[MUSIC]
>> Thanks, Betty. Good morning everyone.
I'll be changing the focus to some of the opportunities and
challenges in adults with congenital heart disease.
I'd like to thank the EACVI,
for the opportunity to speak today.
These are my disclosures.
The adult with congenital heart disease ACHD is
a hybrid field serving patients whose needs are
not entirely met in
classic pediatric or congenital
cardiology and adult cardiology care models.
In ACHD, the anatomy of the congenital cardiac defect and
its structural variation exists alongside
pathology from hypertensive heart disease,
degenerative develop disease, or regional ischemia.
Cardiovascular imaging support in ACHD must navigate
the intersection of congenital
and acquired cardiovascular conditions.
Like a pediatric echo lab would,
it must provide precise anatomic information
and how surgery or [inaudible]
interventions would have modified it.
Like an adult lab would,
it must provide precise quantitation
of cardiac function and pathophysiology.
The North-American jackalope is
a hybrid like IDEA ACHD the echo lab.
It incorporates the features of both the antelope and
the jackrabbit and like the IDEA ACHD echo lab,
it's often dreamt off but seldom seen.
Our goal today is to hunt for that elusive creature,
the IDEA lab for ACHD.
We'll begin by reviewing some of the attributes of
the adult echo lab where the protocols are designed
to quantify the changes
and insults to which the adult heart is predisposed.
Even then these are additive,
as in the low flow,
low gradients circumstances of aortic stenosis, that is severe.
That will taken methodology capitalizes on
strong quantitative techniques like the continuity equation.
It's commonly misleading to assume normal ventricular function and
to use peak and mean
flow velocities for estimation of severity of stenosis.
Estimation of the actual valve area is the standard approach.
Adult protocols are not limited to
apical views but also include parasternal long and short
axes images and these standardized views are recorded in
a consistent fashion to complete
a full assessment of valves and myocardial function.
Sonographers have a highly disciplined routine to
optimize the images for measurement and
to assure that the functional assessments
and chamber volumetrics are accurate,
other labs have prepared to use
contrast injections that are often necessary for border detection.
Strain mapping provides a means to detect
segmental myocardial dysfunction commonly
encountered in atherosclerotic heart disease.
Patients with ACHD are not immune to this problem
and they are more likely to be affected as they age.
These are images of an ACHD patient who had
ultrasound contrast infusion and you can see
the improved visualization of
the left ventricle in endocardial border.
Some other labs use contrast for myocardial perfusion imaging,
which can detect not only a suspected epicardial corner issue
but also a supply-demand mismatch situation.
That is not uncommon in ACHD.
The right panel here shows contrast imaging as
stress when you see delayed replenishment of
the microbubbles in the myocardium manifesting as
extensive profusion defects in the septum and
apical lateral segments indicating it left corner issue.
So in summary for the patient in adult lab,
in terms of cardiac chambers and myocardium,
that measurements can answer for us how large, how thick,
and how vigorous,
and for the valves,
how leaky and how stenotic.
We have well-established standards for nearly all things we
measure in the adult echo lab and compared to the standards,
we can determine if there's a problem and if so, how severe it is.
This approach is broadly
applicable for acquired heart disease in adults,
but it relies on a big assumption.
Quantification and grading methods and
other echo assume anatomic consistency.
All the hearts, like all the rabbits
are built and oriented in much the same way.
But that's not true for pediatrics.
Performance of a pediatric echo
incorporates and approach design for
applicability across a wide spectrum
of anomalies encountered in congenital heart disease.
It makes no assumptions about an atomic consistency and always
begins by asking where
each structure is in relation to everything else.
Echo views, which would be unconventional in the adult echo lab,
takes on added importance in this context.
To illustrate,
consider how the question of visceral situs is addressed.
Many pediatric labs begins exam in the subcostal views as
shown on the left for determination of
the situs of the abdominal organs.
In the adult lab,
you might see an image like what you
see on the right displayed and keeping it
conventions used in the adult exam and a few labs might even use
specific adaptations to show that
transducer positions and allow interpretation.
