The Emerging Recognition of School and Behavior Problems in Children with
Congenital Heart Defects
Gil Wernovsky, MD
Director of Program Development
and Staff Cardiac Intensivist
The Cardiac Center at The
Children's Hospital of Philadelphia
Professor of Pediatrics,
University of Pennsylvania School of Medicine
Advisory Panel, Congenital
Heart Information Network
April 16, 2007
(Journal articles added November, 2007)
September 6, 2011
Summary of Current Findings, 2005
Early Intervention/Special Education
Journal Articles (posted with the author's permission,
Outcomes in Patients with Surgically Treated Congenital Heart Disease
insights regarding neurological and developmental
abnormalities in children and young adults with complex congenital cardiac disease
Children with complex congenital
heart defects (CHD) are now surviving neonatal and infant surgery with a
frequency thought to be impossible only a generation ago. While advances in
medicine and surgery have allowed the ability to “mend” children born with
CHD, the increasing number of survivors has created a growing population of
children now entering our primary and secondary school systems.
In the United
States, over 35,000 infants are born each year with CHD, and more
than a third of these infants are expected to undergo temporary or corrective
surgical interventions in the first year of life. An estimated one million
adults are now living with a variety of congenital heart defects. This figure
includes over 150,000 adults living with complex CHD, meaning a CHD severe
enough to require surgery in the first months of life. There are even larger
numbers of children with complex CHD. As initial survival has increased
significantly, greater attention has been directed toward understanding the
long-term behavioral problems, academic performance and functional outcomes of
this growing patient population.
Much of the information I will summarize below is very new;
unknown to doctors and nurses 5-15 years ago. Many centers are actively
pursuing research in this area, which can be broken down into four main areas:
- What are the types of difficulties that our
children have, and how frequently do they occur?
- What are the causes of these problems?
- What are the best treatments for these
problems, and are medical therapies safe in children with CHD?
- How can we decrease the frequency of these
problems in the future?
What are the types of difficulties that our children
have, and how frequently do they occur?
a group, children with CHD have a higher likelihood of academic, behavioral and
coordination problems compared to children without CHD. This does not mean
that all children with CHD have these difficulties, but the number of children
is much higher than that seen in the general population. These problems
seem to be more prevalent in children with complex CHD: CHD severe enough to
require surgery in the first few months of life, compared to less severe forms
of CHD that either don't require surgery, can be repaired during a heart
catheterization (for example, an atrial septal defect or patent ductus
arteriosus), or don't require surgery until later childhood.
In infants, problems that are more prevalent in babies with CHD include feeding
difficulties (perhaps in half of all children requiring neonatal heart surgery)
and delays in reaching some motor milestones such as rolling over, crawling or
walking. Most newborns achieve full feedings by mouth shortly after
discharge from the hospital, but a small number require supplemental feedings
through a feeding tube into later infancy and beyond. While delays in
motor skills are extremely common, most milestones are only delayed by a few
months. Many hospitals now recommend speech and physical therapy
consultations after newborn heart surgery for evaluation and treatment
In preschool children who required surgery in early infancy, there is a growing
recognition of delays in certain elements of speech and language. To
greatly oversimplify, speech and language can be broken down into two
components: receptive language (the child hears and understands words), and
expressive language (getting the words out with proper grammar and
pronunciation). In most of our children with CHD, receptive language is
normal: if you say “point to the apple”, the child will point to the
apple. However, expressive language is delayed: when you point to an apple
and say “what is that?”, even though the child knows it's an apple, he or
she can't seem to find the word.
