Hello, my name is Paul Freedman.
I'm Chair of the Department of
Cardiovascular Medicine.
And today I have the great pleasure of
being joined by Dr. Elizabeth Stevens,
associate professor of surgery,
who specializes in
pediatric and adult congenital heart disease.
Dr. Stevens, Thank you for joining me.
Thanks so much for inviting me.
It's a pleasure.
Today we're going to talk about
a topic that may be
less familiar to many cardiologists.
And that is vascular rings are slings.
So maybe we should start by simply saying,
what are vascular rings and slings?
Question? So Vassar rings and
slings refer to a group of diagnoses
in which faster structures either encircled
completely or partially encircle
the airway and the esophagus.
The vascular structures that we're
talking about would be
those branches coming off
the aorta as well as the Arctic arch.
So those, depending on the anatomy,
you can have the arch
going to the right or to left.
And then you can have
different directions in which
those vessels going to
the head and arms come off.
And if they're arranged in a certain manner,
they can cause either a complete ring,
so a complete circle and we use
the term ring for those, or a sling.
And a sling refers to a vessel
that snakes behind often the esophagus,
but doesn't and can cause
some compression or issues,
but it's not technically
a complete circle or ring.
I see. So how are these diagnose
and how do patients present and at
what age might we see them
increasingly being picked up
actually on fetal ultrasounds.
So not too many years ago,
maybe about ten years ago,
the standard ultrasound that
a mom gets included,
a view that looks at
the vessels coming off the aorta.
And so if there's
an abnormal branching pattern,
they get additional imaging.
So more and more we have
Fetal Diagnosis and when the baby's born,
we already had the diagnosis.
Now that is the contemporary era,
that's not the vast majority
and historically that was not the case.
And so the most
common way that they are diagnosed,
if you're not diagnosed fatally
would be through cross-sectional imaging,
usually a CT scan.
Now that CT scan could've been
done for completely different reasons.
So it was an incidental diagnoses or there
may be a patient who has been
struggling with vague symptoms,
often shortness of breath,
trouble swallowing,
and in that work-up they get a CT scan,
that diagnosis, the vascular ring.
The hard thing. And in terms
of that diagnosis is that
the symptoms are vague and they are most
commonly associated with other aetiology.
So shortness of breath is much more
commonly going to be COPD or
asthma or lung disease of other mechanisms.
And as much most commonly
going to be a vascular ring.
And similarly, issues with either
swallowing or
other types of dysphagia have
other mechanisms that are much more
common than avascular ring.
So I suspect then
they're often not really suspected
until someone orders a CT scan
and then they see the anomaly.
Is there a specific kind of scan
you would want to order if
you were suspicious,
if someone had unexplained dyspnea and
swallowing problems or other symptoms?
Well, something with contrasts
is best and often even if
the phase is not ideal for reasons,
we can still figure it out.
But CTA, where you wanna be able to see
the head vessels and
basically the ascending aorta and R-chart.
The key areas that you're looking at.
Interestingly, the degree of
airway compression and esophageal compression
is less less well
diagnosed or quantified on a CT.
This is often dynamic obstruction.
And either a dynamic study
or a bronchoscopy can help confirm that.
Or and then in terms of the esophagus,
actually barium esophagus I'm
including more solid foods is the,
are the most helpful.
But the anatomy itself
is best demonstrated with a CTA.
Got it.
Okay.
So any diagnostic study of
the esophagus or
the airways might give clues,
but the CT, at least in the adult,
is best for making that diagnosis, right.
What causes these vascular rings?
Well, we don't have any specific either gene
or a specific mechanism per se,
and it's also nothing
that has been associated
with either an exposure during fetal life,
whether that's a medication of food
or any other sort of exposure,
it does not run in families.
It does not cluster like that.
So we don't have a specific mechanism.
That said we do understand from
embryology how this goes
comes about. So if you
Remember, they aortic arches of embryology.
They're symmetric
and on the left and the right.
And during embryo logic development,
normally certain arches
involute and others remain.
