And the goal is to review
illustrative cases of undiagnosed CHD
presenting and adulthood and
all these patients present it
to the adult, the general Ecolab.
So you will see these
patients in the real world.
So there's a 19 year old male
with elevated blood pressure.
He was diagnosed with the AV at
age four and he was
having issues echoes routinely every year.
And then they all show that the
bicuspid valve is working well.
Then at age nine, he was diagnosed with
hypertension and it was put on Lisinopril.
He was athletic, otherwise healthy,
no other medical issues or whatsoever.
So here you can see it's physical exam.
So blood pressure 176 over 80 on the left,
160 over 82 on the right.
His BMI is normal normal credit upstrokes.
And upon auscultation he
had a systolic click in
keeping with the bicuspid valve
but no other finance.
So as usual he gets a TT.
And you can see here
is bicuspid aortic valves
are really nice-looking by
Ginsberg about fusion of
the right and left as we typically see.
Then when I put color Doppler on,
you can see that the valve
works completely normal.
Now we're going to move to
the suprasternal notch view
and then
the apical findings were completely normal,
so normal structure and normal function.
And this is what we see
on the suprasternal view.
And then at the end of the study
as we move to
the abdominal pictures, this is what we see.
So this is the abdominal aorta, PW Doppler.
So in light of this, what's the diagnosis?
Can generate uric stenosis,
coarctation of the thoracic aorta,
coarctation of the abdominal aorta or this
is artifact we're just sampling and
Branch Council, please go ahead and vote.
Very good. So most of you
said coarctation of the thoracic aorta,
the typical coarctation that
we that we see and you're
absolutely right. In here.
You can see is CT had near
interruption of the aorta.
So very tight coarctation,
as you can see with the arrow,
but you also see these very angry collaterals
as you try to bypass the obstruction.
So this is undiagnosed
coarctation that was very
severe in a patient
with bicuspid aortic valve.
So after very extensive discussion
regarding stenting versus surgery,
the patient decided to go for surgery.
And here you can see the,
the specimen on the left-hand side.
You can see the isthmus there, but does not.
When you look until you
look at the short actually,
you can see how tight the obstruction is.
So you can see all this,
this narrowing of the,
of the descending thoracic aorta in
the opening was actually pinpoints.
So this is near an interruption
of the descending thoracic aorta.
And here you can see what happens with
the patient received a graft.
And on the right hand side you can see
the post-op CT. You can see
this wildly paid and
large graft and there was
no gradient between
the leg in the arms anymore.
Well, do you mind going back
to the echo for just a sec?
There was a question about
was that really a bicuspid?
And so it would be, I think,
nice just to go through
that features once for sure.
So again, short axis view and then this is
one that sometimes this
can be very challenging.
So as you're scanning anything for
stenographers is important that
you pan up and down so
that you open all three commissures.
And statistically speaking,
the most common one would be
fusion of the right and left,
as you can see here, followed
by fusion of the right and none.
And then fusion of the left, none,
none is very uncommon.
And we joke that if you
think that is left to none,
you, you're wrong until proven otherwise.
So, but I think again, this is,
you can argue that this is
the so-called partial fusion.
Some people think that
there's no such a thing.
You either bicuspid, tricuspid.
But it can be a very hard and I think
that's the thing you just pan up and down.
And if you fear personal
view is not sufficient,
you just go to the subcostal.
So you just have to try to interrogate
all three commissures from different windows.
So now there's a different patient.
He is actually in the medical field.
There's a 28-year-old found to have
coarctation in a CT done for cancer staging.
And very similar stories known to have
a back Ginsberg valve with hypertension
that was, was managed medically.
And once again, you can see here
the suprasternal notch view and
very similar findings sometimes by 2D
can be really hard to
find that narrowing of the,
of the descending aorta.
And same thing with doppler
as you put color Doppler on.
It's very hard to see the aliasing
because the opening is pin points,
so you just have to keep that in mind.
That's why the CW is so important.
Sometimes you even do the CW
blindly trying to find that pinhole.
And when you have severe coarctation,
you typically see what you see on
the left where you see
this high velocity in
systole as you can see here.
But also you get this
very typical diastolic runoff,
which is a sign of
severe coarctation of the aorta.
The other question that comes up a
lot is how to measure the grade and
whether you incorporate
the diastolic runoff or not.
And on the right hand side you can see
the way of measuring it.
So you just incorporate the
systolic part of it.
Because if you, if you take
the diastolic component,
you will falsely decrease of the gradient.
So as we did here, we just
measured the systolic component.
And you can see here
the green was quite high,
so I'm engraving of
41 and typically would say that
there's significant coarctation if
the mean gradient is more than 20.
So it's been shown that the mean gradient for
coarctation correlates better with
the peak-to-peak great in the cath lab.
So, so I'm ingredient correlates
better with peak-to-peak measured invasively.
And then can you just tell us
The T wave that you include?
Yeah. Typically the end of the T wave.
So again, hypertensive patients
with very severe coarctation by alcohol.
What should we do? Medical therapy,
coarctation standing for surgical repair.
So stenting as the first option
followed by surgical repair.
