Echocardiography and Cardiac Masses - SD

Introduction

My name is Jim Kirkpatrick
and I am an assistant professor
of medicine in the cardiovascular medicine division at the
University of Pennsylvania.
Today I would like to talk to you about echocardiography
and cardiac masses.
I'm not gonna be talking about a comprehensive look at
cardiac masses, but rather
echocardiography its use in diagnosing cardiac masses
and some of the important things about masses that relate
to echocardiography.

Case Presentation

First, let's start with a case.
Here is an apical four chamber view on the left hand side
of the screen, which is cut down
to emphasize the left ventricle.
You can see that there's a well circumscribed mass right
there at the apex,
and on the right side of the screen, that area is zoomed in.
You can see, sure enough, it's well circumscribed.
It appears to be arising from the ventricular myocardium,
doesn't extend outside
of the heart into the pericardial space.
This is an echocardiogram,
which was recorded from a 19-year-old patient who presented
with fever and chills, sweating and fatigue.
Later in the program we'll actually reveal
what this unknown is,
but for now, I want to go over an outline of
what we're going to talk about.

Outline of the Talk

First of all, I'm gonna describe some background information
about cardiac masses that I'm gonna talk specifically about.
Primary cardiac benign tumors.
Then primary cardiac malignant tumors, metastasis
to the heart, non-tumor masses.
That can sometimes be confusing.
We'll touch on diagnosis and treatment
and then we'll play a game called you make the report.

History of Cardiac Masses

Cardiac masses have a long history.
They've been known at least from the 16th century.
It wasn't until 1951,
however, that the first non autopsy diagnosis
of an intracardiac cardiac, intracavitary cardiac tumor
was made via angiography.
A few years later, there was the first successful excision
of a left atrial myxoma and a few years
after that echo came on the scene
and the first M mode recording
of a left atrial myxoma was made.

Guidelines on Echocardiography and Masses

What do the guidelines say about
echocardiography and masses?
Number one, it is appropriate in fact a class one
indication to evaluate patients with echocardiography
who have clinical syndromes
and events that suggest an underlying cardiac mass.
It's often hard to figure out what these would be, but
nonetheless, if it looks like the patient has a cardiac
mass, it's appropriate to do an echocardiogram.
Number two, patients who are known to have a predisposition
to mass formation echocardiography can be helpful in these
patients to decide surgery versus anticoagulation,
essentially to attempt to identify
and distinguish a thrombus versus a tumor.
Three, it's appropriate to perform echocardiography,
to follow up on a cardiac mass
after surgery to look for recurring masses.
Number four, it's also appropriate to use echocardiography
to stage primary malignancies,
and this is an area that probably gets
underused as we shall see.
Number five, echocardiography can screen patients
with a disease state that's likely
to result in mass formation,
but in the absence of clinical signs
or symptoms of a mass note, this is a two B indication.
And number six, it is inappropriate, in fact,
just plain wrong to do echocardiography on a patient
when you will have no impact on the diagnosis
or the clinical decision making based on the results
of the echocardiogram.

Overview of Primary Cardiac Tumors

Let's do an overview of primary cardiac tumors.
They are exceedingly rare.
The incidence is 0.002
to 0.3% in unselected autopsy patients.
Three quarters of these tumors end up being benign.
One half of these benign tumors are myxomas.
One quarter of these primary cardiac tumors are malignant.
95% of these are sarcomas.
Metastatic tumors are 20
to a hundred times more common than primary tumors,
and as we shall see, they tend to involve the pericardium,
then the myocardium and then the endocardium.

Clinical Presentation of Primary Cardiac Tumors

Primary cardiac tumors present with a wide variety
of symptoms and the symptoms are all essentially related
to the location of the tumor.
Its size, its underlying histology
and the friability of the tumor causing embolic phenomenon.
Cardiac tumor symptoms have been put into a triad,
and this is classically from myxomas.
It includes intracardiac obstruction
and this could be from functional valvular stenosis,
which can vary with body position, can actually end up
with complete obstruction leading
to syncope or sudden death.
There's also what's called the wrecking ball effect.
Basically the tumor on a stalk with each cardiac cycle
just bashes into the valve
eventually causing valve damage and regurgitation.
We'll see some examples of this.
78% of malignant primary cardiac tumors present
with mechanical obstruction because they've gotten so large
before they're detected.
The second component
of the triad is embolic phenomenon when tumor material
or thrombus, which forms on top of the tumor
goes somewhere in the body.
Finally, the third component
of the triad is constitutional symptoms.
These present with a wide variety
of symptoms including fever, fatigue, rash, arthralgia,
myalgia, weight loss, anemia, Raynauds phenomenon,
digital clubbing, and of course when you perform
laboratory evaluation, elevated acute phase reactants,
and this is probably related to tumor release
or some tumors, releasing interleukin six.

Physical Exam Findings

Primary cardiac tumors can be found on physical exam.
They can be suspected when there's a systolic
or diastolic murmur.
In fact, in one half of cases of cardiac tumors,
these murmurs are present loudly and widely.
Split S one can be caused
by a delay in the mitral valve closure.
The tumor plop a classic sign which comes
after the S two cardiac sound
and sounds an awful lot like an S3 can occur in up
to a third of cases.
Pericardial friction rub is common, especially in patients
who have right atrial tumors.
And then of course there are signs of heart failure,
outflow obstruction leading to hypotension,
inflow obstruction leading
to congestion either within the lungs or in the periphery.
You can see an example here.
This is a transesophageal echocardiogram.
There's an extremely large mass that's attached
to the inter atrial septum
and it exhibits that wrecking ball effect on
the tricuspid valve.