And as that process occurs,
that forms our normal anatomy so on normally
a left aortic arch and
then the normal head branching pattern.
When certain vessel, certain
of those arches don't involute and others do.
That's when you get these different.
There's a pretty diverse actually pattern
of anomalies that you can see.
Now, there are a number of
those anomalies that don't
have clinical consequences.
So may be abnormal but
not either cause a clinical issue or Anita,
any sort of repair.
And then there are a group that are causing
clinical issue and we do repair.
So you've touched on the issue of repair.
So let's and I recognize that diagnosis is
often delayed or difficult
because the symptoms are nonspecific.
But person that whatever h gets
a CT or a diagnostic study
and the diagnosis is made.
What's the treatment and what's the approach?
How do you take care of this problem?
Right? So that does depend on
the specific type of vascular ring.
So maybe I'll start out
with one of the most common,
which is right aortic arch, left ligamentum.
And that half of those patients
also have an aberrant less subclavian.
So the anatomy,
if you want to try to picture
it, is the aortic.
Aorta is coming off of the Heart
heading towards the head and it turns,
goes around the esophagus
and the airway to the right.
As it comes around,
there is still the left ligamentum.
So if you remember,
the ductus is connecting
your pulmonary artery to
your aorta and is used during
fetal life and becomes this like a mentor?
Well, that's on the left side,
so that creates a complete ring
around the airway and esophagus.
And as I said, half of
these patients also have
an aberrant left subclavian.
And why that's important is
these patients can have something
called a diverticulum that left
subclavian is actually behind the esophagus.
And that carbonyls diverticulum is
a aneurysmal portion which
is at the base of the vessel.
So patients who are having
compression usually
have compression for two reasons.
One is that left ligamentum
that's creating the complete ring.
And the second is if they do have a chiral.
So there's that area of
aneurysmal artery
that is also causing compression.
So if you do have that anatomy,
you basically end up
breaking or releasing the ring.
And so in this, in that particular anatomy,
you would release
the ligamentum and then also
move that subclavian and get
rid of that aneurysmal part.
So in a younger child we do this to
the left chest is
a pretty posterior incision.
So as phi the scapula,
and we're actually able to move
the subclavian onto the carotid all
through the chest once
you know who comment here,
and that is a group
of blood vessels and ligaments at
essentially strangle the esophagus
and trachea within them.
The question is, how do you release
it? In young children?
It sounds like a thoracotomy
incision between the ribs.
The anomaly the most common anomaly.
Right.
And if they're fairly diagnosed for
doing that on the order
of one to two years of age.
The ribs are really quite
pliable at that point.
They recover very quickly and they don't have
the pain that older child
or adult would have of a thoracotomy,
then you minimize the amount of time that
your airway or esophagus has any compression.
In older teenager, adult,
this is harder to
perform all through the chest.
And so this is something that we
generally move the subclavian,
um, to the carotid and that's
done with our vascular colleagues.
And that's an incision in the neck area.
And then we actually
go through the chest and take care of
the portion of the aorta that
is that needs to be taken care of.
And so then that is also
a thoracotomy and
that's using partial bypass.
So in adults, it is a bigger operation,
it takes longer to recover.
But those are the two main strategies.
And those two approaches are part
of the same procedure or it's
a staged procedure or does
that very great questions.
So for for the latter example,
those would be a stage procedure.
We often do those a day
apart in some patients
where we want to decrease the anesthetic,
such as a teenager,
in particular where there could be
anxiety to undergo surgery twice,
we'll do it under one anesthetic,
but it's
technically two stages
there are back-to-back.
So I see.
So obviously incredibly
complex and specialized surgery.
A couple of questions
come to mind following that.
Urine astute clinician,
your patient has had shortness
of breath swallowing difficulties.
You get the CT scan, you see the problem.
The first question I would have is,
after the patient goes into surgery,
are there special postoperative things?
The cardiologists should watch
for to suggest there has been a problem.
What are the most common?
