0:02

Hello and welcome to noom Conference hosted by MRI Online.

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0:28

Today we're honored to welcome Dr.

0:29

Christy Pomerance for a lecture entitled A Beginner's Guide

0:33

to CT for Coronary Artery Anomalies in

0:35

the pediatric population.

0:37

Dr. Pomerance completed her residency at Weill Cornell

0:40

and her pediatric radiology fellowship at Children's

0:43

Hospital of Philadelphia before returning

0:45

to Weill Cornell in 2018, where she's currently on staff

0:50

as an assistant professor.

0:51

At the end of the lecture, please join her in a q

0:53

and a session where she will address questions you may

0:55

have on today's topic.

0:57

Please remember to use the q

0:58

and a feature to submit your questions so we can get to

1:01

as many as we can before our time is up.

1:03

And with that, we are ready to begin today's lecture. Dr.

1:06

Pomerance, please take it from here.

1:08

Hi, good afternoon everybody. My name's Kristi Pomerance.

1:11

Um, and as they mentioned,

1:12

I am a pediatric radiologist at Cornell.

1:15

Um, and today I'm going

1:16

to be talking about coronary artery anomalies in

1:20

children on ct.

1:21

Um, so we're gonna go, uh,

1:23

I am primarily a pediatric body radiologist,

1:26

but I do read, um, pediatric cardiac ct.

1:30

Um, so hopefully this will be a good introduction.

1:32

Um, I know it's something that a lot

1:33

of people are not comfortable reading,

1:35

don't have a lot of experience with.

1:37

So we're gonna do an overview.

1:39

We'll talk about the CT technique we use at our institution.

1:42

We'll review normal coronary artery anatomy.

1:45

We're gonna go over some

1:46

of the d big disease categories you're looking at,

1:48

and we're gonna show, um,

1:49

some interesting cases from our institution.

1:53

So the gold standard

1:54

for evaluating the coronary arteries in both adults

1:57

and children is cardiac CTA, and that is a CTA that is gated

2:01

or timed for the cardiac cycle.

2:03

And cardiac CTA has been found to be even superior

2:06

to invasive or congen conventional angiography

2:09

as well as MRI.

2:11

And I know pediatrics is a bit of a niche field,

2:13

and pediatric cardiac imaging is

2:15

even more of an niche field.

2:17

But, um, as our, uh, cts are getting better and better

2:21

and faster and faster, even regular cts that are not gated

2:25

and not for cardiac purposes, um, you can see some

2:28

of the cardiac anatomy on.

2:29

So for example, this is a teenager

2:31

who came into their RER, um, for trauma.

2:34

And this is a regular CT chest.

2:36

It is not gated, it is not a cardiac ct.

2:38

And you can see the coronaries on here.

2:40

Um, so these are things that are coming up more

2:43

and more even on regular ct

2:44

and additionally even coronary anomalies that we see.

2:47

These are coronary anomalies that we also see in adults

2:50

and might not present until adulthood.

2:52

And the same imaging features

2:54

and clinical considerations

2:55

that we apply in children also apply to adults as well.

2:59

Um, so hopefully even if you're not a pediatric radiologist

3:02

or you don't even read cardiac imaging, um,

3:04

you'll still find something useful in this talk,

3:07

um, for the future.

3:08

So anomalous coronary arteries are found in about one to 2%

3:12

of the population in the literature.

3:14

Um, but this is based on autopsy studies.

3:16

So this is actually thought to be a underestimation

3:19

because, um, it's only catching a portion, um, of the, um,

3:24

of the people with anomalous coronaries.

3:27

And so the real estimate they believe can almost be

3:29

as high as 5%.

3:31

And it's something that's very difficult

3:33

to diagnose clinically

3:34

because there's such a broad range of presentations.

3:37

So some people with anomalous coronaries are

3:39

completely asymptomatic.

3:40

They go their whole life without even knowing they have it.

3:42

Others present with more mild symptoms such

3:45

as chest pain or palpitations.

3:47

And then there are people who present with cardiac rest

3:49

and sudden cardiac death.

3:50

And sudden cardiac death in athletes is associated

3:53

with coronary anomalies up to 17% of the time.

3:57

And it's the second leading cause of cardiac arrest

4:00

and sudden cardiac death in athletes.

4:02

And it's specifically the anomalous aortic origin

4:05

of a coronary from the opposite sinus

4:07

with an intraarterial course.

4:08

That's the second leading cause

4:10

of sudden cardiac death in this population.

4:12

And an active US military who have sudden cardiac death

4:15

who have gone to autopsy autopsy studies show

4:18

anomalous coronaries are responsible in about 25

4:21

to 27% of those cases.

4:24

Um, so most of these events of sudden cardiac death

4:27

and cardiac arrest occur between the ages

4:29

of 10 and 30 years old.

4:31

It's more common in males

4:32

and it's most associated with basketball followed

4:35

by soccer track and field swimming and cross country.

4:38

So really high intensity endurance sports,

4:40

not strength training sports like weightlifting.

4:43

Um, and for those of you who are sports fans,

4:45

this is Sharif O'Neal.

4:46

This is Shaquille O'Neal's son.

4:48

He played UCLA, uh, he played BAS college basketball

4:51

for the UCLA Bruins

4:53

and he actually underwent surgical correction

4:55

for anomalous coronary in 2018 so he could continue playing.

4:59

Um, and that was publicly released by the family.

5:01

And this is Bronny James, who is LeBron James' son.

5:04

Um, last year he suffered a cardiac arrest in New Jersey

5:07

while playing basketball.

5:09

He subsequently underwent, um, surgery in LA, I believe

5:13

for a congenital issue.

5:14

And they haven't publicly said what he he has,

5:16

but a lot of cardiologists have publicly speculated

5:19

that it was also for repair of an anomalous coronary.

