Address for Correspondence:
Dr. Beena Johnson, MBBS, DCH, MD, PhD, Department of Child
Guidance, Baby Memorial Hospital, Kozhikode, Kerala, India.
Email: jiacam@gmail.com
Abstract
As more children survive with congenital heart diseases,
management of their behavioural problems are becoming
increasingly important. In this article we aim to review the
current status of knowledge on this aspect. Children with
congenital heart diseases have more behavioural problems
compared to children without chronic illnesses. Behavioural
problems in children can be classified into externalizing
behaviours and internalizing behaviours. Externalizing
behaviours are marked by defiance, impulsivity, hyperactivity,
disruptiveness, aggression and antisocial features.
Internalizing behaviours are evidenced by withdrawal,
dysphoria and anxiety. Boys with congenital heart diseases
have more externalizing problems compared to
girls. Preoperative hypoxia as well as peri and
postoperative cardiocirculatory insufficiency can lead to
internalizing and externalizing behavioral problems in
future. High treatment intensity and palliative
interventions are associated with poor behavioral outcomes.
Children who underwent open heart surgery at very young
age are prone to develop attention-deficit hyperactivity
disorder on reaching school age. A comprehensive approach in
this field is essential, so that effective early
interventions and guidance can be planned.
Key Words:
Behavioural problems, Children, Congenital heart diseases
Introduction
Children with chronic physical illnesses are vulnerable for
behavioural problems [1-4]. In the Ontario Child
Health Study, Cadman et al [5] did an epidemiologic survey of
3294 children and adolescents between 4 and 16 years of age in
the general community. They compared children having chronic
medical illnesses with those children free of such problems.
Results showed that children with chronic physical disorders
had greater than threefold risk for psychological disorders
and social adjustment problems. Janus and Goldberg [6] from
Canada, examined the behavioural adjustment of children with
chronic illnesses. This study revealed more behavioural
problems in children with congenital heart diseases.
Behavioural problems in children can be classified into
externalizing behaviours and internalizing behaviours.
Externalizing behaviours are marked by defiance, impulsivity,
hyperactivity, disruptiveness, aggression and antisocial
features. Internalizing behaviours are evidenced by
withdrawal, dysphoria and anxiety [7]. Children with behaviour
disorders are at increased risk for major depression, bipolar
disorder and substance abuse in future. Hence better
understanding and early management of behaviour problems in
children with congenital heart disease has important public
health implications.
Behavioural problems in Children with Congenital Heart
Disease
Research has shown that children with congenital heart disease
have more behavioural and emotional problems compared to
children from the general population [8-11]. Irrespective of
the severity of the heart disease, the congenital heart
disease patients exhibited more behavioural problems [8,11].
Kramer HH, et al [12] from the Department of Paediatric
Cardiology, University of Dusseldorf, Germany, compared 128
children with congenital heart diseases (CHD) and 89 healthy
controls and it was found that the cardiac patients had
increased feeling of inferiority and anxiety as well as
behaviour problems.
In the study conducted at Children's Clinic, University
Hospital, Oslo, Norway, Fredriksen PM et al [8] reported that
children with congenital cardiac diseases had significantly
more behavioural problems than did a reference population and
boys scored higher compared to girls. Analysis using Child
Behaviour Check-List showed a significant impact of physical
capacity on the score representing total problems, as well as
scores for externalising and internalising behaviour.
Fredriksen PM et al [13] also found that boys scored
significantly higher than girls in total problem score and
externalizing scores with more social problems, attention
problems, delinquent behaviour and aggressive behaviour. No
gender difference was found regarding internalizing problem
score. No effect was found related to different diagnoses.
Yang et al [14] studied the neuropsychological and behavioural
states of thirty nine Chinese children with acyanotic
congenital heart disease, of the age group five to fourteen
years. They identified greater behavioural disturbance in
children with acyanotic congenital heart disease, compared to
controls matched for age, educational level and social class.
Cyanotic Congenital Heart Disease versus Acyanotic
Congenital Heart Disease
Gupta S et al [15] from Alberta Children's Hospital,
University of Calgary, Canada, conducted a study on 39
children with congenital heart disease and compared them with
samples of normal children regarding the anxiety, fears,
depression and behavioural problems. There were 24 children
with cyanotic congenital heart disease and 15 with acyanotic
congenital heart disease. The subgroup analysis was also done
between children with cyanotic congenital heart disease and
acyanotic congenital heart disease. The Child Behaviour
Check-List - parent version was completed by mothers. Children
with CHD had increased fear and anxiety compared to normal
children. Children with cyanotic congenital heart disease had
increased fear, anxiety, depression and delinquent behaviours
compared to acyanotic congenital heart disease. The authors
concluded that even children with CHD not showing
psychological adjustment problems are still at risk for
behavioural problems.
Preoperative patients with Congenital Heart Disease
Preoperative patients with congenital heart diseases have a
generalized impairment in neuropsychological functioning and
these children can have anxiety, or impulsiveness [16] Utens
EM et al [17], found that the children scheduled for cardiac
surgery aged 2 to 3 years had significantly higher scores on
the Child Behaviour Checklist than did peers from normative
groups.