In addition to difficulties with expressive language, some preschool children
with complex CHD (probably less than one fourth) have continued difficulties with motor
skills, including large motor (clumsiness) and fine motor (drawing, cutting)
delays. In most children, these problems improve by the time they enter
As children with complex CHD are now entering primary and secondary school in
larger numbers, there is a growing recognition of a combination of problems that
combine to cause academic and social problems. Attention
deficit/hyperactivity disorder (ADHD) is a term that may be over applied in our
current society, and is an extremely complicated combination of learning and
behavioral interactions. Again to greatly oversimplify, the main
components are impulsiveness, easy distractibility and
hyperactivity. As we look more carefully at children with complex
CHD, various aspects of ADHD occur in perhaps one third to one half of children, which
is perhaps two to three times higher than that seen in the general
population. It is not clear if continued frustrations with speech
and fine motor (handwriting) control are related to the higher incidence of
ADHD—in other words, which is the chicken and which is the egg?
Two related issues are now also being seen in larger numbers of school age
children with CHD: problems with visual-motor integration and executive
planning. Visual-motor integration relates to the ability to coordinate
thoughts and images into action. In adults, for example, driving a car
represents highly complex visual motor integration. In school-age children,
however, learning handwriting represents a particular challenge: seeing
handwriting on the board, knowing that it's a particular letter, and getting
your hand to make the letter can be very frustrating to an otherwise bright
child. As you can see, handwriting is a common theme, and many parents
have told me that their child's handwriting is “one of the worst in the class”.
Executive planning is a feature that allows us to plan out a course of action.
For example, first I will get dressed, then I will go eat breakfast, then I will
pack my bag, and then head off to school. As the number of tasks
increases, it becomes increasingly difficult to coordinate. Some
children with CHD have a particular problem in this area, but the exact
frequency of this problem is currently unknown. Finally, there are no
studies as of yet to determine whether ‘higher order’ intellectual functions
(such as complex mathematics or learning a second language), or artistic talents
(such as painting or music) will be affected in our children with CHD.
What are the causes of these problems?
Although it is tempting to point at one feature as the cause of the problems
I've outlined above, that would be a terrible oversimplification.
We are only now beginning to recognize that the effects on the brain of children
with CHD are multiple, and cumulative over the early years of development.
conception, the preliminary portions of the brain form at exactly the same time
that the heart forms, in the first trimester. In most cases, whatever
caused the CHD has left its mark on the heart by the end of the first
trimester; complex CHD is essentially determined by the end of the 8th week of
gestation. In contrast, the brain continues to develop and mature
throughout pregnancy. We are just beginning to learn that the abnormal
circulation caused by CHD in the fetus may be responsible for brain
abnormalities present at birth. It is now recognized that congenital heart
disease and congenital brain disease go together in many children. However, it
is impossible to determine which is the chicken and which is the egg. Does
the same factor or factors that cause CHD cause brain abnormalities as
well? Is the brain “wired” the same way in children with CHD compared
to normal? Does the abnormal fetal circulation put an abnormal fetal brain
at greater risk? These are questions that are only now being investigated,
as technologies such as fetal and newborn magnetic resonance imaging (MRI)
become more routinely available. Recent studies suggest that the brain is either
structurally or functionally abnormal at birth in up to 25% of newborns with
complex CHD. The incidence of brain abnormalities in children with less
complex CHD is considerably less.
In complex CHD, so many things happen so quickly to the newborn, that it is
impossible to sort out the relative contribution of many of them to long-term
outcomes. These factors may include, but are not limited to:
- a low oxygen level after birth
- the effects of the heart lung machine (cardiopulmonary
bypass) during surgery
- the effects of poor function of the heart before
and/or after surgery
- seizures and/or stroke that may occur before, during
or after surgery
- nutritional deficiencies
- the effects of a long stay in the intensive care unit
In past research, much attention was paid to the way the heart-lung machine was
used: how long was the operation, how low was the body temperature during
surgery (hypothermia), did the circulation need to be stopped completely
(hypothermic circulatory arrest) during the most delicate part of surgery, etc.
While these factors are certainly important to the brain and later development,
we are beginning to realize that many other factors, both before and after
surgery, play perhaps an even bigger role.