5:22

Um, so how do we scan, um,

5:24

how do we scan the coronaries in kids?

5:27

So the goal of, um, the CT is to decrease cardiac motion

5:31

as much as possible.

5:32

And how do we do this? Well,

5:34

we do this using a faster scanner.

5:36

Um, we can do this by decreasing the heart rate

5:38

with medications such as beta blockers.

5:40

And we can also do this with cardiac gating.

5:43

So cardiac gating triggers a scan

5:45

during very specific part of the cardiac cycle.

5:47

Usually the stillest,

5:49

well hopefully the stillest part of the cardiac cycle.

5:51

So the patient must be hooked up to the EKG leads.

5:54

So the CT can be done in conjunction

5:56

with the EKG and the heart rate.

5:58

So retrospective gating is when we scan throughout the

6:02

entire cardiac cycle.

6:03

And as you can imagine, this is a large amount of radiation.

6:05

It's equivalent to getting multiple cts at once.

6:08

So we really try to avoid that in children.

6:10

So we do prospective gating where the scan is timed

6:15

for a very specific part of the cardiac cycle.

6:17

We acquire all the images we can during that time.

6:20

If we don't acquire the whole scan, it waits

6:22

until the next heartbeat and require acquires the rest

6:24

during that next phase.

6:25

In the cardiac cycle, in patients less than 70,

6:28

or in patients who have a heart rate less than 70 beats per

6:31

minute, this is done during diastole.

6:33

This is usually most adult patients in, um, heart rates

6:37

that are over 70 beats per minute.

6:38

This is usually done in systole

6:40

and that's most of our pediatric patients.

6:42

We scan cranial coddle from head to toe

6:45

sedation is given on a case by case basis.

6:47

It really depends on the age and the mentation of the child,

6:50

but we usually do have to give sedation

6:51

for children younger than five years old so

6:54

that they can remain as still as possible

6:56

in unsedated patients who can follow instructions,

6:59

we do a breath hold and in sedated patients

7:01

where we're trying to evaluate the coronaries

7:03

or very small structures, we actually ask for intubation

7:06

and we do a breath hold by turning off the VE

7:08

for a few seconds while we scan.

7:11

So heart rate control.

7:12

So for cardiac cts in adults,

7:15

heart rate control is almost always given,

7:17

but we actually rarely give it in kids.

7:19

We actually rarely give beta blockers

7:21

and we almost never give nitrates.

7:22

We have given beta blockers a few times to older,

7:25

more adult sized teenagers.

7:27

Um, if the kid is in, uh, sedated

7:30

and pediatric anesthesias present,

7:32

occasionally we'll ask them to give rate

7:34

control as they deem safe.

7:35

But the point is is that most

7:37

of our scans are successfully done at much higher heart

7:40

rates than the adult cardiac studies are.

7:42

Um, and so another, um, important feature

7:45

of a cardiac CT done for coronaries is good timing

7:48

of the contrast in the coronary arteries

7:50

and things that can affect the contrast.

7:52

Timing include age and size, heart rate anatomy.

7:56

As you see here, this kid has a left-sided SVC.

7:58

That might time that

8:00

that might change the timing of the contrast.

8:02

Any cardiac shunts in any delay in scanning, we try

8:06

to use a right antecubital IV injection.

8:09

We bolus track

8:10

and we trigger the scan off

8:12

of the descending thoracic aorta at the level

8:14

of the Karina at a hundred hounds field units.

8:16

I know other places will do a test injection

8:18

and then time the scan based off that test, test,

8:21

test injection, we use Omnipaque three 50 followed

8:24

by saline flush and contrast dose is determined by scan time

8:28

and patient size for injection rate.

8:30

Um, a lot of times in children we're very limited by

8:33

what size IV we're able to get, but alienates

8:36

and infants, we try to go at one to two ccs per second.

8:39

And little kids, we try to go to three

8:40

to four to ccs per second.

8:41

And in adult sized teenagers we go five ccs, five

8:45

to six ccs per second, which is, you know, more similar

8:48

to an adult cardiac scan.

8:50

Okay, so let's review normal coronary artery anatomy.

8:53

So the coronary arteries arise from the aortic sinuses just

8:57

below the synott tubular junction.

8:58

There's usually two separate ostia.

9:01

There is a right coronary artery arising from the right

9:04

sinus of el salva or right cusp.

9:06

And there's a left coronary artery arising from the left

9:09

sinus of el Salva and cusp here.

9:11

And then there's also a posterior

9:14

or non coronary sinus,

9:15

which usually gives off no vessel whatsoever.

9:18

The left main coronary artery then divides into the LAD,

9:21

the left anterior descending and the left circumflex artery.

9:24

Occasionally it also gives off a third branch called the

9:26

ranis intermediates artery.

9:28

And in most people, usually there's a right dominant system.

9:31

What we mean by that is that the RCA curves around the back

9:35

of the heart to supply the posterior descending artery.

9:39

Coronary arteries are defined by the area of distribution

9:42

that they supply and not by their origin.

9:44

So the LAD is defined as courses

9:46

through the anterior intraventricular group.

9:49

It supplies the anterior intraventricular septum

9:52

and it gives off diagonal branches.

9:55

The left circumflex artery courses in the left AV groove

9:59

supplies the left ventricular free wall

10:01

and gives off obtuse marginal branches

10:03

and the right coronary artery courses in the right AV groove

10:06

supplies the right ventricular free wall

10:08

and gives off acute marginal branches.

10:11

And there are numerous normal variants that can occur

10:14

with the coronary arteries.

10:16

Many of them are listed here

10:17

and many of them that you see on imaging.

10:19

Frequently, we will discuss a high takeoff

10:21

of the coronary arteries, which is something

10:23

that we often see in children.

10:25

So the big disease categories you're looking

10:28

for in kids when it comes

10:29

to coronary artery anomalies are anomalies of origin,

10:32

meaning that the coronaries come

10:34

off from the wrong location.

