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Cardiol Young 1995; 5:56-62

World Publishers Limited


ISSN 1047-9511

The impact of severe congenital heart disease on physical and


psychosocial functioning in adolescents
Per G. Bjornstad, Ingrid Spurkland and Harald L. Lindberg
From the Departments ofPediatric Cardiology, Child and Adolescent Psychiatry and Thoracic and Cardiovascular
Surgery, Rikshospitalet, The National Hospital, Oslo

Abstract A study was made of 26 adolescents, aged from 13 to 18 years, with different types of severe congenital
heart disease, assessing their somatic condition and its impact on mental health and psychosocial functioning.
Physical capacity was reduced, more pronounced in girls than in boys, along with an increased rate of psychiatric
problems. Associations were found between physical capacity and psychosocial functioning, and between psychosocial
functioning and chronic family difficulties. On the other hand, half the patients studied achieved fair function, both
physically and with regard to their mental health. The degree of reduced physical capacity is an important etiological
factor for impaired mental health in patients with congenital heart disease. This knowledge should be taken into
account when dealing with this group of patients. A good social network seems to be a protective factor. It will
probably be rewarding to give patients and their families not only an optimal medical follow-up, but also
psychological, practical and financial support to improve or create such a network.
Key words: Congenital heart disease; physical disability; mental health; psychosocial functioning; adolescents

HE LAST TWO DECADES HAVE WITNESSED MARKED

advances in all areas of cardiological medicine.


Improvements in diagnostic methods, anesthetic and surgical techniques, as well as postoperative
intensive care, have created a new group of patients:
adolescents and young adults with severe congenital
heart disease. Only a few years ago most of these patients
would not have survived beyond infancy or childhood.
Now they will require life-long follow-up.
Since the study of Landtman et al1 on psychosocial
functioning in patients with congenital heart disease,
several researchers have investigated this topic. Strangely
enough, a number of studies1'4 find little or no increase
in emotional problems. When they do, a consistent
relationship between organic severity and psychosocial
functioning is lacking. More recent studies, 56 however,
describe an association between the degree of physical
impairment and psychosocial functioning. The psy-

The project was financed by The National Hospital, The Child Psychiatric Clinic
at the University of Oslo and the Norwegian Directorate of Health.
Correspondence to Dr. Per G. Bjornstad, Pediattic Cardiology, Rikshospitalet,
The National Hospital, University of Oslo, Pilestredet 32, N-0027 Oslo,
Norway. Tel. 47-228 690 92; Fax. 47-228 691 01.
Accepted for publication 02 June 1994

chosocial functioning of this group of severely diseased


adolescents, nonetheless, has hardly been assessed. We
wanted to address this question from the stance of three
hypothesesfirst, that severe congenital heart disease
has an impact on psychosocial functioning in adolescents; second, that the psychosocial functioning is
associated with the degree of physical impairment; and
third, that adolescents with severe congenital heart
disease are more vulnerable to chronic family difficulties than those from the general population.
Material
According to the hypotheses, the degree of physical
impairment, and the possibility of its treatment, are
important factors that may influence psychosocical
development, rather than the specific anatomic lesion.
All patients aged from 13 to 18 in the register of the
National Hospital with our interpretation of the most
serious form of congenital heart disease were selected. A
total of 33 patients fulfilled our criteria which were:
severe cyanotic heart defect without surgical possibilities, with die potential or presence of palliation or with
repair or potential for repair including valvar replacement.

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Bjornstad et al Physical and psychosodal status in adolescents widi congenital heart disease
Table 1. Somatic diagnosis and latest type of operation in adolescents with severe congenital
cardiac malformation (n=26).

Diagnosis
None
Fallot's anomaly with pulmonary atresia or severe
stenosis requiring a valved conduit
Univentricular atrioventricular connection with or
without tricuspid atresia
Pulmonary atresia or severe stenosis with DORV
or complex transpositions
Ebstein's malformation
Severe left-sided valvar disease with valvar replacement
Aortic
Mitral
Total

Type of operations
Repair
Palliation

Total

10

_
-

4
1

_
-

4
1

18

26

DORV: double outlet right ventricle.

The patients included were all considered to have


reduced life expectancies. Events proved this suspicion
to be well founded. Three patients died within a year of
the completion of the study. Three patients refused to
participate. Two patients were excluded because of
additional physical problems (blindness, chromosomal
abnormality) that might have influenced the results.
One patient had emigrated. Another one agreed to
participate, but died from his heart defect during the
planning period of the project. The mean age of the
remaining 26 patients (16 boys and 10 girls), was 16.3
years. The age and sex distribution is displayed in Figure
1. Twenty-one patients had severe cyanotic disease
while five had mechanical left-sided valves. Somatic
diagnoses are grouped, and latest types of operation
summarized, in Table 1. The time for surgical interven-

