So there's a 52 year old male admitted to
the hospital with heart failure
who was actually a class for.
He had no prior history of
whatsoever from a cardiovascular standpoint,
but he had several CV risk factors.
So CKD, diabetes and hypertension.
And over the course of a year he went
from class 1 to class four.
So here you can see it's physical exams.
Yet an enlarge pulsatile liver
at two plus leg edema.
The Basie had an early systolic
ejection murmur with an increase p2.
Then at the apex get
a whole a systolic murmur with an S3.
Of course, the patient came to
the Ecolab and you can
see the Pearson r long axis on the left,
you can see that the ventricle is
dilated and that function is reduced.
The aortic valve is thickened.
And when we went ahead
and measure the ventricle
that confirm to the ventricles dilate.
This was 55 millimeters and end diastole,
which for him was mildly dilated.
We can also see that the RVs
dilate into function is a little bit down.
Are we going to zoom into the mitral valve
here because of that systolic murmur.
And you can see here that the
mitral valve is thickened.
And then there's a jet of
moire that is posteriorly directed.
And also if you focus on the origin of
that jet is a little bit unusual.
And we're going to, we're going to
look at this further.
So here's the short axis view on the left,
you can see that the mitral valve
as an unusual appearance.
And then when we put color Doppler on,
you can see that
that posteriorly directed jet
also originates from a funny spot.
So based on the aqua images,
what would you say that the mechanism of MR
is those interior prolapse
lean into that posteriorly directed
jet ski make
MR. Or there's a mitral valve cleft.
Please go ahead and vote.
Excellent. You don't need a session at all,
so you're absolutely right
that some mitral valve cleft.
And again, the key for the diagnosis when
you zoom into the microbiome,
we can see that
actually then anterior leaflet
is split into two.
And then when you sample with color Doppler,
the jet is originating from the cleft itself.
So very typical findings
of a mitral valve cleft.
Now we're going to move to
the apical images of
a flip damages to match
the anatomy of format.
So on the left-hand side then you have
the LV to the left.
And then we put color Doppler.
We see quite a bit of flow,
flowing in an unusual way.
V on the right to left.
On the course.
I have to have
should've kept in the male form,
but thank you.
Now, what's the final diagnosis?
So put it all together as an isolated
mitral valve cleft is
a partial AV canal
or there's a complete AV canal.
Very good. So partial AV canal.
Now just going over the options here.
So an isolated mitral valve cleft
is very rare
and some other features you're
suggesting that there's more to the story.
In a complete canal comes with a VSD.
So a patient at age 52,
if the patient had
an unrepaired complete canal,
you would see a is'm anger syndrome.
So this has to be a partial AV canal.
And why don't we go back to the,
the AICPA Gua images here you can see
that the hallmark of a partial AV canal,
we can see that both AV valves,
they sit on the same level.
And people have described this.
This is a common valve with two orifices.
So again, the key for
the diagnosis is to see that
both tricuspid and mitral
valves the same plane.
Now on the right hand side,
as we put color Doppler,
you can see that they're shunting across
the atrial septum and it's inferior portion.
So this is very typical for a primum defect.
So valves on the same level.
And then you have this until that happens in
the or part or
more apical part of
the atrial septum, if you will.
Now you can see the same thing from
the subcostal view.
So you can see that defect
again is in the lower part of the septum.
And then as we put color Doppler,
you see all this flow
crossing the atrial septum.
So a very large left-to-right shunt
in the lower part of the atrial septum,
typical for a primum defect left hand side.
We can appreciate how the two valves,
they sit on the same level.
So typical for a primum defect.
So there's the summary of the ACO.
So partially AV canal with
a large left to right shunt,
clef mitral valve with severe
MR mouth LV enlargement
with mildly reduced EF.
That was moderate RV enlargement,
which is not surprising with
that big shunt with mod increase in ARV ESP,
and there was also matter TR.
Before I move forward,
I think the question is,
why is the LV abnormal?
There's a severe MR. Is it because of
the large atrial level shunt or
do we have superimposed
scheming heart disease?
Is go ahead and vote.
So most of you said
severe mitral regurgitation.
So some said HER level shunt with
very few scene schema, heart disease.
So here you can see is angiogram and I think
we've all learned this the hard way.
So congenital heart disease in adults is
typically seen accompanied by something else.
So just make sure that you go
through your typical things.
In terms of LV dysfunction,
just keep your diagnosis broad so you
can see three vessel disease with,
with typical appearance
of of diabetic vessels.
Go back to the options.
So severe MR leading up to
diastolic systolic dysfunction rather
typically would see
the ventricle being very big.
So typically the ventricle gets very
big before it gets weeks.
So that's why we intervene
based on LV dimensions.
And then atrial level shunting has been
implicated in LV diastolic dysfunction,
but not in LV systolic dysfunction.
So we felt that a lot of the
systolic dysfunction was actually because of
superimposed three Vessel coronary disease.
So then again, putting this all together,
what to do and I think it was pretty clear.
I think the patient needs surgery and he
has scheduled for surgery next month.
It's going to be a high risk operation,
but I don't think there's any doubt that
the patient meets surgical intervention.