10:35

Anomalies of course, meaning

10:37

that the coronaries have an abnormal course

10:39

and anomalies of termination, meaning

10:40

that the coronaries communicate

10:42

or terminate with adjacent structures

10:44

that they're not supposed to, such as cardiac tumors,

10:47

systemic vessels or pulmonary vasculature.

10:50

You can also think of these disease

10:51

categories in two other ways.

10:54

Um, there's one category which is benign

10:56

or non hema like significant.

10:58

These are things that we see on imaging,

11:00

but they might not clinically affect the patient

11:02

and they might not need any intervention

11:04

or surgery, whereas we have hemodynamically

11:07

and significant anomalies that are dangerous

11:10

for the patient can lead to serious outcomes

11:12

and may need intervention surgery.

11:14

And then finally I have a third category in my brain

11:17

that I think of is that things

11:18

that cause dilated coronaries in children.

11:20

And that includes, um, p uh,

11:23

coronaries coming from the pulmonary arteries,

11:25

congenital fistulas,

11:26

and then coronary artery aneurysms,

11:28

all of which we'll discuss.

11:30

So the main questions you wanna answer

11:32

with cardiac CT when you're doing a coronary artery

11:35

anomalies, do the coronaries have a normal origin?

11:38

If not, do they arise ECT topically from the aorta

11:42

or do they arise from a structure,

11:43

another structure other than the a, the aorta?

11:47

Does the coronary artery have a normal or abnormal course?

11:50

Does the coronary artery have any abnormal connections

11:53

to adjacent structures such as the cardiac chambers,

11:56

pulmonary arteries, or systemic circulations?

11:59

And are the coronary arteries dilated?

12:02

So we're gonna jump into cases now.

12:04

The first batch of cases we're gonna talk about, um,

12:07

involves anomalous aortic origin of the coronaries.

12:10

So this occurs, the left main coronary can arise from the

12:13

opposite sinus as it does here or from here

12:17

or the the left main

12:19

or the right, uh, coronary artery can arise from

12:21

the opposite sinuses.

12:22

The left main or the right coronary artery can also arise

12:25

from the posterior or non coronary sinus.

12:28

Or you can have a single coronary artery

12:30

arising from any sinus.

12:32

Um, and these also can have numerous, um, courses.

12:36

So you can have a pre pulmonic course of the coronary

12:38

where it runs anterior to the main pulmonary artery.

12:41

You can have a retro aorta course where it runs

12:44

behind the aorta.

12:45

You can have a transseptal course where it dives down

12:48

and runs in the intraventricular septum.

12:51

And these are all benign courses.

12:52

But the first one I wanna focus on is this interarterial

12:55

course where the coronary comes off from the opposite sinus

12:59

and travels between the aorta

13:00

and the main pulmonary artery as you see here.

13:03

Um, and this term, this course is actually the one

13:06

that is termed malignant.

13:07

Well, why is it termed malignant?

13:09

Because this is the course that's associated

13:12

with an increased risk of sudden cardiac death.

13:15

So an interarterial right coronary artery is more common

13:18

than an interarterial left main coronary artery.

13:21

But it seems and both are at risk for sudden cardiac death.

13:24

But an interarterial left main coronary artery is more

13:27

highly associated with sudden cardiac

13:28

death and cardiac arrest.

13:30

And there's two theories about why this is the case.

13:33

The first is that exercise causes expansion

13:36

of the aortic root

13:38

and the pulmonary artery,

13:39

which then can compress the artery.

13:41

But this theory is controversial and not well accepted.

13:45

The more prevalent theory is that most of these cases

13:48

of an intra arterial course actually have an intramural

13:51

segment where this coronary runs within the aortic wall.

13:55

And that portion, that intramural segment is hypoplastic

13:58

and easily compressed and, uh, compressed

14:00

and occluded by the aorta.

14:02

Intramural features include a slit light orifice like we see

14:06

here, an acute angle of takeoff between the coronary

14:10

and the aortic wall, and an elevated height to width ratio.

14:14

You can see that this coronary is being

14:15

squeezed in the vertical direction.

14:19

This is just a nice diagram from this paper showing normal

14:22

takeoff of coronary with a nice obtuse angle

14:25

with the aortic wall.

14:26

And you can see that as the coronary travels longer

14:29

and longer distances within the aortic wall,

14:32

there's a greater acute angle of the, um, coronary

14:35

with the aortic wall and the segment becomes more

14:38

and more narrowed, as does the o.

14:42

So this is our first case.

14:43

This is a 16-year-old who, uh, was completely healthy,

14:47

had no medical history

14:48

and experienced a cardiac arrest during basketball practice.

14:51

So all the cases I'm gonna show you start

14:53

with an axial CT of the chest.

14:55

So these are the great arteries.

14:56

This is the aorta that's well pacified,

14:58

and this is the pulmonary artery.

15:00

And as we scroll down, we can see there is normal takeoff

15:03

of a left main coronary artery from the left sinus,

15:06

but there is abnormal takeoff

15:08

of a right coronary from the left sinus coursing

15:11

between the aorta and the pulmonary artery.

15:13

And you can see that this has a very slit like osteum.

15:16

It has an acute angle of takeoff with the aor

15:19

with the aortic wall,

15:20

and it is very narrowed in that, um, interarterial segment

15:24

before it, um, runs into the AV groove

15:27

and has a more normal configuration.

15:31

And here are orthogonal.

15:33

This is a closeup orthogonal image of the RCA as it runs in

15:37

between the aorta and the pulmonary artery.

15:39

And you can see how narrowed

15:40

and slit, like it's, it has an increased height width ratio.

15:43

These are curved replan or reformats of the coronaries.

