"Questions and Answers with Gil Wernovsky, M.D."
Chat Topic:
Developmental Delay/Learning Disabilities
Gil Wernovsky, M.D., FACC, FAAP
Director, Cardiac Intensive Care Unit
Associate Physician in Cardiology
Children's Hospital of Philadelphia
Associate Professor of Pediatrics
University of Pennsylvania School of Medicine |
 |
Edited by: Mona Barmash
Posted: March 4, 1998
Updated: May, 2003
Allopurinol
Q. My son was in the Allopurinol study at CHOP and
I was wondering if you knew if the study was completed
and whether giving it helped prevent developmental
problems after surgery?
A. For those who don't know about the study, we
are investigating the potential benefit of a drug called
Allopurinol to help minimize postoperative complications.
The study is completed in terms of enrollment - about 320
families participated. The data are currently being
analyzed and I should have preliminary results in about 4
months. I'm afraid that’s how long it takes to get
some answers in these big trials! We hope to continue to
follow these children throughout school age, if we can
receive government funding.
- Q. What does the drug do?
-
A. Allopurinol was designed to treat gout.
It may reduce inflammation in the brain and heart
after the heart lung machine ("bypass")
is used during surgery.
Q. How would you know if your child was in the
study if they were treated at CHOP recently?
A. Before surgery, you would have been informed of
your child's participation, and you would have to have
signed "informed consent".
Incidence/Factors related to Developmental Delay
Q. How common is it for children with CHDs to
have developmental delays/learning disabilities?
A. That’s a difficult question to answer
because there are many different types of CHD. There
haven't been very many studies of children with CHD with
regard to developmental delays/learning disabilities.
These diagnoses are made in school age children for the
most part, thus, they had their cardiac diagnoses made in
the mid to late 80's. The way we took care of children
with CHD then has changed quite a bit, even in the last
10 years. Thus, what we know about children born in the
80's with CHD may or may not be applicable to children
born more recently.
Having said that, the incidence of developmental delay
is only slightly higher than the general population, and
to my knowledge, no one has looked at things like LD or
ADHD ("hyperactivity") in detail yet.
- Q. Are the risks of neurological and/or
behavioral consequences of open heart surgery
routinely discussed with parents?
A. I can't speak for everyone, but I think
that is part of what you should talk about when
you talk about "outcome". It's not just
the risks of surviving surgery; it's the entire
quality of life.
- Q. What specifically are known causes of
neurologic consequences for kids after open heart
surgery?
A. Much of what is being learned is really
pretty new data. It's only really been in the
last 10 years that we've had enough kids SURVIVE
complex surgery to reach school age so that we
can study them effectively. Now that more children
survive into school age, we are learning quite a
bit. We know that delaying surgery and leaving a
child with poor weight gain, ("failure to
thrive"), can have significant impact on the
development of motor skills. We also know that
although there is (in general) little effect on
IQ of long standing cyanosis (blueness or low
oxygen saturations), having low oxygen
saturations for decades DOES have some effect on
intelligence. Finally, we are learning some
things to improve the way the heart lung machine
is operated, and other factors which go into a
complex neonatal heart operation such as
anesthesia, postoperative care, etc., which will
probably give us better long term outcomes than
we saw in the mid 1980's.
- Q. How many kids have major neurological
problems after surgery? And is this decreasing as
techniques improve and are better understood?
A. The percentage of children affected is
VERY variable, based on the type of heart disease
and type of operation. The incidence has
decreased significantly over the past decade.
- Q. Do you see any correlation between the
length of time on bypass and any delays a child
may have?
A. This is a VERY active area of research.
During open heart surgery in newborns and
infants, the heart must be stopped to operate on
the large blood vessels leaving the heart, or to
close holes inside the chambers. The heart lung
machine (cardiopulmonary bypass) is used to
support the vital organs during this period. At
some point in the operation, the heart lung
machine is stopped as well. This is called
"circulatory arrrest". At 98.6 degrees,
the safe duration of circulatory arrest is about
5-6 minutes before there is brain damage.
In order to get around this, and extend that time
period, the body temperature is lowered to about 65
degrees or so. This is called deep hypothermic
circulatory arrest. Surgeons try to keep this period
of time as short as possible, but sometimes, it is
unavoidable in order to get a perfect operation done.