Now let's just go over partial u v canal.
So is the so-called ostium primum defect.
There's a large ASD
typically, but there's no VSD.
That's the difference between the
complete or the transitional for example.
So partially AV canal know
VSD mitral valve is universal.
So the mitral valve is also
abnormal and they are also prone to
having LVT obstruction that can be because of
fibrous bands are because the OVOC small.
So there are
several different mechanisms but also
interrogate the LV OT in a partial canal.
And on the right hand side you can see
a gross specimen and the arrow points to
that large, low-lying ASD.
And as I mentioned,
the cleft mitral valve is universal.
So when you look at the pathology specimen,
that's how he looks and he looks just
like what we see by t, t0.
And this is what you would expect
if you were to 3D it.
So you can see this,
this triangular shape mitral
valve typical for a cleft mitral valve.
And if you were to put Doppler,
the the jet comes from where the cleft is.
I just want to touch upon
mitral regurgitation AV canals
because that's
the most common scenario that we're
faced with in adults
typically to have a repair of their canal
early on and they come back for
follow-up and they have mitral regurgitation.
So this is data
from Mayo and when we looked at the
most common and to be
a re operated mitral regurgitation
is actually the most common cause.
So just keep that in
mind that they might come
back with residual mitral regurgitation,
especially if the cleft wasn't closed.
That's why I Male 80 percent of
the patients will have primary closure the
time a repair just to
avoid recurrent
mitral regurgitation down the road.
Um, here's a great example. So there's
a 20 year old patient that I recently saw.
She was repaired at age four.
She's done great. But as you can see here,
she has quite a bit of MR has been
described as matter to severe versus severe.
The LV is indeed dilated.
But the other thing that we see,
which is very typical, there's also
a component of inflow obstruction.
So when we sampled the mitral valve,
the grain and was three,
so typically they have some degree of inflow,
which of course affects
their candidacy for repair.
So you just gotta keep in mind
that most often this is
mix disease with inflow and riggers.
But now in a patient like this,
for example, she's asymptomatic facts,
you just deliver a baby boy and then
she did great. What would you recommend?
So she has known severe MR. With
a dilated ventricle would
just say observation,
mitral repair or should
we go ahead and replace the valve?
So most of you said mitral valve repair,
interesting, I was not expecting then.
I think that brings us up to the question,
how should we manage MR.
in a canal or how should we
manage MR. in an adult congenital population?
And surprisingly, there are
very few papers out
there trying to support data.
And then when you don't know what to do,
to go back to the guidelines and this is
what the American guidelines would say.
These are the SHG guidelines.
And interestingly, they say you should manage
MR. in that congenital patient just like
how you manage primary MR. How you manage
and Martin and
acquired heart disease patient,
which I always find a big,
controversial or counter-intuitive this
should say because there are
completely different
and to bed niches now here.
But when you look at the guidelines,
they keep harping on this important aspect of
micro repair that needs
to be done at low mortality.
With a higher repair
it so should be greater than 95 percent.
And when you think about someone who's had
cardiac surgery that has
an intrinsically abnormal valve
is hard to conceive
that low mortality with
a high repair rate. So you got it.
You have to keep that in mind that
your patient may go to the 0 or
expecting a repair and they
might come out with a replacement.
And when we look at the outcomes
of the re-operation,
that means this data from the same paper,
you can see here that although
the p-value is not significant,
the repair group did much better.
So if your patient goes for replacement,
the outcome might not be the same.
And when you look at the breakdown here,
numbers wise was half and half.
And then if we're borrowing data from
the acquire heart disease literature,
this reminds me of this.
So when we look at the acquire mitral valve,
population replacement,
it's translated into worse survival.
So just keep in mind in
your canal patients that are doing very well,
maybe they're better off left alone.
And in fact, this patient has been
followed for many years in her memoir has
remained stable and said she just
delivered a baby boy without any trepidation.
Now I think just I just wanted to
finish with this and I think this
also highlights the congenital heart disease
and acquire Hertz's are different.
The mayor experience, our
fellow just looked at this.
And it was interesting when we looked at
canals with significant NMR.
You can see here that the ventricles
are much smaller.
So again, if you're, if you're borrowing
the criteria for acquire heart disease,
you might have a problem
because they're not truly
applicable to
patients with congenital heart disease.
So you can see again,
the ventricle was bigger
but still much smaller
than what you would expect.
For example, in prolapse.
They are VSP was the same,
by the LA, was bigger.
So again, it might
be some repercussion for the atrium,
but not necessarily for the ventricle.
And also the other thing to point
out is that the EF was,
was lowered even into patients with,
with more than moderate MAR.
So again, be careful borrowing the criteria
from the acquire heart disease world.
So just to summarize.
So whenever you see a cleft mitral valve,
think about unrepaired AV canal
in an older adult.
Mr. post AV canal repairs,
the most common reason for
re-operation that commonly have
micro disease that is mixed
so MR. with some degree of MS,
which may affect how we proceed surgically.
And I really think that we need more data
in congenital patients to
know how to manage this stuff. Population.