Potentially the complications are things that
require attention post hospital dismissal.
Why? To your point,
that's an excellent question.
One of the things that,
that I think clinicians should be aware
of is someone who's had a quote,
unquote vascular ring repair
potentially as a child,
may not have had a complete repair.
So if you have a ligamentum
division as a child,
you can still have,
for instance, as subclavian and
a diverticulum that's giving them symptoms.
So this is something that I've run into
a number of times where I think
it's confusing to clinicians
because they think that
they've had at Vassar ring
completely repaired.
The patient is reporting
symptoms and it seems to be
not consistent with their story.
So the first thing to note is
a ligamentum repair may
not have solves all your problems.
The second thing is if you've have had
a complete repair and that
includes what's called a
translocation or transfer
of that subclavian to
the carotid as well
as the aortic replacement.
Pretty unusual to have
issues with that portion of the aorta.
More commonly would be
standard kinda
peri-operative cardiac surgery.
Issues like wound that
you might be concerned about,
effusions, things like that.
We do get actually repeat CTA imaging
after the discharges to make sure
our anatomy is as expected.
I think those would be the main things.
Got it. Now, given how
complex and uncommon these conditions are,
what's our own experience at Mayo Clinic
in treating rings and sleeps?
Well, Dr. poker Latino, my colleague here,
has really done a lot
for adult vascular rings,
which I think would
have the largest experience.
And the country here at Mayo.
He has added so much to the field with that,
I trained in Chicago under
a very historic place
for investors, rings and slings.
And it was the eminent place
in the country for, for that,
which tended to have
more of the younger patients,
pediatrics and teenagers, young adults.
We are lucky that we
have so much experience in this.
We see a lot of referrals
from all over the country and
sometimes all over the world
who are asking for second opinions.
I think as such,
diversity and vascular rings
in terms of their anatomy,
what a patient might need,
that It's really a niche and they're just,
if you haven't seen a lot
or had a lot of experience,
it it, it does pay to just
have years of seeing
all sorts of types and
how best to repair those.
So we're very lucky in that Ricard to be
able to offer this for our patients.
One last question.
What happens if a vascular ring
or sling is untreated?
What sorts of complications do you see?
That's a great question.
In general, it does depend on the type.
So for instance, double aortic arch
is much more likely to have
significant consequences and they
tend to appear much younger.
Interestingly, and this is of course,
how we're made patients
really learn to compensate.
So when you meet patients who are older,
who have just had a
vascular being diagnosed, you may.
As the patient may mention, Oh, well,
I didn't I don't really like bulky foods.
I tend to like smoother foods.
On the last person to leave
the table at the family dinner table.
Those things because as
a child and as you grow up,
you learn how to compensate.
And so that means eating slower,
chewing your food very thoroughly.
Drinking water with food or
things like that to help compensate for this.
So there is overall this is
symptomatic relief that were
mostly helping with it's unclear.
Tracheal malaysia and bronco Malaysia.
We wouldn't expect it.
The earlier that's repaired,
the less consequences you would have.
There isn't good data with that.
We certainly do see
even children who get faster rings repaired,
who's still have Bronco and tracheal malaysia
that's significant and can
even need a tracheostomy.
But we don't have
good data to specifically say that when you
repair earlier you're going
to relieve and avoid those complications.
We do know in adults
that after Vassar ring repair there's
a significant percentage who
have intrinsic esophageal disease.
And so even though
the vast majority do have
a significant improvement in their symptoms,
there is a minority that
has some residual and it's
actually intrinsic
esophageal motility issues.
And of course, the question is, well,
is that because they've had
years and years of compression and
we there's so much that we don't
know that we're still looking to learn,
but hopefully that answers
some of what you're asking.
Well, Dr. Elizabeth Stevens,
Thank you for this fascinating
discussion on this
important and uncommon and
often late diagnose conditional.
It's nice to see that that's evolving
with improved intra-uterine imaging.
Thank you for joining me today.
It's been terrific.
Thank you so much.