A considerable amount of research has been done on the use of the heart-lung
machine (cardiopulmonary bypass) in general and hypothermic circulatory arrest
in particular. While it is beyond the scope of this article to go into all
of the details, the majority of studies have shown little to no effects for short
periods of cardiopulmonary bypass and/or circulatory arrest. It would be
naïve to point at a particular cutoff as being ‘too long’; if one chose,
for example, 45 minutes, does that mean that 44 minutes is ‘safe’ and 46
minutes is ‘dangerous’? Of course not. However; there is general
agreement that shorter periods of both cardiopulmonary bypass and hypothermic
circulatory arrest are preferable to longer periods, exactly how long and in
what combination continues to be a subject of research and
Finally, recent studies are suggesting that longer overall stays in the
intensive care unit and hospital as a newborn are associated with worse school
performance years later. This most likely reflects the cumulative
effects of the CHD and its treatment.
What are the best treatments for these problems, and are
medical therapies safe in children with CHD?
Unfortunately, the research into the treatment of the problems I've outlined
above is still in its infancy. It has been only in the past couple of
years that the scope of the problems of inattention, hyperactivity, school and
behavioral difficulties has been recognized. Prior to that time, there
were simply not enough children old enough who survived with different forms of
complex CHD for this to be fully recognized. Increasingly, physicians who
care for young children with CHD are now suggesting formal developmental testing
in infancy and the preschool years to identify potential problems and institute
therapies such as speech therapy, occupational and physical therapy, and to
recommend changes in learning environments that facilitate learning in children
with different strengths and challenges.
In my opinion, there is insufficient data at the current time to comment on the
safety or potential benefits of the psychotropic drugs (for example, stimulant
medications for ADHD, anti-depressants, etc.) in children with CHD.
Many of the psychotropic medications currently marketed for children with
structurally normal hearts slightly increase the risk of rhythm problems;
children with CHD are possibly at increased risk for rhythm disturbances when
using these medications. It must be emphasized that no large study has
determined how safe (or dangerous) these drugs are in children with CHD, or even
if they work the same way as in normal children. The decision to use medications
to deal with behavioral issues or ADHD must be individualized to the child,
balancing the unknown risks of these medications in children with CHD against
the lifelong implications of academic and social difficulties. Close
follow-up with the child's cardiologist is warranted when beginning any new
How can we decrease the frequency of these problems in
In my opinion, the outlook for our children with CHD remains quite
optimistic. There are certainly continued challenges in improving overall
quality of life, but not only are patients surviving in numbers thought
impossible one or two decades ago, but serial studies show improvement in many
areas of functioning compared with older children with complex CHD repaired in
the past. It must be emphasized that it is quite difficult to apply to babies
born in 2005 the reported results in current school-aged children (who were
diagnosed and treated in the 1980's and 1990's). This is due to improvements
in the last decade—including prenatal diagnosis, post-operative care, and a
better understanding of cardiopulmonary bypass. A number of additional factors
will ultimately contribute to the academic success of our children, including
parent and teacher expectations. It is highly likely that babies born
today will have better outcomes than those just a generation before.
best way to improve the outcomes for future generations is a continued
partnership between patients, parents, researchers, nurses and physicians.
Advocacy at the government level for continued research funding by physicians,
parents and patients is crucial to continue to pursue the causes and treatment
of heart disease in children, as well as the secondary effects on the brain and
quality of life. Philanthropic contributions play a significant role in
start-up funds for research as well. Finally, if families and
children are willing, voluntary participation in clinical research studies
remains the cornerstone of the process.
In the absence of an associated brain abnormality or
genetic syndrome, cognitive function (IQ, intelligence) is typically within
the normal range for most children with CHD
Parental IQ and socioeconomic status are most strongly
associated with IQ in CHD patients, rather than the type of CHD or its
Behavior problems, especially impulse control and
attention difficulties, are prevalent
Deficits in visual-motor integration and language
skills are common
Executive functioning—the ability to plan and perform
complex tasks—is impaired in some children
The association between open heart surgery and
postoperative care with later cognitive, language, or behavior difficulties
continues to be an area of investigation
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