EKG Findings

When you do an EKG on a patient with an intracardiac mass,
you can find a lot
of different things including a completely normal tracing.
Atrial fibrillation ends up being the most common
and occurs in 15% of myxomas.
Logically ventricular tumors produce ventricular tachyarrhythmias, infiltrative tumors,
if they infiltrate the conduction system
can lead to heart block.
The main point to remember here is
that when you see these things on the EKG,
you should keep intracardiac mass on the differential,
even if it's pretty far down that differential.

Specific Benign Primary Cardiac Tumors

Myxomas

So let's now turn to the tumors themselves.
Looking at actual etiologies,
here's a subcostal transthoracic view.
You can see in the inter atrial septum right here,
there in fact is an intracardiac mass.
It's attached to the septum, it appears somewhat villous
and it's extending into the left atrium.
This is a myxoma as is the one here
that we see down a little bit lower
or a little bit higher, excuse me.
In the inter atrial septum,
myxomas are the most common cardiac tumors in adults.
They occur in the third to sixth decade
with a mean age of 56 years.
70% are found in females
and for the most part they're sporadic
with notable exceptions.
The myxoma syndromes include the Carney complex.
This is familial in about 10%.
It's autosomal dominant and localized to chromosomes two
and seven genetic defects.
The PRKAR1A gene and chromosome 12 have also been implicated
and the mean age is a lot earlier 25 years.
Myxomas that occur in the Carney complex are more likely
to be in the ventricles
and to occur in multiple spots in the heart.
Essentially it's tumors, tumors everywhere, schwannomas,
breast tumors, pituitary adenomas, primary adrenal,
cortical micronodular dysplasia
and Sertoli cell testicular tumors are also found along
with the intracardiac myxomas.
Other syndromes include the NAME syndrome, nevi,
atrial myxomas, myxoid neurofibromas and ephelides,
and the syndrome LAMB, atrial myxomas and blue nevi.
Myxoma pathology is gross.
In fact, it's downright disgusting.
It can, they can grow
to be very large in size 15 centimeters.
Most are about six centimeters in size,
which if you think about the size
of the heart, that's pretty large.
They can be polypoid, villous or lobulated.
They tend to have large blood vessels at the base
of the stalk and the surface is often covered
with thrombi giving it this,
rather arresting appearance we see on the
pathologic specimen.
Microscopically myxomas appear
to have multipotential mesenchymal cells from endothelium,
endocardium, excuse me,
in the stroma there can be calcification with foci
of metaplastic bone, about 10%.
This is more common in right atrial myxomas.
There can also be extramedullary hematopoiesis
and you can see in the example here there's free floating
spindle cells and stellate cells and of course a lot of myxoid
ground substance location is very important in
real estate, but also in myxomas.
This is an example of some Philadelphia real
estate where I live.
75% of myxomas are found in the left atrium, 15
to 20% the right atrium.
And of course the remainder are going to show up
in the left ventricle or the right ventricle.
They often in, in, in the atria are attached
to the inter atrial septum.
At the fossa ovalis about 90% are here.
And then in with decreasing frequency,
they're found in the posterior atrial wall,
the anterior atrial wall and the atrial appendage.
They do grow at somewhat fast pace every year.
On average of course there's a lot of variability,
1.8 centimeters per year.
This equates to about 14 grams.
They get symptomatic in general.
And of course this depends on the size of the patient,
the size of the heart.
When they reach about 70 grams,
they can get much larger in the right atrium
before they're discovered
because there does tend to be fewer symptoms
that are alarming that come from right atrium myxomas,
compared to left atrium myxomas.
And here's one of the key points
that I want you to remember.
30 to 40% at least of myxomas
end up embolizing.
Myxomas can also make a comeback if you will.
They can be recurrent in the first four
years postoperatively.
This is of special concern.
Why does this happen? It could be
because of incomplete resection.
It could be because there actually were other foci
of myxomas which were missed.
12 to 22% of familial
or complex myxomas actually end up coming back.
The numbers are much lower in the sporadic myxomas
and this has led to a recommendation
that echo be performed every six months in patients
after resection of a myxoma.

Papillary Fibroelastomas

So here's another tumor.
This, as you can see from this
transesophageal long axis view
of the aortic valve is hanging off the aortic valve leaflet.
It's large and it appears to be attached via a stalk.
It's fairly well circumscribed
and it doesn't exactly exhibit the wrecking ball effect,
but it's definitely moving
around an awful lot in the aortic root.
This tumor is a papillary fibroelastoma,
papillary fibroelastomas are 10%
of primary cardiac tumors making them, of course very rare,
but this ends up being 85% of all tumors
that arise from valve tissue.
They occur in the elderly.
They look like sea anemones with a short pedicle.
And fronds especially
as shown in this microscopic specimen.
They're generally small, less than 20 millimeters.
They're well delineated
and they can often be confused with vegetations, Lambl's
excrescences blood cysts,
but they tend to have stalks, which isn't always true
for some of these other things.
In the differential diagnosis,
papillary fibroelastomas are generally attached
to the valve apparatus.
They occur more commonly on the aortic valve
and then the mitral valve, they're very rare on the
pulmonic and tricuspid valves.
70% are associated with some sort of valvular disease,
whether, usually regurgitation
and there may be a high incidence of cerebral coronary
and what has been reported in the literature retinal
embolization, but it's still quite, unclear
what should be done with these if they haven't embolized.
One of the points to make,
at least traditionally in one series was that 48 out
of 141 were actually missed.
So these easy to miss these,
especially when they're quite small, as with a lot
of tumors, if something grows larger than one centimeter
and of course if there are any symptoms,
surgery is recommended.