Much research in the last 5 years or so has suggested
that PROLONGED periods of deep hypothermic circulatory
arrest ("DHCA") are related to some abnormal
neurologic findings later. Exactly how
"prolonged" is "too long" is not
exactly known.
In addition, there are some very exciting preliminary
data with the use of new drugs to extend the period of
DHCA and make it safer. I must emphasize that DHCA is
necessary to make the operation successful in many
circumstances. We try to keep it down below an hour or
so, but that number is somewhat arbitrary.
- Q. How do they lower the body temperature?
-
A. During the beginning of the operation,
after anesthesia, the baby is placed on a cooling
blanket. After the heart lung machine is started
the temperature of the blood put back into the
baby is dropped to about 65 degrees F (16 degrees
C). This is perhaps the BEST way to protect the
brain during surgery because it allows the
circulation to be temporarily stopped so that the
surgeons can operate on a very tiny heart and can
see everything they need to.
- Q. What changes have occurred to improve
outcomes for our kids?
A. First, it was a philosophical change to
try to repair children as early in life as
possible to minimize the effects of unrepaired
heart disease such as failure to thrive and
prolonged low oxygen levels. Next, it was the
miniaturization and improvement in the heart lung
machine. Most children who get open heart
procedures in infancy have their body
temperatures lowered to protect the vital organs
like the brain. This is routinely done in all
neonates. We are learning better ways to
supplement that protection with drugs like
Allopurinol and others.
- Q. Is there any evidence to suggest that
kids who need more than one surgery are
significantly more delayed?
A. Children who need multiple operations
(like those with HLHS or other forms of single
ventricle) have many potential factors to
consider - not just the three operations
themselves. These include what the oxygen
saturation is like, the overall growth and
nutritional status, the psychological effects of
multiple doctor visits, etc. All of these add up.
Having said that, I've been looking at children
with complex CHD for about 10 years now, and I am
continually amazed at how well they seem to do
despite all of the potential risks. I think that
this says a lot for the type of support they get
from their families.
Behavioral Difficulties
Q. Do you think that the trauma of surgery affects
infants who have had surgery psychologically as they grow
up?
A. Yes, I do, but it is VERY difficult to
quantitate. Actually, as I'm sure you know better than I,
it affects the entire family. ANY type of chronic illness
can be associated with psychological stress.
There was a
very interesting study done at the Mayo clinic and
published about 6 years ago. Children who had reparative
surgery in the 70's and 80's tended to be OVER achievers
(maybe their docs told them not to do sports, so they hit
the books), but they did have higher than expected
incidences of behavioral difficulties such as obsessive
compulsive disorders, etc.
- Q. Do parents often report behavioral
difficulties after surgery- especially in
pre-school age children?
A. A lot of the time, a PC visit is filled
with more traditional "medical" issues
such as Digoxin, SBE prophylaxis and so forth.
Comment: So then it is not often discussed?
A. I think it is something that should be
brought up. I must admit, that if I don't ask the
question, may parents do not volunteer the information or
share their concerns. When I ask children in my office,
most children above the age of 6 or so have no recallable
memories of their surgery or hospital stay--of course
some do have VIVID memories, but they tend to fade over
time. Whether there are more "hidden" effects,
I just can't be sure.
- Q. Do you think the behavioral problems
are due more to the surgery or illness or the parents' attitude toward the child, like spoiling
them rotten? (Which is very hard not to do).
A. You've hit on a very important point -
ALL of this is inter-related. I'm sure if my son
or daughter had cardiac surgery, I wouldn't let
them cry for more than 2 seconds!! I’m
afraid that there really isn't any
"right" answer to your question. If
your child's heart has been fixed, and your PC
says its OK if she cries, then its safe. That
doesn't mean it's easy.
Developmental Screening
Q. Are developmental screenings typically
recommended for kids after open heart surgery?
A. I think that routine screening in the
pediatrician’s office are usually just fine. The
pediatrician should be aware that the child has had heart
surgery, and thus may be slightly more "at
risk".
- Q. Is there a particular subset of skills
that may be delayed more frequently than others?
For example, is it more common to see
gross/visual motor delays than speech/language
delays?