Congenital Heart Disease - Post-cardiopulmonary bypass
Patients with operated tetralogy of Fallot and transposition
of great arteries have special psychosocial problems and hence
they need multi-disciplinary care [18] O'Dougherty M et al
[19] conducted a study on 31 children with transposition of
great arteries who had undergone open heart surgery during
infancy. In this study, it was found that adverse
developmental outcome including behavioural problems was
significantly associated with medical risk variables such as
prolonged hypoxia, growth failure, congestive heart failure,
stroke and central nervous system infections.
In a systematic review on the psychological adjustment in
children and adolescents following open heart surgery for
CHD, Latal B et al [20], concluded that a significant
number of children who survived, have risk for psychological
problems and impairment in the quality of life. Non specific
effects of cardiac surgery with extracorporeal circulation can
lead to cognitive impairment, especially, problems with
attention [21].
In a randomized clinical trial, Bellinger et al [22] evaluated
155 children with surgically corrected TGA at ages between 4
and 8 years using the Child Behaviour Check-List - parent
version, Conner's parent rating scale and Conner's teacher
rating scale. The total behaviour problem scores were high as
per the Child Behaviour Check-List and the Teacher's report
form. This study gives evidence that children with
congenitally malformed hearts are at risk of developing
behaviour disorders.
Parents perceived a higher degree of behavioural problems in
children who had undergone cardiac surgery [23]. On assessing
the occurrence of behavioural problems of 125 congenital heart
disease children after invasive treatment, parents of
congenital heart disease children reported high levels of
behavioural problems compared to reference group. The
proportion of boys with congenital heart disease scoring in
the deviant range (21.4%) was significantly greater than that
of the reference sample (10%). According to parent report of
child behaviour checklist, higher scores were found for the
scales - somatic complaints, social problems, attention
problems, internalising and total problems compared to the
reference group [24]
Higher behavioral problems were also found in postoperative
school-age children with acynaotic congenital heart defects
[25] In this cross-sectional study using Child Behaviour
Checklist, 15 school age children who underwent surgical
correction of septal heart defect showed more withdrawn
behaviour, social difficulties, thought problems and
attentional problems compared to healthy controls.
Treatment Intensity and behavior problems
Janus and Goldberg [26] examined the behavioural problems in
patients with congenital heart disease; which were then
compared with that of healthy siblings. It was reported that
high treatment intensity was associated with elevated
behaviour problems in patients with congenital heart disease.
The neonatal arterial switch operation for transposition of
great arteries is associated with long term behavioural
impairment. In this study, Hovels-Gurich et al [9] analyzed 60
children with transposition of great arteries operated at
neonatal period with deep hypothermic circulatory arrest and
low flow cardiopulmonary bypass. These children were evaluated
at ages between 7.9 and 14.3 years. The parent reported Child
Behaviour Check-List total problem score was higher in these
children compared to the normal population. Preoperative
hypoxia and parent reported social problem were found to be
inter related. Children with peri and post operative
cardiocirculatory insufficiency had greater score on
internalizing, externalizing, attention and total behaviour
problems on Child Behaviour Check-List.
In the study conducted at Royal Belfast Hospital for Sick
Children in United Kingdom McCusker et al [27], evaluated the
relative effects of cyanosis and surgical interventions in
the behavioural outcomes of 90 children with cyanotic
and acyanotic heart diseases who underwent corrective or
palliative surgery. Children with complex congenital heart
diseases and who required palliative interventions had poor
behavioural outcomes. The authors suggested that secondary
prevention programs should be implemented at the earliest for
the at risk population.
Attention-deficit/hyperactivity disorder and congenital
heart disease
Attention-deficit/hyperactivity disorder is a common
behavioral disorder of childhood.
Children with CHD are at increased risk
for attention-deficit/hyperactivity disorder. Greater
prevalence of inattention symptoms are seen in children with
cyanosis or single ventricle physiology. Children
with preoperative hypoxemia in infancy due to cyanotic cardiac
defects are at increased risk for attentional dysfunction
[28]. In a study conducted at Children's Hospital of
Philadelphia, Shillingford AJ [29], examined a population of 5
to 10 year-old children who underwent newborn cardiac surgery
for complex congenital heart disease and assessed the severity
of hyperactivity and inattention. The number of children
receiving clinically significant scores for inattention and
hyperactivity on the Behaviour Assessment System for Children
was 3 to 4 times higher than observed in the general
population. Children who underwent open-heart surgery at
younger than 1 year of age are more likely compared to healthy
controls to have ADHD when they reach school age [30].
Parent ratings of inattention are significantly higher and
ADHD symptoms are more prevalent in children with congenital
heart diseases. There is increased prevalence of inattention
symptoms in children with cyanotic heart diseases [31].
Parenting styles affect the behavioural outcome of congenital
heart disease patients.
Conclusion
With the advances in the field of surgical management for
congenital heart diseases, there is increased survival of
infants born with complex congenital cardiovascular disorders.
Scientific evidence shows that children with CHD have more
behavioural problems than their healthy peers. These children
are at risk of long-term motor and behavioural problems.
Preoperative, operative and postoperative factors contribute
to the behavioural outcome. Assessment and management of
behavioural problems should be an integral component of the
comprehensive treatment of children with CHD. Therapies to
ameliorate the long term consequences must be implemented at
the earliest in children with congenital heart disease.
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