So this is a 66 year-old female referred
for evaluation of pulmonary hypertension.
History is noteworthy for
a murmur noted early in life.
She had a new primary care physician
who noted that murmur and center
for a transthoracic echo.
And there was a long report,
but among other things,
that ACO reported pulmonary hypertension
and the patient's daughter
was in health care and said,
I think we should get an opinion elsewhere.
So cardiovascular review system,
she had some fatigue.
She exercised every day and had
no exertional dyspnea, rare palpitations,
but no reported a arrhythmias,
know lightheadedness or syncope,
and then very rare lower extremity edema.
Her exam blood pressure was
normal, BMI, what's normal?
You can see height and weight.
Their venous pressure was
normal, no carotid breweries.
And then she had a grade for
hollow systolic murmur noted
at the left sternal border.
The first second heart sounds
were not distinctly audible.
There was no third or fourth heart
sound detected,
and the apex was displaced.
No lower extremity edema.
And here's her electrocardiogram
and chest x-ray.
And then we'll look at her echocardiogram.
So and diastolic dimension
enlarged for this patient 57.
So velocity across that area of
color flow is 4.7 meters per second
in flow here,
and velocity over 4 meters per second.
And then apical imaging.
And I left this in the congenital format,
so left-sided chambers, right-sided chambers.
And then the velocity obtained from the apex,
again around four meters
per second as reported.
So what do you think? What's the diagnosis?
Pulmonary hypertension,
VSD and pulmonary hypertension?
Severe TR or something AS
so VSD and pulmonary hypertension.
You'd agree with the referring?
Okay. What would you do next?
More imaging, cardiac cath,
exercise, reassurance.
So more than one
of the above is often correct,
but when more imaging is an option,
it's usually the correct.
So here I think is
a very important part of
this patient's echo exam.
We're looking at the pulmonary valve.
There is trivial or
mild pulmonary valve regurgitation.
And we can see here
that the pulmonary regurgitant
and diastolic velocity is
about one meter per second.
So well, what's the likelihood that
the patient has
severe pulmonary hypertension?
I think you should. It's I
believe if you believe the signal,
then this is consistent with
normal peer pressures.
And I just want to point out something else.
We put a lot of emphasis on the,
on the end diastolic velocity
because that's how you can
get the PN diastolic.
So basically you get that velocity,
you just Bernoulli it,
and then you add the IVC.
But I think it's important also to
pay attention to the opening.
Can see here that the opening
pressure is below two.
So that matches the PA,
early dice or at least systolic velocity.
And then if you were to bernoulli that again,
you would see that the peer pressure is very
low at the beginning of diastole.
So sometimes you can have
a low end diastolic pressure,
but you can have a high opening pressure
and that can be seen in pH.
So the fact that you have both normal values,
I think is reassuring.
And then we'll commented on
the ADHD guidelines published
in Jack and circulation and 2018.
So these are the this is the schema for
ventricular septal defect in the adult.
If there's left to right shunt,
then you really need more information.
Is the left ventricle enlarged?
What is the Q peak us?
What's the pulmonary artery,
systolic pressure, et cetera.
And so obviously this patient
needs additional
information with a cardiac cath.
So both groups, of course, we're right.
And this is actually a case that, well,
did you want to comment on what
we see here? True.
And this was the old way of diagnosing VSD.
You can see on the,
on the right hand side
is we inject contrast in
the LV you can see flow going to the PA,
can see the bone wedge
gathers in the PA. You can see
flow crossing the ventricular
septum and going up the PA.
So this is fits with,
with the patient's VSD.
And I don't have
those beautiful human dynamic drawings
like Will does,
but membranous be SD was the diagnosis.
The Q peak US was 1.6.
And then you can see the pressures there.
Well, any comments
are pretty self-explanatory.
Yeah, I agree.
And so I guess my big question is,
why was this patient thought
to have pulmonary hypertension?
Why did you think she had
pulmonary hypertension?
And I think going back to the echo
and another imaging tip so
important for all of us to image
patients for the Sinatra fors in particular,
but also those of us
who image patients regularly.
In this patient, it was
an artificial report
of pulmonary hypertension.
And that's because the VSD flow
contaminates that quote unquote TR velocity.
So here you can see that it was a,
it was a pretty authentic looking jet
of tricuspid regurgitation.
But actually if we go back to
the short axis view,
we can see the flow through the VSD here.
And then it kinda takes a turn
and goes through the tricuspid valve.
And so it really contaminates
that TR velocity.