A. In general, the more complex the heart
disease, the more "at risk" the
patient. For example, children with single
ventricle are more at risk than those with a
simple septal defect. -
- We tend to see more delays
in speech and language; actually, EXPRESSIVE
language more than RECEPTIVE language (the
children typically understand everything, but
their ability to speak is delayed). Intellect is
usually normal in the great majority of children
with all types of CHD. Fine and Gross Motor
function may be just a little bit impaired.
- Q. What about kids with AV Canal who,
after repair, need later valve surgery? Many of
them will have Down Syndrome. Does that make more
problems in evaluation?
A. About 1/2 of children with Down's
syndrome also have CHD. This, of course,
complicates the assessment.
- Q. I would like to know how early you can
see any delays, or if it can occur after any of
the surgeries for complex problems, like HLHS.
A. HLHS is a bit more complicated since
there are (usually) 3 operations. Perhaps I
should explain a bit more about the interaction
between the brain and heart disease. -
- Children
with congenital heart disease (CHD) may be at
risk for brain problems for a variety of reasons.
Some children also have congenital structural
abnormalities of the brain. NOT treating heart
disease can cause problems, and the TREATMENT of
heart disease can cause problems. There are
significant advances being made in protecting the
brain during and after surgery. The outlook for
children born with congenital heart disease now
is significantly better than 5-10 years ago, both
in terms of their survival and their quality of
life.
- Q. Have there been any learning problems
associated with Supravalvular Aortic Stenosis
surgery, and how long does the operation take if
all goes well?
-
A. To my knowledge, there are no
associated neurologic or developmental problems
typically associated with this type of surgery.
However, some children with Supravalvular Aortic
Stenosis may have associated conditions like
Williams syndrome, which may be associated with
learning problems.
Lasix and Hearing Loss
Q. Can you tell us what the relationship/incidence
of hearing loss and being on lasix is?
A. The problem with the assessment of hearing is
that multiple medicines have been related to hearing
loss, especially those given in the CICU like certain
antibiotics, etc.In my experience, hearing loss is very
uncommon (and I prescribe a LOT of lasix).
- Q. Is it true that lasix related hearing
loss is more frequent with the IV variety, and
how fast it is given?
A. Yes. If given in large doses very fast
as an IV push, it can cause hearing loss. Other
diuretics such as Bumex can do the same thing.
Velocardial Facial Syndrome
Q. How soon are learning disabilities noticeable
in a child with VCFS?
A. Many of the children with VCFS will have CHD
such as tetralogy of Fallot, VSD or Truncus Arteriosus.
These children also may have problems with the palate and
speech and language problems may be present (even in
those WITHOUT CHD). I believe that ALL children with VCFS
should have comprehensive, multidisciplinary follow-up
that includes a cardiologist, speech therapist, genetics
counselor, etc.
- Q. Is a cleft palate always evident from
birth - if not, what kind of tests are done?
A. It's usually an evaluation by an ENT
surgeon.
- Q. Are you saying it's not always obvious?
A. Yes, sometimes it's what is called a
"submucous" cleft. It frequently is
associated with nasal speech.
- Q. What is the latest research with
tetralogy of Fallot and chromosome 22 as it
relates to developmental problems?
A. Much of that work is just being
analyzed. Patients with 22q deletion WITHOUT
heart disease can have speech and language
problems, and the combination of 22q deletion and
an open heart procedure may be additive in that
regard. The presence of a cleft palate futher
complicates the analysis.
Stroke
Q. How common is a brain hemorrhage in a newborn
after open heart surgery?
A. Bleeding in the fluid AROUND the brain is
common, especially in premature babies. Bleeding actually
IN the brain tissue is much more uncommon.
- Q. What would you say is the incidence of
stroke with CHD heart surgery?
A. For children with cyanosis (oxygen
saturations <95%) it's about 3-5%. For those
with normal oxygen saturations, it’s much
less.
Exercise/Therapy
Q. What kind of exercises are good to help build
up upper body strength?
A. It is important to emphasize that there may be
some limitations depending upon the type of CHD - for
example, isometric exercises (like weight lifting) are
not a good idea for certain types of CHD such as aortic
stenosis. If there is concern about upper body weakness,
a consultation with a physical therapist with a
prescription plan by your PC is probably the best way to
go. Also, activities to strengthen the upper body will
vary according to the child's developmental age.