Rhabdomyomas

Here's another tumor. This is a rhabdomyoma
and the fact that this is a still frame
and not very good quality speaks to the fact
that it's pretty rare and I don't have a
moving example of it.
Rhabdomyomas are the most common cardiac
tumor in childhood.
75% of them occur in patients
who are less than one year old.
They are associated with abnormalities on chromosome nine
and 16 may involve tumor suppressor genes.
They appear to have striated muscle
quote unquote spider cells.
They mostly occur in the ventricles.
They can be multiple, they can show up
as pedunculated masses.
They can cause obstruction.
Classically, and perhaps the most interesting thing about
it, especially in children is that these,
their cells lose mitotic ability
and there may be spontaneous resolution.
They're associated with tuberous sclerosis, which
as you may remember includes mental retardation, epilepsy,
adenoma sebaceum and angiofibromas.

Lipomas

Here's another tumor, rather two tumors.
This is obviously an explanted heart.
There's a large one in the inter atrial septum
and there's a little space in between.
And then there's another large ball like tumor.
And this actually is a rather extreme example
of lipomatous hypertrophy of the atrial septum.
Let's use this to actually talk about lipomas.
Lipomas consist of 10% of cardiac neoplasms.
They tend to be encapsulated. There's a wide size variation.
They can be intrapericardial. Intramyocardial.
They can grow from the valves.
And then of course there's lipomatous hypertrophy
of the inter atrial septum, which is essentially a lipoma.
There is an increased incidence
of supraventricular tachycardia,
especially when the septum is involved.
It can also be SVC obstruction.
It's not hard to see how this tumor could obstruct
the superior vena cava.
And of course, MRI is very good at showing the traditional
fat appearance, fat signal intensity,
and of course lack of perfusion when gadolinium
enhancement is performed.
And here's just an MRI example, we can see a very large
lipoma tumor lights up on the black blood
and it's actually either arising from
or going into the anterior myocardium there.

Fibromas

Here's another tumor.
This is a short axis of the left ventricle.
You can see the mitral valve here, right in the middle
down here in the inferior wall.
There's a well encapsulated tumor inside the myocardium.
This is a cardiac fibroma.
Here's another rather dramatic example labeled F here
of a cardiac fibroma taking over the heart.
Essentially 2.8%
of primary cardiac tumors or fibromas.
This makes it the number two benign cardiac tumor.
Central microcalcifications or cystic degeneration can arise
because these things grow and outstrip their blood supply.
They can occur in the intramural free wall.
They can be well demarcated, they can be obstructive,
not surprising, and they tend to happen in childhood,
but they're very slow growing.
So they're often detected as adults,
in adulthood, excuse me.
Now even though they're benign,
they can grow into the conduction system
and the cause of ventricular arrhythmias and sudden death.
And this happens in about a third of patients.
They're associated with the Gorlin syndrome
and the Gorlin syndrome includes basal cell carcinoma,
generalized body overgrowth, jaw keratosis
as shown here in the picture
and other skeletal abnormalities.

Hemangiomas

So you remember this, this was our first case
that we showed right at the beginning of this talk.
It's well encapsulated tumor here in the left
ventricular apex.
If you still don't know what it is,
here's the pathologic specimen
and see there's these big vascular channels in it.
And this is a hemangioma five to 10%
of benign cardiac tumors can be hemangiomas.
They can be capillary cavernous intramuscular just like
the one we've seen in our example
where they are doesn't seem to influence prognosis.
They can present with dyspnea, exertion, angina,
pericardial effusion,
and of course if you perform angiography,
you can see them light up with a tumor blush of contrast.
Total surgical excision,
unfortunately is usually not possible
and patients have ventricular tachycardia
and sometimes can be left
with large pericardial effusions causing tamponade.
They can also spontaneously resolve
and it has been reported
that steroid therapy can hasten this.
Here's an example of a very large hemangioma.
You can see how vascular it is.

Other Benign Tumors

Under benign primary cardiac tumors.
We should also mention mesotheliomas of the atrioventricular node, cardiac
pheochromocytomas, granular cell tumors, hamartomas, myxomas,
lymphomas, neurofibromas.
These are all in the case report category.

Review of Primary Benign Cardiac Tumors

So let's review primary benign cardiac tumors.
Which ones are located on valves occur in the elderly
and have a sea anemone appearance.
Papillary fibroelastomas. Which ones are located in the interatrial
septum can cause atrial fibrillation 15%
and are associated most of the time with murmurs.
That would be myxoma.
Which of them occur in the ventricles, can be multiple
and are generally found in the pediatric population.
Rhabdomyomas which grow out of the walls
of the ventricles are well demarcated,
also occur in pediatric patients,
but are slow growing, found in adults
and can cause ventricular arrhythmias,
especially if they get large enough fibromas
and which are intramuscular and very hard to resect.
Hemangiomas.
And just to make a point, our patient actually
did have a successful excision
of her hemangioma and she did very well.