15:47

And you can see that the l the normal left main coronary

15:50

artery is nice and wide open at its osteum

15:53

and has no areas of narrowing versus

15:55

that anomalous right coronary artery,

15:57

which has a very slit like osteum

15:58

and has this long segment of narrowing,

16:00

which is highly suspicious for an intramural course.

16:05

This is a companion case.

16:07

This is a 7-year-old with chest pain.

16:09

Um, again, this is the aorta here

16:11

and the main pulmonary artery.

16:12

You can see there's a normal takeoff

16:14

of the left main coronary artery from the left sinus.

16:17

And there is an anomalous takeoff

16:19

of the right coronary artery from the left sinus,

16:21

again coursing between the aorta and the pulmonary artery.

16:25

This one is mildly narrowed at its osteum and proximally,

16:29

but not, probably not as greatly

16:31

as the last case I showed you.

16:32

And again, here are orthogonal images just showing

16:35

that it is very slightly probably, uh, squished

16:40

or narrowed in that, um, intraarterial in

16:43

that intraarterial region.

16:45

So this is more equivocal for an intramural course,

16:47

but this still has an intraarterial course.

16:50

And again, here's a nice picture of that.

16:52

Um, anomalous right coronary artery.

16:54

Again, it does have an acute angle of takeoff

16:56

with the aortic wall, um, and maybe slight mild narrowing.

17:00

And that's proximal portion.

17:01

And here's a curved replan or reformat of that artery.

17:07

This was a 14-year-old with syncope

17:11

and again, we have the aorta, the pulmonary artery.

17:14

This is an axial ct.

17:16

This time we have a normal takeoff

17:18

of the right coronary from the right sinus of El Salva here,

17:22

but we see

17:24

that we do not have a normal left main coronary artery

17:26

arising from the left sinus.

17:28

Instead, the left coronary artery,

17:30

left main coronary artery is arising from the right sinus

17:33

of salva just to the right of

17:35

that inter coronary commissure.

17:37

And again, having an interrater course between the aorta

17:40

and the pulmonary artery.

17:42

And here it's here. And again, we have an acute angle

17:45

of takeoff, we have mild narrowing,

17:47

we have a slip like osteum.

17:49

And then in that intra arterial segment we have a very

17:53

narrowed or ovoid configuration of the, um, coronary artery.

17:57

Again, suspicious for an intramural segment.

17:59

We can see that the left main coronary artery more distally

18:02

after it exits that intramural

18:04

and intra arterial region, um, demonstrates more rounded

18:07

and more normal configuration.

18:09

So this was an interarterial left main coronary artery

18:12

with an intramural segment.

18:14

So why is it important to detect these?

18:17

Um, because, um, we do surgery for these.

18:20

So the surgical technique

18:21

that's preferred is a coronary on roofing where they take

18:24

that intramural segment that's running in the aortic wall

18:27

and they actually make a slit in it to open up that segment

18:30

with the aorta and hopefully create a neo osteum

18:33

or a new opening in the more normal or orthotopic location.

18:37

Um, but there's two reasons

18:39

why you might not be able to do that.

18:40

The first is if the coronary runs to close

18:43

to the aortic valve commissures, if the, if the, if the um,

18:47

coronary artery crosses at that level.

18:50

So this is always something that we like

18:52

to report in our report in our dictations

18:54

and tell the surgeons about.

18:55

The other reason you might not be able to do

18:57

that is if the intramural segment is too short.

19:00

Um, and so this is another thing we do is we always measure

19:03

how long the intramural segment is

19:05

and report it in our dictations.

19:07

So if you can't do a coronary

19:08

and roofing, then they have to do a coronary reimplantation

19:10

where they actually take the coronary off

19:12

and they sew it back in,

19:14

in the orthotopic or normal location.

19:16

Um, but this comes with more long-term complications,

19:18

which is why coronary and roofing is referred.

19:22

Okay, so now let's talk about some

19:24

of the more benign variants

19:25

of an anomalous aortic origin of the coronary.

19:29

Um, so this is a case, this is a 23-year-old with chest pain

19:33

and here we have a normal right coronary coming from the

19:35

right sinus, but we see we have an anomalous left coronary

19:38

coming from the left sinus.

19:40

And at first it almost looks like it's interarterial

19:43

because it's traveling between the aorta

19:44

and maybe the pulmonary artery.

19:47

But if you look more closely, this um,

19:49

vessel is actually the left main coronary artery is diving

19:52

down, it's swooping down,

19:53

creating what's called a hammock sign.

19:55

And if you look on the sagittal image, you see that this,

19:58

um, left main pulmonary artery is actually traveling within

20:02

the intraventricular septum.

20:03

So instead of being up here

20:04

where an interarterial artery travel,

20:06

it's traveling down here.

20:08

So this was actually a transseptal course.

20:10

This is more common with the LAD in the

20:12

left main coronary artery.

20:13

Um, this hammock sign has been described to de to um,

20:17

describe, you know, when it dives down into the

20:19

intraventricular septum

20:21

and this is when the coronary, um, uh, travels

20:24

below the level of the pulmonary valve in the

20:26

intraventricular septum.

20:27

So how do you tell the difference between an interarterial

20:30

and a transseptal course?

20:31

In a transseptal course, the arteries surrounded

20:34

by septal myocardium, whereas in,

20:36

in an inter arterial course the artery surrounded

20:38

by aortic wall or epicardial fat in a transseptal course,

20:42

the artery course downward creating

20:44

that hammock sign we talked about.

20:46

And it travels below the level

20:48

of the pulmonic valve like we see here

20:50

on the sagittal image.

20:52

And an interarterial course, the artery travels

20:55

above the level of the pulmonic valve,

20:58

the artery In a transseptal course,

20:59

the artery does not have an oblong or slit like appearance.

21:02

And in an inter arterial course,

21:03

the artery may have an oblong

21:05

or slit like appearance if it has an intramural course.

