- Good morning and again I'd like to welcome you all
to this conference and to Ann Arbor,
and I hope you enjoy your time here.
My talk is on perioperative factors and infant development,
and that will certainly be my focus,
but I wanted to included this slide from a presentation
that I gave earlier this year.
In part to highlight how important neurodevelopment is
from infancy across the lifespan.
And how everything along the way makes a difference
in outcomes for adult survivors of congenital heart disease.
And I know I'm preaching to the choir on that point,
but also the relationships and how neurodevelopment
is related to parental stress and vice versa.
And parenting behaviors contribute to neurodevelopment.
And how it's related to physical
and psychosocial functioning and quality of life.
And how it certainly impacts transition and readiness,
the readiness to transition to adulthood,
Not necessarily transfer.
And so how important it is,
including that perioperative period.
I know most of you are very aware of the
American Heart Association scientific statement
on neurodevelopmental outcomes in children
with congenital heart disease.
But a really important statement, I believe,
that is there in the twenty pages
of this important document,
is that survivors at risk for neurodevelopmental morbidity
caused by both biological and environmental risk factors.
And I hope that we can talk a little bit more
about those environmental risk factors
that may be more modifiable.
As described in the document,
some of the biological risk factors include
underlying syndromes or genetic developmental disorders
as you've heard about today,
which exist in up to 30% of children
with congenital heart disease.
Include the work of Dr. Gaynor and colleagues
on polymorphisms of the apolipoprotein E gene
and how important that is for neuronal repair.
The defect itself certainly circulatory abnormality
specific to the heart defect,
result in altered cerebral blood flow
which effects neurodevelopmental outcome.
And we know that there's an increased risk
of neurodevelopmental deficits associated
with greater complexity of congenital heart disease.
But the focus here on the medical
and certainly surgical therapies that are required
to treat congenital heart disease.
Cardiopulmonary bypass
and deep hypothermic circulatory arrest
may result in cerebral macroemboli and microemboli
to the central nervous system or a period
of global cerebral ischemia.
Although there are certainly several studies
that do not find necessarily a relationship
between bypass or circ-arrest
and neurodevelopmental outcomes.
But it's a potential risk factor.
We know that subsequent operations
with cardiopulmonary bypass during infancy
in addition to the neonatal surgery add to that risk.
Perioperative seizures related to the surgery
is a well identified risk factor.
The need for ECMO or Ventricular Assist Device
and the need for CPR.
But important one that I think I'm
gonna spend a little more time focusing on is
hospital length of stay
Or that exposure to the hospital environment.
This was a very important study from 2003.
So we've known this for a while
that hospital length of stay was very important
and related to cognitive outcome at eight years of age
in patients with transposition of the great arteries
who had undergone an arterial switch procedure.
And you could see that there are cognitive scores
on the vertical axis and the ICU length of stay
in quartiles so that the four is the longest
ICU length of stay.
And you can see the significant decline
in full-scale IQ and verbal IQ
and a trend toward a decline in performance IQ
in mathematics achievement related
to longer time in the ICU.
In the same...in the next panel
full-scale IQ, verbal IQ, and performance IQ
were all significantly related to a
longer hospital length of stay.
So something to that.
And they actually concluded that longer
post operative length of stays associated
with worse later cognitive function
even when adjusted for perioperative events,
profusion times, and sociodemographic variables.
Really important.
And they suggested that further research is necessary
to determine the mechanisms underlying this relationship.
And I'm gonna postulate on a mechanism a bit.
In a later study, Dr. Fuller and Dr. Gaynor and colleagues
looked at predictors of impaired neurodevelopmental outcomes
at one year of age, after infant cardiac surgery.
And again they found some of the factors
that we heard described already.
But postoperative length of stay
was a significant predictor
of psychomotor development indices on the Bayley Scales.
The need for preoperative intubation,
longer post-operative length of stay
and use of ECMO or VAD were all associated
with worse neurodevelopmental outcomes.
Well we recently published results of a study
that we did on infant motor skills
after a cardiac operation.
And the purpose of this study was to characterize
the motor status of hospitalized infants
after cardiac operations.
And we also were trying to develop and field test
the congenital heart assessment
of sensory and motor status metric,
that we've referred to as CHASMS.
Because Jenna Romano, one of our cardiac surgeons,
who also spends some time taking care of patients
in the ICU, was really concerned
about the declines in developmental skills
that she observed in those babies
while the were in the Intensive Care Unit.
And what we were doing,
and what we could do to make it better.
But she felt like first we needed to be able to measure it.
So we tried to develop a measure at the same time
that would help us to study this and learn more.
Most of the measures that had been developed
were pretty long and complicated
and had complex scoring.
This is an example of one.
Or needed to be administered by trained physical therapists
or occupational therapists and were really more conducive
to an outpatient setting.