Primary Malignant Cardiac Tumors

Malignant primary cardiac tumors is our next topic
and essentially this topic is all about sarcomas.
95% at least in this series of a wide range of years.
You can see 1976 to 1993
ended up being sarcomas.
Here's a picture of an angiosarcoma.
This is a transesophageal view.
You can see the aortic valve
and this tumor's actually located in the left atrium
and growing and getting very close
to the mitral valve if not having a wrecking ball effect.
Angiosarcomas occurred in the third to fifth decade.
They're actually rare in children.
They occur with a male predominance.
They occur in the right atrium or the pericardium about 80%.
So our example is quite unusual.
They can produce a continuous murmur
because they have a large vascular supply
and there can actually be dilated vascular channels which
supply these tumors.
They are recurrent and they can cause pericardial
effusion and tamponade.
They can lead to cardiac rupture.
They are rapidly progressive survival is six months at
the very best.
Debulking might alleviate symptoms depending
where the tumor is and what the symptoms are.
There is some reported success with chemotherapy,
with radiation therapy and IL 2.
There is recurrence with immunosuppression.
However, in patients who are transplanted
and usually these patients die from widespread infiltration,
intracavitary obstruction and or metastasis.
Here's another example
of a rhabdomyosarcoma.
And these are also exhibit a bimodal peak in infancy
in the sixth to seventh decades.
So they're found both in children and in adults.
They also occur in males.
They can occur in any cardiac chamber,
but they tend to arise from the left ventricle.
60% have multiple locations.
Paraneoplastic syndromes
are found in them hypertrophic osteoarthropathy
polyarthritis amyloidosis, neurofibromatosis,
which is you remember caused the cafe au lait spots
and eosinophilia.
They are noted to have hematogenous spread
and so survival is less than a year.
There may be an effect from chemotherapy,
but for the most part, surgery is really the only option.
Primary cardiac lymphomas are quite different than these
other malignant cardiac tumors.
Most of them are non-Hodgkin's B cell lymphomas.
They can be a diffuse B cell type, about 80%, 1.3%
of all cardiac tumors can be cardiac lymphomas.
Rising incidence is happening recently with AIDS
and of course transplant immunosuppressives.
They're still quite rare,
however, they are marked by rapid progression.
But, and here's the key difference.
They are chemotherapy responsive
and surgery's actually usually not helpful
unless it's palliative to relieve
obstructive or other symptoms.
Then there are a few more, the unusual gang of suspects,
plasmacytomas, fibrosarcomas, mesotheliomas, liposarcomas,
and even osteosarcomas.
Again, most of the case report material.
Here's an example of, such a case myxosarcoma.
You can see this very large tumor located at the base
of the inferolateral wall extending out
from the heart into the pericardium.
You can see how it's obstructing mitral inflow
and also obstructing outflow
through the left ventricular outflow tract.

Review of Primary Malignant Tumors

So let's review primary malignant tumors,
which tumor arises more often in the right atrium
or the pericardium occurs in adults
and has a male predominance
that's angiosarcoma which tumor occurs in the left
ventricle, can be multiple, has a bimodal peak,
but also occurs with a male
predominance rhabdomyosarcoma.
Which one occur in patients who are immunosuppressed
and the incidence is increasing primary cardiac lymphomas.

Metastatic Tumors to the Heart

So now let's turn to the far more common metastatic tumors.
Here's a compilation of four different studies
that was performed in 2004.
Each one of the bars on this graph represents
the data from a different study.
So white bars are all in one study
and gray bars all in another dark gray and a third
and the striped bars and a fourth.
And it's interesting what we can see across these studies.
Melanoma by far is the most common
metastatic tumor to the heart.
Really in all of these different studies, breast looks
to be second and many of the studies followed by lung.
Interestingly, two of the studies showed thyroid gland,
metastatic tumors to be fairly common.
And then of course, lymphoma was,
found in all of the studies.
There have been a categorical approach
to characterizing metastasis to the heart.
They've been divided into three categories,
including tumors which are uncommon,
cancers which are uncommon
but tend to go to the heart.
And those include melanoma, germ cell tumors and thymoma.
Then there is the category of tumors
and cancers which are common and they may go to the heart
or may have an intermediate potential to go to the heart,
stomach, liver, ovary, colon, and rectum.
And then there are tumors which are pretty common
but almost never go to the heart.
And that includes cervical tumors.
Cardiac metastasis shows about a 10% incidence.
And then of course if you look at the autopsy patients,
it can go up to about 25% of patients dying
of malignancies having evidence of cardiac metastasis.
The one huge exception appears
to be primary neurologic tumors,
which tend never to go to the heart.
When tumors go to the heart,
they can show up in different places.
Perhaps the most common is involvement of the pericardium.
We can see here in the top middle of the screen.
Then they can go to the myocardium.
As you can see multiple studded areas in black here on the
lower right hand part of the screen.
And they can also show up as intracavitary tumors,
either just having the tumor material lodge,
somewhere within the ventricular or atrial cavity
and setting up shop and growing from there
or perhaps even growing in from the wall.
Here's an example of a thyroid carcinoma which went
to the right atrium
and may actually be growing out of this superior vena cava.
Not well defined here on this transthoracic echocardiogram.
An off axis view in which
the right ventricle is on the left side of the screen.
The left ventricle is on the right side of the screen.
You can see the aortic valve in the middle
and then the huge tumor mass in the right atrium.
You can see that it on the right side,
the right panel here that there's actually quite a bit of,
obstruction to tricuspid valve inflow
and essentially functional tricuspid
stenosis caused by the tumor.
Here's an example of a tumor that went to the pericardium.
We see this large circumferential pericardial effusion from this
short axis view.
The left ventricle is right in the middle.
You can see some brightness
and almost studding of the pericardium.
And then of course, this mass
that's actually growing out in the upper right hand
portion of the screen.
This ended up being an adenocarcinoma.