Another point of confusion is how
the pediatric imageable display
the fetus structures at the bottom of the screen
and the superior structures at the top where is
the reverse is true for apical images obtained in our little lab.
Having the image accurately reflect
the cardiac structure within the chest is
important when the emphasis is on
definitely anatomic diagnosis but is unimportant,
then the emphasis is on functional quantitation.
Use of the sweep is another area in which the
approaching the Pete's Lab differs from the adult lab.
For example, consider the image acquisition
from the subcostal tensity supposition.
The probe is not sitting still,
but it's swept across a set of planes along
axis sweep is shown on the left
and the short axis sweep on the right.
The same is true for
parasite journal apical and suprasternal images.
It's important that the probe sweeps through
these planes to define the spatial relationships within the heart.
But it constructively normal adult heart sweeping
the transducer surface solid to
disrupt the standard image acquisition.
Pediatric echo cartographers even sweep through the structures in
the right highlight peristyle position as shown here.
From the other lab perspective again,
this consumes a lot of time that would be better
spent acquiring images for functional quantitation.
Indeed, acquisition of all these sweeps from
all these transducer positions is some heavy lifting.
This is published table showing
the transducer positions and structures in
a standard pediatric cardiac exam
and the long list is shown here just
to emphasize how complex the task is, it's time-consuming.
It depends on a high degree of sonographers expertise and often
requires direct collaboration between
the sonographer and the cardiologist,
so is it worth the effort?
As we study this images,
we learn a great deal about
structural anatomy and segmental connections.
We diagnose even the most complex and confusing defects
like abnormal situs,
congenitally corrector transposition, and also of
normal intraventricular relationships like
the upstairs, downstairs heart.
We can evaluate specific structures
that are likely to be abnormal,
like the coronaries, the pulmonary veins,
the branch pulmonary arteries, and the aortic arch.
We can also visualize
surgically relevent anatomy that may have a bearing on how to best
prepare a specific variant of a double outward like
ventricle or how a Fontan deletion can be constructed.
>> Pediatric images must always remain alert
to what they haven't learned from an echo codons on answered,
then they must recognize the need to pursue definitely than
atomic diagnosis with further tomographic imaging, MRI or CT.
In summary, the pediatric approach is applicable
across a wide spectrum of anomalies,
it requires many transducer positions and it made sweeps and
close collaboration between the cardiologist and
sonographers is required in complex cases.
Quantitative echo is a special challenge
in peads because of the broad
body size variability requiring
measurements to be expressed relative to body size.
Z-score calculators like the one on the right,
have been developed to support this task.
Branded adult and peads echo approaches are different,
but do they arrive at the same conclusions?
Harmonic tall, checked if applying
the adult and pediatric guidelines
resulted in concordant results on LV size.
In the last series of echoes in
adults without congenital heart disease,
there was agreement to lean 41 percent of
the males and just nine percent of females.
It appears we have an answer,
and the answer is no.
At this point, you may be wondering what it would take to serve
these ACHD population by
bridging the gap between the two approaches.
So far it appears we must reach
consensus on what images are necessary,
how they need to be acquired,
how they need to be displayed,
and the methodology for quantitation.
But there are at least a couple of other considerations.
First, when does the structural anatomy
modify the needs for functional assessment?
Second, how to address templated reporting.
We'll briefly consider the problems reporting
ventricular function in complex congenital hearts and
the challenges of doing so under the constraints of
reporting template designed for structurally normal hearts.
The trouble with templates in ACHD is
akin to the classic square peg round a whole problem.
We find ourselves trying to fit a square peg of complex
surgically modified congenital heart disease into the round plague
of templated reporting designed
around the structurally normal heart.
The report format does not allow
convenient reporting of critical anatomic findings and
also functional data that we
know doesn't make sense
because of the peculiar and atomic configuration.
I will illustrate this with two examples.
Hyper-plastic left heart syndrome and
congenitly character transposition of the great arteries.
Increasingly patients with their Fontan palliation
for single ventricles like
hyper-plastic left out survive into
adulthood and receive care in ACHD clinics.