- Q. Say that a child with a low heart rate
starts to be very active in his daily activities,
is there anything that should be watched - with
sats around 75-85?
A. Without knowing the specifics of your
child’s CHD, it’s probably a good idea
to check with your PC to see if periodically
performing a Holter monitor would be helpful (so
that the heart rate can be tracked DURING the
periods of increased activity). The types of
things to look for would include sweating, rapid
breathing, etc.
- Q. Have you found that children have more
problems with tummy activities such as pushing up
on their arms?
A. No, that's not something that I am
aware of. Most surgeons close the breast bone
with stainless steel wires and sometimes they can
become irritating.
- Q. Can you explain Apraxia & the
cure(s); how long is the cure process?
A. Of all of the developmental delays that
we have begun to recognize after open heart
surgery, delays in speech are the MOST common. If
speech is a problem, it is particularly important
that hearing is evaluated. I have had the
impression that many children with speech delays
had feeding difficulties after surgery, and it
probably reflects impairment in the same
oral-motor area of the brain. Fortunately, most
children have just delay, not loss of speech. If
hearing is OK, then usually by 4 years or so the
children have caught up. Speech therapy may be
very helpful.
- Q. With PT, OT and infant educator not
making much progress, should I be concerned? My
child has had a Norwood and Hemi-Fontan.
A. It is VERY common to have motor delays
and sub-normal growth after the Hemi-Fontan and a
short period of recovery afterwards. I wouldn't
be too concerned unless the delays last another 6
months or so. If your child is making progress
each month, s/he may just be following
"his/her own curve".
- For additional information, please see:
- Developmental
Milestones: Medline Plus
-
- Early
Identification of hearing impairments in infants
and young children
-
- Learning
Disabilities (LD) and Attention Deficit Disorder
(ADD)
-
- Screening
Infants and Young Children for Developmental
Disabilities
-
- Zero
to Three:National Center for Infants, Toddlers
and Families
References
Utens EM, Verhulst FC, Duivenvoorden HJ, Meijboom FJ,
Erdman RA, Hess J Prediction
of behavioural and emotional problems in children and
adolescents with operated congenital heart disease.
Eur Heart J 1998
Bellinger DC, Rappaport LA, Wypij D, Wernovsky G,
Newburger JW
J Dev Behav Pediatr 1997 Apr;18(2):75-83
Patterns
of developmental dysfunction after surgery during infancy
to correct transposition of the great arteries.
Wernovsky G, Wypij D, Jonas RA, Mayer JE Jr, Hanley
FL, Hickey PR, Walsh AZ, Chang AC, Castaneda AR,
Newburger JW, et al
N Engl J Med 1995 Mar 2;332(9):549-555
Developmental
and neurologic status of children after heart surgery
with hypothermic circulatory arrest or low-flow
cardiopulmonary bypass.
Miller G, Eggli K, Contant C, Baylen B, Myers J: Postoperative
neurologic complications after open heart surgery on
young infants. Arch Pediatr Adolesc Med
1995;149:764-768
Bellinger DC, Jonas RA, Rappaport LA, Wypij D,
Wernovsky G, Kuban KC, Barnes PD, Holmes GL, Hickey PR,
Strand RD, et al
N Engl J Med 1993 Oct 7;329(15):1057-1064
A
comparison of the perioperative neurologic effects of
hypothermic circulatory arrest versus low-flow
cardiopulmonary bypass in infant heart surgery.
DeMaso DR, Campis LK, Wypij D, Bertram S, Lipshitz M,
Freed M
J Pediatr Psychol 1991 Apr;16(2):137-149
The
impact of maternal perceptions and medical severity on
the adjustment of children with congenital heart disease.
Brandhagen DJ, Feldt RH, Williams DE
Mayo Clin Proc 1991 May;66(5):474-479
Long-term
psychologic implications of congenital heart disease: a
25-year follow-up.
DeMaso DR, Beardslee WR, Silbert AR, Fyler DC
J Dev Behav Pediatr 1990 Dec;11(6):289-294
Psychological
functioning in children with cyanotic heart defects.
- This article was reviewed prior to publication
by:
- Alan D. Tong, MD, FACC
Cedars-Sinai Medical Center
Los Angeles, CA
Your feedback is very important! Please e-mail
us with any questions or comments about this article.
Return to Resource Room.
|