Differential Diagnosis of Metastatic Tumors

The differential diagnosis of metastasis to the heart,
is wide, includes vegetations, foreign bodies, thrombi
perhaps most commonly.
And of course thrombi occur on the left ventricular apex.
Most commonly in patients with
low left ventricular function, especially with aneurysms.
And of course in patients with, atrial fibrillation,
it can have left atrial appendage
and right atrial appendage thrombi.
Wall motion abnormality obviously predisposes to stasis
and thrombotic formation as does low ejection fraction.
Thrombi can be pedunculated or mural
and can show up basically the same way
that any tumor can show up.
Maybe with the exception of papillary fibroelastomas catheters
and contact lesions can lead to intracardiac thrombi
and of course thrombi can have embolic potential depending
as, we shall see later, even more
so on size and mobility.

Non-Tumor Masses and Mimics

There is also a long list in the differential of things
that are just not tumors,
but can often be mistaken as such in the right atrium,
these can include the Chiari network, eustachian valves,
the crista terminalis of course catheters and leads.
Pectinate fatty infiltration of the tricuspid valve annulus
and venous varices have been reported in the left atrium.
The heart transplant suture lines can sometimes show up
as intracardiac masses.
The fossa ovalis can look like a mass coronary sinus
can look like a mass.
The left upper pulmonary vein
and left atrial appendage ridges can appear to be mass like
and of course inter atrial septal aneurysms when seen on
echo can look like masses on the valves.
We already discussed this,
but vegetations Lambl's excrescences.
And then of course just the normal nodules
of Arantius, the right ventricle.
The moderator band can sometimes be mistaken for mass,
especially if it's not well seen.
And the right ventricle does tend to be heavily trabeculated endocardial fibroelastosis can also be very mass like
and fill the right ventricular apex.
Reverberation artifacts are fairly common
in the left ventricle.
False chords, papillary muscles,
trabeculations are all normal structures.
Thrombus as we mentioned, hypertrophic cardiomyopathy
and non-compaction, pericardial cysts, pericardial fat,
and then of course even hiatal hernias
and lung atelectasis can look like masses.
This is an interesting example of a Chiari network.
It has a basket like appearance,
which is fairly unusual.
Normally they look like just thin rope like structures,
but Chiari networks have been reported in 1.5
to 3% of adults.
They have been described as embryologic
remnant in a continuum with eustachian valves
and fill right atrium,
they can be sites of thrombosis.
You can imagine that a thrombus can form within this basket
and then go somewhere afterwards.
This is another finding which looks very mass like you can
see in this transesophageal view.
Right in the middle there is this
rather bright structure which appears to be
arising from the lateral annulus of the tricuspid valve.
Above we can see the right atrium
below is the right ventricle.
This is fatty infiltration of the tricuspid valve annulus.
It is a normal structure.
Cysts have also been reported in the heart hydatid cysts of course can show up in the liver but also in the heart.
It's been, found more often in Greece and
or at least reported more often in Greece and Turkey.
Intracardiac findings less than 2% on autopsy series.
So it is actually quite rare.
They can show up in the LV free wall.
The septum, the right ventricle
and the pericardium tend to be septated
and can be calcified.
And of course, even though they're technically benign,
they can lead to rupture dysrhythmias
and they can embolize Pericardial cysts are somewhat
considered normal structures.
They can be rounded adjacent to the right atrium
as the most common location and they can be asymptomatic,
but their potential for causing problems is in the fact
that they can compress heart structures.
Blood cysts tend to be congenital.
They're actually rare in adults.
They appear on lines of closure of valve endocardium
and they can be well circumscribed
masses with very thin walls.
Here's an example of a hydatid cyst,
or two at least hydatid cysts that are seen within the heart.
And this is a pericardial cyst.
You can see how it's compressing the left ventricle
and here are some blood cysts.
These are not necessarily arising from the valvular
structures, but they're very disturbing in appearance.