21:09

Another benign variant that we see is a pre pulmonic course.

21:13

So this was a 23-year-old with tachycardia

21:16

and an anomalous left coronary seen on a PE study.

21:19

Again, hearkening back to the fact that we're starting

21:21

to see more and more coronaries on our non cardiac imaging.

21:26

Um, and this was a follow-up

21:28

cardiac CT done on this patient.

21:30

So again, we see a well pacified aorta in the

21:32

main pulmonary artery.

21:33

And this time we actually have a single coronary arising

21:36

from the right sinus of El Salva.

21:38

And you can see there's no left coronary coming off from

21:41

the left sinus.

21:43

And if we trace it out, we see

21:44

that there is a normal right coronary artery,

21:47

but we also have this additional vessel,

21:48

this left main coronary artery coming off from the single

21:51

osteum and coursing in a pre pulmonic fashion anterior

21:55

to the main pulmonary artery here.

21:57

And we can see it here on this 3D image.

22:00

And so this was a pre pulmonic left coronary artery.

22:04

This is more common with the left main

22:06

coronary artery than the right.

22:07

And we actually see this with tetrology of fallot.

22:10

It's associated with tetrology of fallot.

22:13

Um, so this was a five-year-old, um,

22:16

who was undergoing a presurgical workup for, um,

22:19

before a pectus excavatum surgery

22:22

and a coronary artery anomaly was incidentally found.

22:26

Um, so again, we see a well opacified aorta

22:30

and this is the main pulmonary artery.

22:32

We see normal takeoff of a right coronary

22:36

here arising from the right sinus.

22:38

But when we look at the left sinus,

22:40

we do not see a left main coronary artery coming off.

22:43

But what we do see is we see a left main coronary artery

22:46

coming off from the right sinus here

22:50

and coursing posteriorly, retro aortic behind the aorta

22:55

and then giving off the LAD in the circumflex.

22:58

And again, you can see that here and here.

23:00

So this was a retro aortic left main coronary artery.

23:04

Um, a retro aortic course is more common

23:06

with the left main coronary artery than the right.

23:09

And again, it's totally benign.

23:10

You don't have to do anything about it.

23:11

But it is important to know if this patient ever goes on

23:14

to get aortic valve surgery.

23:18

A single coronary artery is a very extremely,

23:21

extremely rare um, anomaly.

23:23

It is when you have a single aortic root osteum

23:26

with no additional ectopic

23:27

or atretic osteum, it can be associated with other types

23:31

of congenital heart disease.

23:32

And I just wanted to show you guys this very rare case we

23:35

had in a very young child.

23:37

They did an echo and they said we don't see a left main

23:40

coronary artery anywhere.

23:41

We don't even see it, you know, distally

23:44

and we're not sure sure where it's coming off of.

23:46

So we did a cardiac CT and again, here's the aorta

23:49

and we see a single coronary artery

23:51

and it's very large in size, especially

23:54

for this very young patient, um,

23:55

coming off from the right sinus.

23:57

And if you follow it around,

23:58

it's go traveling normally within the right AV groove,

24:02

but it's coming around the back of the heart

24:05

and it's actually giving off the left circumflex

24:10

And the left anterior descending artery as well.

24:13

So this was a very rare case

24:15

of a single coronary artery feeding the entire heart.

24:19

Osteo atresia is a companion case.

24:22

Um, some can be somewhat similar in appearance.

24:25

Um, but this occurs when coronaries become occluded in feal

24:28

or neonatal li uh, life.

24:31

It almost always involves the left mean coronary artery

24:34

and it's characterized by an atretic

24:36

or fibrous connection between the left coronary cusp

24:40

and the LAD and left circumflex.

24:42

There are usually prominent right to left collateral vessels

24:46

and there's usually a large conal branch coming from the

24:49

right coronary artery to supply the LAD,

24:52

which has a pre pulmonic course

24:54

and may mimic a pre pulmonic vessel.

24:56

And these patients usually will develop myocardial ischemia

24:59

later in life and eventually need some sort

25:01

of revascularization or bypass.

25:04

So this was a 2-year-old

25:06

who had an absent left main coronary artery on echo.

25:10

And we see as we scroll down, there is again takeoff

25:14

of a right coronary artery

25:15

and the right coronary artery is pretty big.

25:18

Could pretty good in size.

25:20

We can see that there is an LADA circumflex

25:23

and even a ramus intermediates artery,

25:26

but there is no left main coronary artery connecting it

25:29

back to the left sinus.

25:30

Um, you can imagine there's like a short segment here,

25:32

which you know, kind of should be there.

25:34

It looks like it almost made it there but not quite.

25:37

Um, and if we follow up that right coronary, we can see

25:40

that there is a large conal branch here coursing in front

25:43

of the main pulmonary artery connecting with a bunch

25:46

of collateral vessels here

25:47

and then feeding the left anterior descending artery here.

25:52

And again, here is that network of collaterals.

25:54

And here is that, um, conal branch feeding the left uh,

25:59

circulatory system.

26:00

So this was osteo atresia with a pre pulmonic vessel.

26:04

Okay, hi aortic takeoff.

26:06

Um, so this is one

26:07

of the normal variants I alluded to before.

26:09

This is actually one of the most common

26:10

thing we see in kids.

26:12

Um, it's much more common than the other, um,

26:14

anomalous aortic, um, uh, origins of the coronaries.

26:18

So the coronaries usually come off

26:19

below the OTT tubular junction,

26:22

but a hi aortic takeoff is defined as a coronary

26:24

that comes off five or even 10 millimeters

26:27

above the OTT tubular junction.

26:28

And it's benign, but it's important

26:30

to know about if the kid is ever getting cardiac

26:32

catheterization or cardiac surgery.

26:34

And it is much, much more common

26:36

with the right coronary than the left.

