0:00

Okay case number two.

0:02

Is a 25 year old man

0:05

who presents with chest pain we'll go through this still images

0:08

first.

0:09

Physical examination is remarkable for ectopia

0:12

lenses. Can everyone see the screen

0:15

here? So 25 year old man ectopiolentus presence

0:18

for a chest pain presence for chronic examination going to

0:21

show some still images and some then some movies.

0:24

This is a oblique sagittal view showing

0:27

the ayota Groot arch in the

0:30

listening aorta.

0:31

This is a short axis view of the aortic valve the aortic route

0:34

and the valve.

0:36

This is a three chamber view showing the asynngiota in

0:39

the root mitral valve left ventricle left

0:42

atrium.

0:44

once again three images

0:46

public sagittal View

0:49

Toyota View and an lvu now I'm

0:52

going to go to some of the videos on

0:55

this the same images which are in video format.

0:58

Okay, this is the

1:00

Public sagittal view I think we're going to keep this

1:04

image playing while we answer the questions the correct diagnosis.

1:07

There's four five choices marfans by casparatic

1:10

Valve.

1:11

louis-daxyndrome turn a synome and Noonan

1:14

syndrome

1:15

cast the audience to take a take a

1:18

walk and the correct answer, please.

1:20

Okay, good. Excellent. This is Martin syndrome. The key

1:23

features here are your dilatation

1:26

of iot crude and effacement of the signature blood

1:29

Junction notice the Tulip shape configuration of the iot Groot

1:32

and proximation. The valve

1:35

was bicuspid. Go back

1:38

to the Val moving in a minute. The other key feature

1:41

here was the fact that there was natural valve

1:44

prolapse. I'm going to play the video was the

1:48

video playing. Yeah. So this is the three chamber view notice that

1:51

the natural valve is a mix of mattress and prolapsing.

1:54

The aortic Groot is dilated tulip-shaped iotic root

1:57

effacement of the cyanotubular junction March around

2:00

prolapse key features of Marfan syndrome in

2:03

a young man with another feature of Mumford syndrome,

2:06

which is ectopian lenses.

2:08

It's a track has been valve. The other other choices

2:11

are wrong. Lowest dates syndrome is characterized by

2:14

arterial tortuosity. Turn us syndrome is

2:17

not characterized by these findings. Yes aneurysms

2:20

can occur. But typically it's smaller vessels

2:23

iot computations, which are known in syndrome is

2:26

fact that harmonics Nelson, hypertrophy, myopathy.

2:29

The next question pertaining to this case is

2:32

regarding surgical repair. I'm just

2:35

going to

2:36

step back for a few seconds and allow

2:39

the audience to read these questions and

2:43

we'll go to the poll and then discuss the right answers.

2:46

So there's five choices here as to when surgery should be

2:49

performed for Math and syndrome and nonmarf and aortic disease.

2:58

Surgical treatment of asymptomatic patients with

3:01

degenerated should be concerned diameter exceeds 5.5. So the

3:04

correct that is the correct answer. The question here was what is

3:07

false. What is not true?

3:09

the correct answer to this question is e

3:13

which is

3:15

operative intervention to replace that excise or replace acing aorta.

3:18

It is a confusing question. I apologize either excuse me,

3:21

asyncote has indicating patient black husband balance the diameteries

3:24

4.5. So the general

3:27

rule.

3:28

5.5 is considered the cutoff for non congenital heart

3:31

disease and non-genetic or connector

3:34

tissue disorders for repair of the Yota when not

3:37

a company by valuable disease 5.5.

3:39

Five is the cutoff in patients who have Marfan syndrome

3:42

or connective tissue disorders a lower

3:45

lower threshold is used for more dangerous conditions.

3:48

Particularly lowest diet syndrome where 4.4 to

3:51

4.6 is used as a threshold for surgery.

3:54

If excuse me in asymptomatic

3:58

patients with degenerative asenergic

4:01

aneurysms.

4:02

Repair is considered when the diameter exceeds 5.5 centimeters

4:06

or 5.5 the cutoff for surgery for degenated by

4:09

utilities five centimeters from offense syndrome. However,

4:12

the caveat here is that a lower

4:15

threshold should be considered if there's a family history of sudden

4:18

death due to iot disease rapidly progressing

4:21

in dying each other or this content

4:24

value disease. So they're eating valve needs to be

4:27

a place where you can sufficiency and the Asian yoga has only 4.5 the

4:30

surgeons may go ahead and replace the Ace in the order because

4:33

the valve needs repair anyway, and this is a progressive disease.

4:36

So five centimeters from often syndrome, except if

4:39

it's rapidly progressing family history of certain death due tomorrow

4:42

friends pregnancies another condition where earlier search

4:45

surgical repair can be considered. So it's a general rule

4:48

of thumb one can remember that 5.5 for degenerative

4:51

iotic aneurysms five form often syndrome,

4:54

but the threshold can be decreased in specific circumstances

4:57

rapid increase in size family history of

5:00

sudden death you telling disease

5:02

Open content valve literacies.

5:06

There is one more question pertaining to to this

5:09

case, which is

5:11

All the following genetic conditions associated with iot aneurysms,

5:14

except or dissections except there's one of

5:17

all this four diseases in here which associated with

5:20

biotic aneurysms and dissections except

5:23

one of them.

5:24

Okay. Yes, correct. Seems like a very educated audience

5:27

here William syndrome is characterized by arterials stenosis

5:30

typically pulmonics stenosis and super valuetics stenosis

5:33

all other conditions associated with aneurysms and

5:36

dissections. So the

5:39

correct so the correct answer is E Williams syndrome not

5:42

associated aneurysm Associated arterial stenosis.

5:45

so

5:46

to summarize this case Martin syndrome. It's also

5:49

more dominant.

5:50

complications most common complications progress of

5:53

iot growth annotation

5:55

and a basement of the signature of the junction annular eucharectation surgical

5:58

repair is recommended when the aotic route or any part of

6:01

the aorta Region's five centimeters from often syndrome.

6:03

And in the absence of genetic conditions

6:06

in general for atherosclerotica utpcs

6:09

5.5 centimeters is the cutoff for thoracity aneurysms

6:12

with regards to surgery.

6:16

Any questions on this case, then we could go on to the next case.