Diagnostic Techniques for Intracardiac Masses

One of the main issues that we deal
with in echocardiography is the fact
that we can miss intracardiac findings.
And in fact when we're looking at
specifically intracardiac thrombi,
the transthoracic sensitivity can actually be as low as 75%.
This is especially the case when we're considering small
and laminar thrombi when there's poor image quality
and when there's foreshortening of the ventricle,
of course we can also overcall findings
and we already talked about a lot of different artifacts
and normal structures.
There's actually been a scheme proposed
to identify artifact versus mass
and just to distill it for you, if it has defined borders,
if it has a distinct acoustic texture,
if there's an associated wall motion abnormality,
if it's present in greater than two views,
if it's present throughout the entire cardiac cycle.
And if you can pretty reliably say
that it's not a papillary structure or trabeculation.
And then of course if you administer echocardiographic
contrast to light up the ventricular cavity
and it shows an area of defect, this might be consistent
or is more consistent with an actual mass, be it thrombus
or tumor rather than an artifact.
Now there's some other things you can do aside from giving
contrast, which is the example we see in the lower right
hand side of the screen right now.
Contrast filling the left ventricle
and showing this dark contrast void at the apex
of the left ventricle, which is consistent with a mass.
We can also perform high frequency
transducer, echocardiography.
It has a short focal length
and can resolve images better at the apex.
We can also perform off axis apical imaging looking
for these structures.
We can also do color doppler imaging
and then perhaps the most powerful thing is to look
for change in the characteristics
or the resolution of the thing that might be a mass.
Over time, three dimensional echocardiography has
been proposed to be helpful.
Mostly in that you can see several different imaging planes
all at once and differentiate, between cardiac mass.
The problem of course is
that three dimensional echo has a relatively low frame rate,
so you in fact may miss things or see even more artifact.
And as we already mentioned, contrast can be used
to opacify the left ventricular cavity
and delineate the borders of a mass.
And as we shall see later, contrast can also be used
to check for perfusion of masses to help
with the differential diagnosis.
Other echo predictors
of finding a thrombus within the heart,
and these are specifically related to patients
who have had a heart attack,
include wall motion abnormalities,
a low ejection fraction less than 35%,
a large left ventricular end systolic volume index greater
than 35 milliliters per meter squared.
Indexed of course to body surface area
left ventricular aneurysms
or dyskinesis restrictive filling,
interestingly has been noted to accompany the formation
of thrombus within the heart,
as has a myocardial performance index, the so-called
Tei index performed by tissue doppler imaging.
And that gives us a different, scale.
So that's why the,
myocardial performance index here cutoff is
greater than 0.6.
Echo predictors of embolization
of LV thrombi include mobility and then whether the tumor
or thrombus, protrudes into the cavity or is pedunculated.
And this is in contradistinction
to mural thrombi which tend not to go anywhere
if it changes over time, if it has architectural
homogeneity, especially an absent center, which might signal
that the thrombus is active, that there are areas of
necrosis and other areas that are,
that are unstable adjacent wall motion.
And this time it's actually hyperkinetic wall motion.
As you can imagine, if there's a mass within the heart
and there's an adjacent segment of the myocardium that
because of its hypokinesis continues to hit that mass,
that mass might be more likely to embolize size.
Interestingly, and low swirling density echoes have also
been noted, but probably are not quite as powerful as some
of these other bolded issues or characteristics.
40% of embolic risk in fact can be explained at least in
some series by mobility
and the shape, seen on echocardiography.
Here's just some examples of mural versus pedunculated
and in this particular case,
there's both in the same patient.
The upper left hand corner, we have a short axis view.
Here you can see in the, at about a 10
or 11 o'clock, there's a mass
that's protruding into the left ventricular cavity
and the mid axis view.
Interestingly enough,
it looks like this patient may have non-compaction.
You can see a large, area
of non-compacted myocardium relative
to the compacted myocardium.
On the lower left hand corner, you can see
that there is a component of this that is mural that is,
villous and stretching into the cavity.
It's very large, but it is in fact mural.
But then right next to it, right in the middle
of the left ventricular cavity, near the apex, there
is a ball like structure, which represents the pedunculated,
characteristic of, another portion of this mass.
And then on the upper right hand corner, it's pretty,
it's more easy to see the pedunculated, aspect of it
and the lower, right.
You can also see the large nature of the,
mural thrombotic component.
Now here's an example of an echocardiogram
and I have to apologize for the foreshortening,
in the, right image
and the image in the center on the bottom,
but it appears that,
there's really not much going on here as far
as masses within the left ventricular cavity,
despite the low ejection fraction.
But when we perform off axis imaging,
you can see there is a mass at the apex
and it's protruding into the left ventricular cavity.
This is an example of how we need
to be performing off axis imaging to get all
of our imaging planes in to look for these masses.
When we do the off axis imaging,
we can better define the border.
We can see the full extent of protrusion
of the mass into the left ventricular cavity,
and of course we can see the full extent
of mobility of the mass as well.
Here's another example here.
You can imagine that this patient would definitely have a
predisposition to forming a mass with
or thrombus within their heart.
You can see the apical four chamber view in the upper
left hand corner there.
There's a massive aneurysmal dilatation
of the mid ventricle all the way to the apex
as shown in a two chamber view,
somewhat zoomed in the upper right hand corner.
And then the short axis view is
perhaps the most striking at all.
It almost looks as if there are two
ventricles short axis side by side.
In this particular case, it's an enormous
anterior apical aneurysm.
Now. One of the things that we have a problem
with is determining whether there could in fact be small
thrombi within something like this
if this patient had had a gastrointestinal bleed
or some other reason why it would be
dangerous to anticoagulate the patient.
Although we'd like to, it would be important to know,
if there was in fact a thrombus
and anticoagulation was absolutely necessary.
And also give us more information about the
characteristics of the thrombus.
And this is where contrast comes in.
These are contrast images, roughly the,
the same images on the two top panels as we saw in the last,
and then another example of an apical four chamber
with a little bit brighter contrast in the bottom panel.
And we can see here that in fact there's contrast swirling.
There's definitely stasis,
but we don't see any protruding, masses in there.
We do, however, and this is best illustrated in the bottom
panel, see that there is a mural thrombus layered in the
left ventricular apex.
Other techniques, of course we can use TEE gives us
better resolution.
We can see smaller tumors on TEE
CT CAT scan and MRI resolution has getting better.
It's probably better than this now,
but traditionally five to 10 millimeter sized tumors making,
echo in general still better at
visualizing the masses.
CT and MRI are better.
However, at tissue composition, we already saw examples of
that when we looked at lipomas.
The other thing that we can use,
although using less now
that there are concerns about radiation,
is filling defects on radionuclide angiography
and also angiocardiography.
Of course there is the risk
that when you put a catheter near the thrombus it's going
to go somewhere, but angiography can help us identify blood
supply and the vascularity of a tumor.