26:38

And a high takeoff of the right coronary is associated

26:41

with the bicuspid aortic valve.

26:44

So this was an 8-year-old with an anomalous coronary, uh,

26:47

right coronary on echo.

26:49

Um, they said, Hey, you know, we did this echo,

26:51

we really can't see where the right coronary is coming from.

26:53

We're worried that it might have a malignant course

26:55

or it might be coming off somewhere bad.

26:58

And so we did a cardiac CT

27:00

and you can see there is a normal left coronary arising from

27:04

the left sinus, um, of El Salva.

27:06

But we see this right coronary here, it has a normal course,

27:10

but as we trace it back, we see it goes up, up, up, up, up,

27:13

up, up and it is actually coming off from

27:15

the ascending aorta.

27:16

And here on this coronal image you can see that it's arising

27:19

above a level of the synott tubular junction.

27:21

So we said, hey, this is just a high aortic

27:24

takeoff of this coronary.

27:26

Um, this was a two week old, um,

27:29

with an echo done for murmur.

27:31

Um, they did the echo

27:32

and they couldn't see the right coronary artery very well

27:35

and in fact they were actually worried it was coming from

27:37

the pulmonary artery

27:38

because they could trace, almost trace it back

27:40

to the pulmonary artery, um, in which case

27:42

that would've required surgery.

27:44

And so they said, Hey, can you, we just wanna know

27:47

where the right coronary is coming off from.

27:48

That's our only goal from the ct.

27:50

And this is not a very pretty CT

27:52

'cause this is a very young child

27:53

with a very high heart rate

27:55

and we did it without any medication

27:57

but we ans we're able to answer their one question.

28:00

Um, so this is scanning from, um, cranial to coddle.

28:03

Okay, this is the aorta

28:05

and this is the main pulmonary artery here.

28:07

And as we scroll down we can see

28:09

that there's a very tiny right coronary artery here arising

28:12

from the aorta and not from the main pulmonary

28:15

artery as was suspected.

28:17

And then going down to the right AV groove

28:19

and having a normal course.

28:20

And here is a coronal image of that high takeoff

28:24

of the right coronary artery

28:25

above the synott tubular junction.

28:27

So again, we were able to say, hey,

28:28

this is just a high aortic takeoff

28:30

of the right coronary artery.

28:31

You guys don't need to do anything.

28:34

Okay, so now let's talk about dilated coronary arteries.

28:37

So speaking of coronaries

28:40

that come off from the pulmonary arteries, um,

28:42

there is something called the anomalous origin

28:45

of the left main coronary artery from the pulmonary artery.

28:48

It's also known, um, by the pneumonic Al Kappa.

28:51

Um, it's very rare, 90% of them present in infancy

28:55

with left ventricular heart failure

28:57

and only 10% of them are able

28:58

to reach adulthood without any surgery or intervention.

29:01

And interestingly enough, at birth,

29:03

the coronaries actually usually are not dilated.

29:07

Um, this is extremely rare.

29:08

So we don't see many cases of this.

29:10

But as in infancy, the as pulmonary resistance falls,

29:14

this causes shunting

29:15

or stealing from the coronaries to the pulmonary arteries.

29:18

And then you quickly develop very dilated coronaries

29:21

and collateral vessels in older children and adults.

29:24

There's also a companion to this called r kappa

29:28

anomalous origin of the right coronary

29:29

from the pulmonary artery.

29:31

And this is similar but much,

29:33

much rarer than the already very rare Al Kappa.

29:36

And these kids are actually usually asymptomatic

29:38

and at birth, again, the coronaries may not be dilated,

29:42

but um, as pulmonary resistance begins to fall in infancy,

29:46

this can again cause shunting or stealing

29:48

and cause markedly dilated coronaries

29:50

and collateral vessels.

29:52

So this was a fascinating case.

29:54

This was a 20-year-old presenting with fatigue

29:57

who somehow made it into adulthood

29:59

with Al Kappa not knowing that she had this.

30:03

Um, and I wanna draw your attention

30:04

to the main pulmonary artery here.

30:07

And if you look along the right side

30:08

of the main pulmonary artery, you see this blush

30:10

of un opacified blood coming from the right aspect

30:13

of main pulmonary artery.

30:14

And if you trace it back, you see a very, very large

30:17

and dilated left main coronary artery, um,

30:21

then giving off very large l uh, dilated LAD

30:24

and left circumflex arteries.

30:26

And this was a case of Al Kappa.

30:28

You can see that there is a normal origin

30:29

of the right coronary artery,

30:31

but this is also markedly dilated and tortuous.

30:34

You can see all of the coronaries are markedly dilated

30:37

and there are multiple collateral vessels.

30:39

So again, here's a 3D image showing that,

30:41

and here is an image showing that anomalous, um, origin

30:45

of the left main coronary artery from the pulmonary artery.

30:48

And this was a case of Al Kappa.

30:51

Um, this was a 7-year-old with palpitations.

30:55

Um, this kid underwent an echo

30:57

and was found to have dilated coronaries on echo.

31:00

Um, so the cardiologists were thinking, oh,

31:02

we wonder if this kid has coronary artery aneurysms from,

31:05

you know, Kawasaki or from Missy.

31:07

And so we did a cardiac CT

31:09

to further evaluate his coronaries.

31:11

And again, um, I'm gonna ask you to look at the,

31:13

the pulmonary artery here, the main pulmonary artery

31:16

and then along the right aspect of it, you see this blush

31:19

of contrast here

31:21

and then coming off, you can see this really massively

31:24

dilated right coronary artery, massively dilated,

31:27

tortuous right coronary artery.

31:28

And this kid had a normal origin

31:30

of the left main coronary artery here.

31:32

But you can also see that it's very, very dilated

31:35

and tortuous as well

31:36

and that there are multiple collateral vessels.