And so you cannot rely on
the TR velocity to assess
pulmonary pressure in
this particular patient,
even in our lab,
we couldn't say for sure
what the TR velocity was, but of course,
knowing what the pulmonary
regurgitant velocity
was was very helpful.
Yeah.
I can hear. I think it goes with wave.
We're just saying I think I presume
this is a cast person too.
There was L v2 are a shunt.
And from a technical standpoint,
given the proximity between
the VSD and the tricuspid valve.
And typically there is TR because
the septal leaflet tries to close a defect,
some of the VSD flow goes backwards.
So if you were to sample the RA
for your saturation room,
for example, is typically high.
So that's why you go SVC, IVC.
So on the Android looks like
there's LAL phi2 are a shunt,
but it's just the TR taking some of
that contrast back into the, the RE.
So you're not a true Garibaldi defect.
And send that then
to determine what to do next,
I think we go back to the guidelines and here
our patient's hemodynamics cubic us
greater than 1.5 to
one PA systolic pressure was less than 50
percent systemic and she had
LV enlargement and so
we recommended surgical closure.
Device closure really wasn't a good option in
this patient because of the location
of that VSD.
And this is
just the guidelines for your reference.
So the learning objectives for
this case where to understand
the clinical diagnostic
and imaging features of
BSD and then to differentiate causes of,
quote, pulmonary hypertension
reported by transthoracic echo,
similar to Will's anatomic demonstration.
This is from Dr. Bill Edwards
collection and this is a membranous VSD.
And you can see the close proximity of
that beautiful example of
the membranous VSD to
the aortic valve cusps here.
And so it's not surprising that
associated features
include aortic valve disease.
And then as we saw in our patient,
tricuspid valve disease exactly as Will said,
that septal tricuspid leaflet
tries to close off
the VSD and then causes tricuspid regards.
Other associations include endocarditis and
then an entity which I'll show you shortly
called double chambered right ventricle.
Then management depends on
the size and associated features.
And here's just a different patient.
And you can see also that
this is a membranous VSD.
It's close to the tricuspid valve.
Some people like to say,
look at the aortic valve as a clock face.
I actually like to say,
is it close to the tricuspid
valve or is it close to
the pulmonary valve to determine
what type of VSD it is.
So this we would say
membranous or pairing membranous
VSD a in close proximity
to the tricuspid valve.
And then you can see
the left to right shunt here.
There's not that contamination
with the TR velocity in this particular case.
And then long axis, again,
difficult to exclude an outlet
VSD and the long axis view,
but you need the combination of long axis,
short axis again,
the imaging of these patients is so critical.
So membranous VSD, the most
common type of VSD that you'll see in adults.
Adjacent, as I've already
explained to the aortic and tricuspid valves.
As we mentioned,
the tricuspid valve can become
adherent as it tries to close off that BSD.
And rarely you see
aortic cusp prolapse that
leads to progressive AR.
And then as demonstrated here, long axis,
short axis views nicely demonstrate
the anatomic features. Yeah.
So there was a question here. I think it was
actually a communist, absolutely right?
That the tricuspid valve
looked abnormally thick.
And so sometimes the tricuspid valve
may be primarily abnormal.
And I think just a tip when you're
actually scanning these patients,
I find the subcostal view very helpful
because the VSD will be coming towards you.
Where's the tricuspid valve?
Gradient jet will be going the wrong way.
Then when you're adopting,
they're going opposite ways and it might
be easier to separate the two that way.
And I mentioned I just
make a comment about double
chambered right ventricle.
So it's not an uncommon entity
that you will see or that we see in patients.
And they've always had a history of
a murmur since early in life.
So this is a schematic that
demonstrates the features of
double chambered right ventricle.
So occurs in association
with a membranous defect.
It's progressive hypertrophy of
right ventricular muscle bundles
at the infundibulum and
superior margin of the VSD.
And there's a proximal high pressure area
here and then a distal low pressure area.
And we see them not infrequently.
So report it to occur in
three to 10 percent of patients
with membranous VSDs,
rarely associated with a arrhythmias,
but patients can present
with symptoms of dyspnea.
And the treatment of choice for
severe obstruction is operative intervention.
So take home points with regard to
ventricular septal defects in adults,
a small VSD shouldn't
cause pulmonary hypertension.
I think we could see
the anatomic boundaries of
that BSD pretty well
and didn't think it was large.
The VSD jet can contaminate
tricuspid regurgitation velocity and
cause that pseudo
pulmonary hypertension picture.
And then LV enlargement is an indication for
VSD closure in the absence
of significant pulmonary hypertension.
So in this case, clearly Kath was
required to confirm the pulmonary pressures.
But then also
operative intervention was suggested.