:30

the Native annulus.

6:32

But to keep it there and to Temporary fasten

6:35

the valve the tube graph they

6:38

do sort of they do place so

6:41

called pledges just to hold the graph down.

6:44

It's sort of temporary sutures.

6:47

And one of them must have been right here and

6:50

I talked to the cardiac surgeon actually did that case and he

6:53

said oh, yeah. I remember I put a big pledged

6:56

right there and I was wondering if that frail tissue of

6:59

this little kiddo will actually do well with the

7:02

legend.

7:03

And remember I also said that this child has an underlying

7:06

iropathy. She actually has a lower date syndrome

7:09

and the tissue of the

7:12

of the wall, the mural tissue of these

7:15

patients is just abnormal. It's just not

7:18

the normal.

7:21

stability and integrity as a patient

7:24

without familiar your top of these

7:27

do have

7:28

and so they're very susceptible to lack

7:31

of Integrity of their pledges of their

7:34

temporary stitches and it's actually something that our cardiac surgeons

7:37

have now become more and more aware. So when they do

7:40

these replacement surgeries and they put temporary stitches

7:43

they use actually very small ones not to

7:48

create large holes that are then

7:51

pretty much maintaining maintained

7:54

open when they do

7:57

these surgeries and this this was a quite a

8:00

substantial sudanisms seven by

8:03

four by three centimeter suda aneurysm that

8:06

this child had

8:09

And she underwent.

8:12

Surgery and had to have this excise and close and

8:15

I see that there's a question in the Q&A. Could you please oh,

8:18

this is Loyd diet

8:21

syndrome. It's

8:23

This is the name of the disease. It is

8:26

a familial erotopathy. It's in

8:29

the spectrum of the most common familial

8:32

aerotopathy that we know is marfan's disease

8:35

Lloyd Deeds syndrome is a

8:38

similar entity that has a

8:41

different genetic footprint and has a

8:44

much bigger impact on the vascularity because

8:47

these patients not only develop aneurysms of

8:50

the aorta, but they pretty much develop aneurysms from

8:53

tip to toe. So we screen those patients on an annual

8:56

basis most preferably with

8:59

MRI and we look at the circle of Villas the

9:02

neck the chest abdomen pelvis visceral arteries

9:05

Etc. But as

9:08

I mentioned this child had a PDA coil

9:11

and have a complete a blackout because

9:14

of the susceptibility artifacts so that we couldn't put her in the magnet.

9:17

but these are

9:20

very they're very very

9:23

how to say that they're they're very susceptible to

9:26

developing aneurysms and there are vessels are simply

9:29

abnormal.

9:31

Yeah, so this was a pseudo aneurysm my chronic

9:34

aneurysm. I like that. But remember I

9:37

also said that the kid was asymptomatic. They did not have

9:40

a fever. They did not have a white count. They did not have any pain

9:43

and it's unusual for Central mycotic aneurysm

9:47

to present without any of those clinical symptoms.

9:53

Obviously could that have been an infection and

9:56

then become a pseudoism aneurysm potentially, but

9:59

this is in the region of a

10:02

vascular Stitch done by the surgeon. So it's

10:05

the classic description of the pseudo aneurysm with the

10:08

small neck and then the mushroom that comes out at the

10:11

location of the Stitch, which is also something that I want to

10:14

submit to you.

10:15

It is really important to know what

10:18

surgery the patient had and how these surgeries are

10:21

done. I know it sounds crazy that we as Radiologists should

10:24

know how the surgeries are done. But I think that if

10:27

you understand what the surgeon did you can understand better

10:30

and scrutinize your Imaging better for

10:33

potential pitfalls of set surgeries because

10:36

valve sparing surgery will have different complications

10:39

then for example a valve

10:42

replacement surgery.

10:45

Or a Tube replacement of Justice ascending

10:48

order and and it's also important that

10:51

you know where the anastomoses are because that's the anastomoses

10:54

are the weak points. And you want to scrutinize those

10:58

at anastomosis really pay attention to

11:01

the Stitch Integrity Etc.

11:04

And then that's why I would submit to you in it's helpful

11:07

to know well for first of all what

11:10

they had done and what this actually means how that surgery

11:13

is performed.

11:15

This is a depiction of the valve sparing aortic replacement.

11:18

See how they cut out. The aneurysm. This is the clamped

11:21

Arch. This is the valve of the patient

11:24

and then they take out the buttons of

11:27

the coronary artery Osteo. So

11:30

these are the so-called buttons and they put the graph on

11:33

top of the graph is stitched at the very bottom of the valve ring.

11:37

And so the native self will remain in

11:40

there and see these are these stitches these Holdings stitches

11:43

these pledges. This is exactly the

11:46

Stitch that the cardiac surgeon who did

11:49

that surgery on that child that I just showed you thought that

11:52

was too big of a stitch and it tour a

11:55

little hole here and that's where the suda aneurysm arose off.

11:58

And then ultimately those coronary arteries are

12:01

reimplanted onto the graft and then the graft

12:04

is connected to the arch. So this is how a valsparing is

12:07

sending aortic replacement is done.