31:39

Um, and again, here is a reformat showing the takeoff

31:42

of the right coronary artery from the main pulmonary artery.

31:45

So this was a very rare case of r kappa

31:50

coronary artery fistula.

31:52

So, um, this is another entity

31:54

that can cause dilated coronaries in children.

31:57

So the normal coronary artery should terminate in the

31:59

capillary bed of the myocardium.

32:02

Um, but a coronary artery fistula occurs when the

32:04

termination of the coronary arteries

32:06

or its branches terminate in a cardiac chamber

32:08

or a low pressure vascular structure.

32:11

60% of these cases terminate in the right-sided heart

32:14

chambers and this can cause enlargement

32:17

and tortuosity of the coronary artery

32:19

or the branches due to shunting and steel phenomenon.

32:22

And a lot of these, and

32:23

although we do see it in kids,

32:24

they're often there asymptomatic.

32:27

So usually these present later in life in adults in the

32:30

fifth or sixth decade of life.

32:32

So this was a kid, um, a three-year-old with an echo done

32:35

for murmur and they incidentally said, Hey,

32:38

the left main corona artery looks really

32:39

dilated on this echo.

32:41

And again, they were worried.

32:42

They, they said, oh, is this a coronary artery aneurysm from

32:45

Kawasaki or from Missy that you know, we didn't know about?

32:48

So they asked us to do a CT to investigate further.

32:52

Um, and again, here's the main pulmonary artery.

32:54

Here is the aortic root.

32:56

And then as we scroll down,

32:58

we can see there is a very normal looking right coronary

33:01

artery arising from the right sinus of El Salva.

33:04

But we have a massively dilated left main coronary artery

33:08

coming off, um, normally from the left sinus,

33:12

but it is very, very dilated.

33:13

It's giving off what looks like a normal LAD

33:17

and a normal left circumflex artery,

33:20

but it is also giving off this additional vessel.

33:23

So it's very dilated. It's giving off this very dilated

33:25

additional vessel which is coursing retro aortic

33:29

behind the aorta.

33:30

And then finally terminating with the right atrium here

33:34

and you can see that there's contrast going from the vessel

33:37

into the right atrium.

33:38

And so this was actually a dilated sa nodal artery.

33:42

The sa nodal artery is a normal artery

33:44

that arises from the right coronary artery about 60%

33:48

of the time and arises from the left main coronary artery

33:51

about 40% of the time.

33:52

In this case, this anodal artery has had a coronary artery

33:57

fistula with the right atrium making it massively dilated

34:00

and making the left main coronary artery massively dilated.

34:04

So we were able to tell them, hey,

34:05

this isn't a coronary artery aneurysm,

34:07

this kid has a coronary artery fistula.

34:11

Um, so speaking of coronary artery aneurysms,

34:13

this is another, um, cause of dilation

34:16

of coronary arteries in children

34:18

and in pediatrics it's almost always caused

34:20

by Kawasaki syndrome

34:22

or by Missy, which is the multi-system inflammatory syndrome

34:26

in children after a COVID-19 infection.

34:29

Um, Kawasaki's disease is way more common than missi.

34:33

Um, and coronary artery aneurysms in both are actually going

34:36

down due to better recognition of Kawasaki's disease

34:39

and newer treatments, but also

34:41

with the newer co covid variants.

34:43

Um, it doesn't seem to be as associated as highly associated

34:47

with missi and Covid vaccination has also played a, uh,

34:51

has played a role in decreasing, um,

34:53

in decreasing the rates of Missy.

34:55

Um, so, uh, the coronary artery aneurysms in both tend

34:59

to be more proximal than distal

35:01

and the aneurysms may be ular or fusiform

35:04

and they may be multifocal

35:05

and they can be any size small, moderate, or giant.

35:09

And complications include thrombosis, stenosis,

35:13

occlusion rupture or later on calcification.

35:17

So this was a two-year-old who presented

35:19

with a fever and chest pain.

35:22

Um, and you can see that there are normal origins

35:25

of both coronary arteries.

35:26

There's normal origin of the right coronary

35:29

and there is normal origin of the left coronary.

35:32

But you can see that there are multifocal areas

35:34

of aneurysmal dilation, most notably involving the LAD here

35:38

where you can see on these replan reformats,

35:41

but also involving the left circumflex artery

35:43

and also multiple areas of, um,

35:46

aneurysmal dilation involving the right coronary artery.

35:49

So this was a case of Kawasaki's disease.

35:53

This was a 6-year-old who had a fever rash

35:57

and abdominal pain who presented in September of 2021

36:00

during the covid delta variant peak.

36:03

And we can see if we start the CT over from the beginning,

36:10

um, we can see

36:11

that there are normal origins of both coronaries.

36:14

The left comes off from the left sinus,

36:16

the right comes off from the right sinus.

36:19

You can see that there are multiple areas that

36:22

of massive aneurysmal dilation,

36:24

particularly involving the right coronary artery,

36:27

but also involving the LAD and the circumplex artery here.

36:30

Is that right? Corona with at least two

36:32

large aneurysms here.

36:34

And here's the left sided, um, coronaries.

36:37

So again, these were multifocal aneurysms in the setting

36:40

of Missy and you can see

36:42

that this patient was very, very sick at this time.

36:44

They were intubated and that they were in the ICU.

36:47

Um, so on imaging, um,

36:50

the coronary artery aneurysms between kasa in Kawasaki's

36:54

and Missy are pretty indistinguishable.

36:57

Um, to my knowledge there's really no features

36:59

that differentiate the two.

37:01

Um, so, uh, Kawasaki's

37:03

and Missy are often differentiated clinically.

37:05

So Kawasaki's disease is found in younger children,

37:08

usually less than five years old.

37:10

Um, left ventricular dysfunction is very uncommon.

37:13

It's really not associated with any GI symptoms.