Treatment of Cardiac Masses

Let's talk a little bit about treatment.
A lot of things have been tried,
but surgery's really the mainstay for most of these tumors,
especially those that are malignant.
It's usually curative for myxomas
and fibroelastoma benign tumors
and it's usually not for vascular malignant tumors,
although it can be palliative.
It is important however, to remember that
surgical treatment should be performed as soon as possible
because the risk of embolus
and sudden death is high even in benign tumors.
In fact, 8%
of myxoma patients in one series died while they were waiting
for their operation.
Operative mortality in general is pretty low for myxomas,
it's probably,
the upper level is probably lower than this.
Now, however, we one must remember
that conduction system can be damaged
after surgical resection.
There can also be septal and valve damage that occurs.
And of course, perhaps the tumors have not been
completely resected.
Transplant has been used
for unresectable tumors without metastasis
and for benign tumors, the survival range is decent,
obviously not as good as for patients who are,
transplanted without tumors.
But for malignant, it hasn't been that good.
In one series, 14 out
of 21 had recurrence in death from metastasis at a
mean of only 12 months.
It's been recently proposed that you can cut out a lot more
of the heart and get wider excisional margins if the patient
can be placed on mechanical support.
And this might actually facilitate some surgical resections
of tumors, in the future,
although it is probably not widely performed at this point.
Intraoperative, TEE
of course can be very useful in guiding the excision
of cardiac masses.
It can obviously diagnose immediately the sequelae
of mass removals.
As we mentioned before, damage to cardiac structures
and of course residual tumor can be seen in some cases.
Defects created in the inter atrial septum in the,
IVS have been particularly helpfully, seen if looked
for carefully and valve function can be assessed.
Chemotherapy is very limited in cardiac tumors.
As I mentioned earlier, there has been some reports
of success, but most of the sarcomas are generally treated
with anthracyclines.
And of these of course cause cardiotoxicity in an already
compromised heart, especially after surgical resection.
This may not be the best thing.
The role and efficacy of chemotherapy
and radiation therapy have been poorly defined,
therefore in most of these tumors except in primary cardiac
lymphomas,
which has really increased the average survival from one
month in untreated patients
to five years in treated patients.
However, we have to remember
that even x-ray therapy has its costs
and the heart is very sensitive and radiation can produce cardiotoxicity.
Anticoagulation is obviously, very important.
Chemical treatment for intracardiac masses
that are thought to be thrombi.
Of course, big and embolizing thrombi
that have already gone somewhere
or are highly likely to should go to the OR
for surgical resection even if it's not
technically malignant.
And there is this issue of what to do
with organizing thrombi
because thrombi eventually will grow, blood vessels
will become part of the ventricular wall
and lose its embolic potential.
And of course, especially for primary cardiac tumors
that are malignant or for metastasis, we should keep in mind
that hospice is a very important treatment option.