I'm glad that none of you
chose medical therapy.
And I think that's that's
important to me and I'm glad that
there was kind of split
and we'll talk about a
little bit more at the end.
At the end we actually decided to
proceed with coarctation stenting.
The patient saw surgeon, so
an interventional cardiologists and
I think a lot of the decision
was based on the fact that he
was just recovering from
cancer and had had a recent operations.
So we decided to go with stenting.
And there's pros and cons in both approaches.
And we can talk a little bit about that as,
as we wrap up.
And here you can see the images.
So the mission was taken to the cath lab.
On the left-hand side, you can see
this obvious narrowing here with this shelf
like obstruction that you can
see on the on the lateral camera as well.
So a very tight coarctation of the aorta.
And as I mentioned,
the patient underwent stenting
in and on the right hand side you
can see the final result.
So wide open,
descending aorta with a grain of 0.
And the other thing you can see
as if you wait long enough,
you can see these very large,
I amaz that we're
bypassing the area of obstruction.
So again, this is another typical finding.
When you have severe coarctation of the Euro,
these large collaterals bypassing
the level of obstruction.
So I just saw him a year
after follow-up. The intervention.
He was complete symptomatic
infected was off BP meds because
on therapy became profoundly
orthostatic. So he's doing great.
There was no residual blood
pressure gradient at the bedside.
Now let's talk a little bit about
the echo doppler diagnosis.
So as we've talked about,
is important to interrogate
the descending aorta by 2 D. But
sometimes you can miss that because the,
the narrowing so severe
that you're not able to see it.
So as important they do users
CW Doppler need to just sweep around.
And sometimes the peat off is
actually better than the guide at
one because you're trying to
catch that pinhole narrowing.
But it is also critical that we interrogate
the abdominal aorta by BW.
And here's what you
expect to find in severe coarctation.
There's reduced
systolic forward flow velocity.
There's absence of
the diastolic reversal that
happens in early diastole now, surely.
And when you combine the two,
you have the typical sawtooth pattern
with this diastolic runoff.
And this is what we're talking about.
You can see on the lower panel,
this is the example from the first patient.
So this typical sawtooth pattern.
And then in contrast,
when we look at the normal,
you can see that there's very brisk upstroke
with a complete normal max velocity.
And then in early diastole you have
this reversal and then
you have forward flow again.
This is the so-called tried phasic pattern.
So forward flow that is
very steep than reversal
and then forward flow again,
sometimes this component, bye,
bye, might be missing.
And there's the so-called bi phasic,
which is also normal,
so steep upstroke normal velocity
with early diastolic reversal.
That's a sign of of normal findings.
Now the question that comes up
a lot is whether you're
you have coarctation when you're
sampling a side branch.
And I think the, the key thing
is to pay attention to the upstroke.
And you can see here that the
upstroke is very steep.
You might have seen
a smaller branch because of resistance.
But the key for the diagnosis of
a side branch is actually the upstroke
where steep versus very
slurred in someone with coarctation.
So don't be fooled by by
this diastolic runoff if
the systolic component is normal.
Always talk about the sawtooth pattern,
but why, why does it happen?
And I think this is the example
from the second patient.
You can see the aorta is in blue,
the ascending aorta is in red.
And when we look at
the descending aorta contour,
the upstroke is very slurred.
And that gets translated into
this slurred upstroke of the PW.
And then when you look at
the pressure difference between
the ascending and descending aorta,
as you can see that
there's a great in diastole
and that's what caused that diastolic runoff.
So this is the reason for
this sawtooth pattern that we see by Doppler.
And here you can see your post and data.
So the descending and the ascending aorta
traces now overlap.
There's no gray none whatsoever.
And when we post is
abdominal aorta during follow-up,
you can see that that early
diastolic reversal is now present.
So complete normal flow,
no residual coarctation.
Now just a summary in terms of
coarctation of the aorta is a common,
uncommon cause of hypertension.
So think about an AMD patients
presented with denovo hypertension.
So whenever you have someone
with a new diagnosis of hypertension,
don't forget to palpate
the femoral pulses and
also check the blood pressure in
the legs at least once
to ensure that you're
not missing the diagonal.
Now, why is it important to diagnose
coarctation is because if you,
if you do not diagnose or repair,
that leads to decreased survival.
So unrepaired coarctation is
associated with increased mortality.
Having to remember the common association.
So bicuspid valve is
president and 50 percent of patients
brain aneurism and 10 percent is actually
work of Dr. Connelly
and also think about Turner syndrome
because that's very common
in patients with Turner.
So look for the other feature.
So webbed neck, no low hair
and align so on and so forth.
So just remember that
it can also be syndromic.
Now, if you had a patient that has had
a coarctation repair, the surgical stent.
Just keep in mind that
some things are common and follow-up.
So hypertension is very
prevalent even
if there's no residual obstruction.
So that happens in 80% of patients.
Don't forget to exclude
reorientation that can happen
in 30% of patients,
especially those they hadn't neonatal repair.
Now just interrogate the valve,
the bicuspid valve is there.
And also ensure that you're doing
aggressive management of CVA
risk factors because those patients,
they have higher risk for stroke and MI.