37:15

Obesity is not a risk factor

37:17

and it seems to be more common in, um, people

37:20

of Asian ethnicity, whereas Missy tends to be, um,

37:23

found in older children.

37:24

Adolescents usually over six years old

37:26

and these kids are very, very sick.

37:29

They usually have, um, left vent,

37:31

left ventricular dysfunction from a myocarditis,

37:34

they can have really bad GI symptoms as well.

37:37

And um, it is associated with obesity

37:40

and it is found to be more common in people of Hispanic

37:42

and African American heritage.

37:45

So in summary, uh,

37:47

cardiac CTA can successfully be done in children

37:50

for coronary artery anomalies with prospective gating

37:53

and little to no medication or sedation.

37:56

Um, the most important coronary artery anomalies

37:59

to recognize because they require intervention

38:01

or surgery, um, include that anomalous aortic origin

38:04

of the coronary arising from the opposite cusp

38:07

with an intraarterial

38:08

and intramural segment as well as Al Kappa

38:11

and r Kappa, the left

38:13

and right coronary arteries arising from

38:15

the pulmonary arteries.

38:16

The other subtypes, um,

38:18

of anomalous coronaries are important to know about.

38:21

They're benign so they don't require intervention,

38:23

but they're important to know about if the patient is ever

38:26

getting any sort of chest or mediastinal surgery.

38:29

And other important causes

38:30

of coronary artery dilation in children include coronary

38:33

artery fistula, Kawasaki's disease, and Missy.

38:37

Um, so that is all I have. Thank you so much.

38:41

This is my email if anybody needs

38:43

to email me any questions or concerns.

38:46

Um, so I will open it up for questions now.

38:51

Thanks so much for sharing your lecture, Dr. Pomerance.

38:54

And yes, if you have a question, please place it in that q

38:57

and a feature so we can get through as many as we can

39:00

before we close our session.

39:04

So q and a at my

39:09

institution we use loron.

39:11

Okay, so the first question is my institution.

39:14

We use lomon 400 for, uh,

39:18

cardiac CTI in adults.

39:19

Is there a specific reason you don't use a more concentrated

39:23

contrast material or do you you

39:25

or do you just don't seem to need to?

39:28

Um, so we use omnipaque three 50 even in adults

39:33

at my institution.

39:34

Um, I don't know why we don't use LM on 400.

39:37

Honestly, I think we don't have it at my institution.

39:40

Um, I've never heard of anyone using it at

39:42

um, at, at Cornell.

39:44

I think we just have OMNIPAQUE 303 50

39:47

in the different dilution.

39:48

Um, so I started using omnipaque three 50 just

39:51

because that was what I was trained with

39:53

and that was what the adult people were using.

39:55

Um, I I'm not sure if there's any reason, uh, we've,

39:59

we've had, we've gotten good imaging

40:00

with OMNIPAQUE three 50, so, um, we haven't found

40:03

that we've needed a more concentrated contrast.

40:07

Um, excellent presentation. Thank you very much.

40:11

Um, yeah, we'll pause for a couple seconds.

40:13

Sometimes it takes a moment for questions

40:15

to come in, so Sure.

40:17

We might get a couple more.

40:25

One more question. Have you ever encountered a dominant

40:28

conal branch arising from the LAD

40:32

Dominic Conal branch rising from the led?

40:35

Um, no, I, I have not encountered that.

40:40

Um, I don't know if the adult,

40:42

I mean the adult people read a lot more cardiac cts than we

40:46

do, especially for coronaries.

40:47

Um, so, um,

40:49

I'm sure my adult colleagues probably have encountered that,

40:52

but I have not seen that in kids.

40:54

Please do use beta blockers in child cardiac CT frequently.

40:58

We do not. So, um, we, uh, I have talked

41:03

to the pediatric cardiologist about this

41:05

and most of our cardiac cts are done as an outpatient

41:09

on our outpatient scanners,

41:10

which are much better scanners than our inpatient scanners.

41:14

And for young children, we just weren't comfortable giving

41:16

beta blockers in an outpatient setting.

41:19

Um, so the only time I've given it to, um, outpatients

41:24

is in older kids who are really like almost adult patients,

41:27

like 17, 18 year olds, um, in, uh,

41:32

in younger children, in very young children

41:34

where we are sedating them, they're sedated,

41:36

they're intubated pediatric anesthesia's there.

41:38

And we are physically in the hospital

41:40

with the pediatric anesthesia team there.

41:42

I will ask them to give, be beta blockers as they,

41:46

as they deem safe because I mean, I have a whole team there.

41:49

Um, so I feel like it's safe to do that, um,

41:52

to lower the heart rate and usually those children

41:53

have very high heart rates.

41:55

Um, but that's the only time I give it.

41:56

Most of those scans that you saw were done as an outpatient

41:59

with no rate, um,

42:01

with no heart rate lowering, um, medications.

42:04

Um,

42:08

Awesome. I think you got

42:09

'em all. Okay.

42:10

Thank you guys so much and feel free

42:12

to email me if you guys have any other questions you don't

42:15

wanna put in the chat or you think of later.

42:16

Okay. Thank you for having me.

42:19

Of course, Dr. Pomerance

42:20

and thank you so much for your lecture today

42:22

and for everyone else for participating in this lecture.

42:25

We, we appreciate you being here.

42:28

You can access a recording of today's conference

42:30

and all our previous noon conferences

42:32

by creating a free MRI online account.

42:35

And be sure to join us next Thursday,

42:37

March 21st at 12:00 PM Eastern, where Dr.

42:40

Samir Rega will deliver a lectured entitled

42:44

Thora Columbar Spine Injury at ct,

42:46

A Systematic Search Pattern.

42:48

You can register for that@mrionline.com

42:51

and follow us on social media

42:53

for updates on future noom conferences.

42:55

Thanks again and have a great day.