So she wanted to do something that could be done
by a bedside provider.
So, that surgical support of something so important
is something that I want to acknowledge and appreciate.
So we had 100 infants,
who were evaluated
within the first 10 months of life,
following their cardiac surgery procedure.
In a third we were actually able to evaluate them
prior to their cardiac surgery.
A variety of diagnoses.
About a mix of STAT category.
For those who are not familiar with that,
is a categorization of surgical complexity
associated with increasing mortality.
So five is high complexity.
One is low complexity.
And there were about a third
that were category one and two,
a third that were category three,
and a third that were STAT category four or five.
And we found that there was a significant relationship
between CHASM scores
and
lower CHASM scores,
or lower motor skills were significantly associated
with being intubated for more than two days,
or being in the ICU for longer than five days.
So lower CHASM scores after adjusting for age,
again were associated with longer duration
of mechanical ventilation and ICU length of stay.
With similar findings based on the TIMP and the AIMS,
which are established metrics
that we use to validate our CHASMS.
So it wasn't just our little measurement
that created this result.
Of note, and this analysis was certainly prompted
by the reviewers of our manuscript,
we found no significant associations between motor skills
as measured by the CHASMS or other measures
and STAT category, single ventricle
or prior cardiac surgery.
So what was it about hospital length of stay
that was making this very significant association?
Well my contention is that the ICU hospital environment
is an environment of immobility.
And the more prolonged that immobility,
certainly it makes sense that you're gonna see
a more significant decline
in infant motor development and skills.
And in fact we saw decline from pre-op to post-op.
So, to me the answer then is we need to do things
to increase mobility during hospitalization.
Well what are some of the things that we can do
to increase mobility and then hopefully improve
neurodevelopmental outcomes in infancy?
One I think is early extubation.
Certainly extubation is associated with greater use
of physical and chemical restraint,
drugs to keep those babies from moving.
We need to increase the time they spend out of bed.
Increase parental holding,
which is gonna happen much more frequently
when those patients are not intubated.
Now I think not that long ago we would say
that that's one of those unmodifiable factors.
If they are intubated it's because they need
to be intubated.
Well the recent collaborative learning study
designed a clinical practice guideline.
And in the studies the centers that were randomized
to the early extubation protocol,
they saw a significantly increased rate
of early extubation with no change
in the rate of re-intubation.
So, we can do it.
And we need to do it.
Other things, we need to minimize excessive use of sedation
and Dr. Ranovsky talked a bit about that.
That's facilitated by removal of lines and chest tubes.
And I think we sometimes think we're leaving them in
as long as they need to be,
but sometimes we're not thinking about it so much,
and probably can think about it more,
and try and remove some of those lines and tubes earlier.
And of course non-pharmacologic pain and comfort measures
are really, really important.
I won't go into that more because I think you're going
to be hearing more about it.
Physical therapy referral.
I think we're gonna be hearing more
about what physical therapy can do
from one of our future speakers.
So I won't say more about that either.
And overall earlier mobilization and holding
and I think Amy's gonna be talking to us
about developmental care
and even the importance of positioning.
How often our babies' parents complain,
they're always frog-legged
or how many go home with torticollis.
And what we can do even with positioning
to improve motor development.
I want to end with giving a couple of case scenarios
that really make the point to me.
And these are babies that were part of our CHASMS study.
The first baby was a four-month old with Trisomy 21,
so a genetic anomaly,
who had repair of an atrial ventricular septal defect.
We administered the CHASMS on post-op day six.
In the prone position, he could lift his head to 45 degrees
between the head and chest.
In supported sitting position, he could maintain his head
in midline for greater than 30 seconds.
And he scored at the 50th percentile for age
on the Alberta Infant Motor Scale, the AIMS.
Note, the infant had been previously enrolled
in a study with a tummy time intervention.
And you're going to be hearing more tomorrow
from Dale Ulrich who has done a great deal
of research in infants with Down Syndrome.
The second patient was a four and a half month old
with Tetralogy of fallot.
So just two weeks older.
Had the CHASMS administered on post-op day seven.
In the prone position, he was inconsistently able
to turn his head from side to side
to clear his nose from the surface.
In support sitting position he had head bobbing.
And he scored at the 10th percentile for age on the AIMS.
And we made a referral to physical therapy.
Note, his mom reported that she was instructed not
to put the baby in the prone position prior to surgery
because it would put pressure on his heart.
I have no idea who told her that
and most often when I ask that question
they don't wanna throw anyone under the bus.
But look at the contrast in those two babies.
All related to something that is very behavioral,
very within our control to change and impact.
So, I believe that we can make a difference
in changing neurodevelopmental outcomes,
especially related to motor skills
in infants with congenital heart disease.
Thank you.
(applause)