Interactive Case: You Make the Report

So now let's talk a little bit about the differential
diagnosis and look at one particular case.
And this is where you get to make the report.
So think in your minds about how you would define
and report out this finding.
You see this as apical four chamber view.
There's one rather striking finding
that most everyone should pick up,
and then there's a more subtle finding.
So to tell you the way I would look at this,
in the left ventricular apex, there is a very large mass.
It's accompanied by a wall motion abnormality.
In fact, I would characterize it as akinesis of the apex
with, some brightness in the myocardium
and probable thinning.
This mass is not terribly well circumscribed
and it is partially mobile mostly due
to the adjacent cardiac motion.
I think everybody can pretty much pick that up.
Now what we've done is we've administered
echocardiographic microbubble contrast
and it's lighting up the ventricular cavity.
And what we can see is
that this large mass is being highlighted.
We can see the borders quite well
and it also helps us to define the wall motion abnormality.
And one of the things that I want you to notice is
that there is no contrast within the mass at all.
And this would suggest that the mass
actually has no blood vessels in it or has very few.
So now we have a closeup view
and it's focusing on the other finding here.
The one that I said that was more subtle,
and this is in the extracardiac space.
Here you can see the contrast that is lighting up this mass
that's outside of the heart.
You can see it right there. And then we give a high
mechanical impulse that's that flash of white on the screen.
It destroys some, but clearly not all of the microbubbles.
And then you can see them flowing back into this big tumor
mass that's there outside the heart.
You can actually almost see the individual clumps of bubbles
that are perfusing through this large mass indicating
that in distinction to what's composing the mass within the ventricle.
This is highly vascular.
Now, with some of the new echocardiographic techniques,
we can actually perform some quantitative analysis
using video intensity detection software to actually look at the relative,
video intensity and
therefore the perfusion of the different structures.
And so what we've done here is drawn what we call regions
of interest in the mass
that's within the left ventricular cavity,
and that's the light blue circle.
We've drawn another one that's colored red
and this one is in the myocardium in the
distal lateral wall.
And then finally we've drawn another region
of interest within the mass that's extracardiac
and we can graph this video intensity over time.
And so on the left hand axis we see pixel
intensity in decibels.
We see time on the horizontal axis
and we've labeled the extracardiac mass
myocardium and intracardiac mass.
As I mentioned before, we can see the intracardiac mass
fails to show any increase in pixel intensity over time,
and in fact is just completely flat in its video intensity.
The myocardium shows an increase in, pixel intensity
and then it levels off as does the extracardiac mass.
But the extracardiac mass has an increase in pixel
intensity earlier and it levels off at a higher level.
And this would suggest
that the extracardiac mass has more blood supply,
more blood vessels, higher vascularity than the myocardium.
And that's seeing a lot because myocardium is a
highly vascular tissue.
If we look at the pathologic specimen of the mass that's
outside the heart and compared to the myocardium,
which is the small box up in the upper left hand corner,
we can see when we stain with, for CD 34
that there are these brown circles here.
And in fact, when we perform quantitative analysis on the
pathologic specimen, there is higher vascularity,
and this was consistent with a metastatic adenocarcinoma
as diagnosed on pathology.
Do not have a comparable, assessment
of the intracardiac mass
because let's face it, it's pretty clearly a thrombus
and this patient went to hospice.

Echocardiographic Hints for Malignancy vs. Benign

So now let's look at some other potential ways
to diagnose echo malignancy versus benign.
I want to emphasize here that it's not gonna be definitive
until we have the pathologic specimen.
We can't tell for sure whether something is malignant
or benign, but in some cases
echocardiography can give some very helpful hints, at least
to give us a sense of whether the mass we're seeing
is a malignancy and
therefore pertaining a very poor prognosis versus a benign
tumor that has a better prognosis.
Right-sided location tends to mark malignancy.
Not always the case, but that is part one
of the things that we can use.
And that's mostly for metastasis, pericardial effusion
and pericardial mass for exactly the same reason.
There are exceptions as we've gone over,
but again, if it's in the pericardium, we have to suspect
that malignancy is what it is.
If it does not look typical of a normal structure,
rather obvious if there's evidence
of invasion into the myocardium or adjacent structure.
And part of that can involve alteration of function
of the myocardium tethering wall motion abnormalities.
And then of course, if there's a mass adjacent to the heart
and a mass inside the heart, that's usually indicative
that this is gonna be a malignancy.
And of course, as we already showed, if it's highly perfused
with contrast perfusion imaging,
and here's just an example of a tumor that's
outside the heart, it is absolutely enormous
and this is gonna be much more likely to be a malignancy.
Here's an example of a mass at the left ventricular apex
near the septum.
If we give contrast,
you can actually look at the myocardium.
It's down here, contracting normal myocardium.
It actually lights up a little with contrast.
You can see the mass does not light up at all.
Contrast also helps us see there's a wall motion
abnormality with thinning there.
This of course, is gonna be much more consistent
with thrombus from the get go,
but if we actually perform our pixel intensity analysis
of the mass and the adjacent myocardium,
we can see the myocardium is perfused, the mass is not.
And of course it's consistent with thrombus.
So I apologize that this,
echo is not playing very well,
but hopefully you can identify in the right ventricle.
There's this enormous mass and it is extending there.
This is a modified, apical three chamber view
with visualization of the right
ventricle, absolutely enormous.
The other picture,
which you can't see, doesn't add a whole lot.
This ended up interestingly being a myxoma,
it's very, very large.
It's right sided. This tumor,
and again, we're gonna have difficulty with one of these,
pictures, but it doesn't add a whole lot.
This is a transesophageal echocardiogram.
See the inter atrial septum up here.
So the mass is nowhere near that inter septum.
It's actually down near the free wall of the right atrium.
Tricuspid valve is down on the
right hand side of the screen.
This one would think would be something consistent
with the thrombus, but in fact it is a thrombus, excuse me,
it would be something consistent with myxoma.
But it turns out that this actually was the thrombus.

Review and Conclusion

So let's review myxomas.
Primary malignant cardiac tumors are two primary cardiac
tumors that we discussed, and for all intents
and purposes, the benign ones are myxomas,
the malignant ones are sarcomas.
Then there's metastasis that we discussed.
There are non-tumor masses, which always have
to be kept in mind even though it's a really,
really long list.
Diagnosis and treatment.
Treatment is generally not very good
and for primary malignant tumors and metastases.
But echo can provide some really key
and important diagnostic tools.
So I wanted to thank you very much for your attention.