In this setting, the mitral and aortic valves
are atritic or nearly so,
and the left ventricle is extremely small.
The result of a series of surgical paliations is
that the saturated central venous blood will flow
directly into the pulmonary arteries
without an intervening ventricle
while pulmonary venous blood causes
the atrial septum into the right atrium,
right ventricle reconstructed is in the aorta and the aortic arch.
Even though the integrity of the post surgical anatomy,
the novel flow patterns and
systemic ventricle are functional important considerations.
You'd be hard-pressed to adapt
an adult cardiology reporting template in echocardiography.
We'll have more to say about meaningful reporting of
ventricular function in my next example,
contingently character transposition.
In CCTGA, the left atrium connects to the right ventricle,
which connects to the aorta.
The right atrium connects to
the left ventricle which connects to the pulmonary artery.
Left ventricle is on the right
and the right ventricle is on the left.
How do we write a report in this condition if
the template is organized in
the usual way and displayed in the red box,
with the left heart and entry lines connecting to
the mitral and aortic valves and the right heart
asking about the tricuspid and pulmonary valves.
Ventricular function assessment is complicated as well.
Unlike a supplementary right ventricle,
the systemic RV has
a circumferential contraction pattern
and this peculiar anatomic characteristic
renders established RV quantification techniques
like TAPSE and systolic excursion velocity unhelpful.
Here the use of RV longitudinal strain and
fact fractional area change FAC are more suitable.
Of course, this problem is not unique to CCTGA,
gate-fold baffling procedures for
the treatment of standard transposition,
de-transposition also result in the systemic right ventricle.
Effective ACHD imaging depends on a successful synergy.
The International Society for adult congenital heart disease as
proposed in imaging protocol that's elaborate and comprehensive,
starts with subcostal views,
includes many quantitative techniques.
It's a nice synthesis of many of the things we have been talking.
Ultimately a hybrid echo protocol is the ideal we aspire,
but how we get there depends on where we are now.
Some of us are based in pediatric facilities,
others in adult hospitals.
Let's take a look briefly at
the road forward from each of these perspectives.
For those of us working in a pediatric Echo lab,
we will need to learn how to tackle
poor accostic windows and utilize quantification techniques.
Facility with echo contrast and transits aphasia, Lego,
and a lab culture that is
good to apply these tools will be required.
Expertise with
segmental wall motion analysis need to be developed.
Quantitative methods like annular tissue Doppler,
and atrial volumetrics need to be routine and accurate.
Similarly, quantitative methods to assess
valve dysfunction will need to be incorporated.
The scope of Echo lab support will
need to be broad and include adoption of
technologies and protocols that
supports structural interventions which
we heard in the previous talk,
which are increasingly performed in adult patients,
specifically for the mitral, aortic,
and tricuspid valve repairs and replacements.
For those of us working in adult Echo labs,
we need to learn to tackle complex anatomy and surgical history.
Incorporate an atomic sweep method and
standardized techniques for diagnostic and atomic complexities.
Sonographers will need to be more
comfortable applying an atomic classifications
for congenital heart and learn to show
venus anatomy and segmental connections.
Reporting templates must need to be flexible and should not compel
us to describe a right heart that is not a classic right heart.
Finally, we should think surgically,
routinely measuring parameters of surgical importance
like valvular diameters and pulmonary arterial sizes.
What are our take-home messages?
The ACHD Echo lab must
effectively address both are quite heart disease
the adults get and
the complex and atomic context in which this take place.
They draw both from the adult lab model with its emphasis on
precise functional quantitation and
the pediatric lab model with its medical as attention to anatomy,
the result is a hybrid which is greater than the sum of
its parts and relies on a high degree of cardiologists,
sonographers interaction and collaboration.
In conclusion, in hybrid specialty has unique
as ACHD deserves a hybrid lab to support it.
We aspire to standardization, precision,
and quantification while accommodating heterogeneous anatomy.
Wanted to acknowledge my colleagues who helped this talk,
and thank you for attention.