16

17

18

;dBoys GirlsJ

Figure 1. Distribution of age and gender of the 26 adolescents


in the study. All of them had different, severe congenital heart
defects. Number of patients on the vertical, age in years on the
horizontal axis.

tion differed widely, but none underwent surgery withi n


the last year before the study. The data on growth and
history are summarized in Table 2. The hospital data
apply only for The National Hospital, and do not
include admittance to local hospitals.
Methods
Somatic assessment

All patients underwent separate clinical examinations


following a standard protocol by a pediatric cardiologist
and a cardiac surgeon. The history included a survey of
social factors, the judgment of their scars, and the
impressions of the family concerning the medical and
social support they had been given. General clinical
examination and routine electrocardiography, chest xray and echocardiograms were carried out. A standardized bicycle stress test was included. Hematological
parameters were studied in cyanotic patients. Cardiac
Table 2. Data on 26 adolescent patients (16 boys, 10 girls)
with severe congenital heart disease. The hospital data apply
only for The National Hospital and do not include stays in
local hospitals. Operations exclude non-cardiac surgery.
Parameter

Mean

SD

Range

Age (years)
Percentiles (height)
Percentiles (weight)
Days in our hospital
Admissions here
Catheterizations
Operations

16.3
31
47
80.0
7.5

1.7
24
36
46.5
2.7

13- 18
10<3 - 75
10<3-2>97
6 - 178
3 - 12

4.7

1.8

2-9

2.3

1.3

0-5

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Cardiology in the Young

catheterization was performed only when indicated for


treatment or counseling. This was done in 15 patients.
Due to the anatomical heterogeneity (Table 1), hemodynamic or anatomic parameters are not useful in
describing the group. We therefore chose to rely on
functional parameters. Both the cardiologist and the
cardiac surgeon classified twice and independently the
physical capacity of each patient according to the classification of the New York Heart Association.7 One was
based on the response of the patients, the second relied
solely on the information from the parents. Functional
class was also derived from a standardized bicycle stress
test. The test started with 60 watts for two minutes,
increasing with 30 watts every minute until the patient
gave up. We divided the maximal performance in watts
endured for more than 29 seconds by the body surface
area in meters squared. We used cutoff points at 50, 75
and 100 watts per meter squared. The class of the New
York Heart Association thus derived was used in all
calculations.
Psychiatric assessment

All adolescents were interviewed by a child psychiatrist.


This interview included the Child Assessment Schedule,8 a semistructured interview designed to provide
psychiatric diagnoses according to the Diagnostic and
Statistical Manual of Mental Disorders.9 It also provides a total score for pathology. Each interview lasted
from two to four hours and covered details concerning
the cardiac malformation, attitudes, feelings, expectations for the future, and so on.
At least one of each adolescent's parents was given a
semistructured interview lasting for two hours. It covered detailed information about previous and present
medical problems and psychosocial functioning, the
somatic and mental health of the other members of the
family, and how the family functioned and coped with
a disabled child.
The psychosocial functioning was classified according to the Children's Global Assessment Scale.10"12 Only
scores on the 10-level interval scale were used.
The mothers were asked to complete the Child
Behavior Checklist13 which covers different areas of
psychosocial functioning of the adolescent. It consists
of 118 items concerning problems with behavior and
emotion and 20 items concerning social competence.
Chronic Family Difficulties1415 were assessed, based
on all available information, including economy, housing, employment, network support and possible marital
or family discord, as well as the mental and social health
of the parents and other members of the family. Care
was taken to avoid the effect of the cardiac malformation itself. The test was scored on a scale from zero to six.
The highest value indicates the most severe strain.
The inter-rater reliability for the interviews for child

January 1995

assessment, global assessment and chronic family difficulties were checked and found satisfactory as reported
elsewhere by Bjornstad, Lindberg & Spurkland.15 Socioeconomic status was classified according to national
criteria.
Statistical procedures
In most instances non-parametric tests were used, because our data only rarely were normally distributed and
continuous. For correlations the Spearman rank correlation coefficient was used, with contingency tables and
Chi square test used to test binomial variables. Differences in paired observations were assessed with Wilcoxon's rank sum test. Group analysis was performed either
with the Kruskall-Wallis test, or Mann-Whitney's l i test. The parameters were entered into a data base in an
IBM 4341 computer. The calculations were made with
BMDP Statistical Software, version 1983 (UCLA, Los
Angeles, CA, USA). A statistical p-value 0.05 was
considered "significant," a value 0.01 "highly
significant," and lower values "very highly significant."
These terms are subsequently used in place of specific pvalues in the text.
Ethical aspects
In each case, written informed consent was obtained for
the study and additionally given separately for videotaping of the interviews for the assessment schedule.
The study was approved by the ethical committee of
The National Hospital.
Results
Clinical status
Eight patients were visibly cyanotic, while six had
clubbing. One was slightly cyanotic and the rest were
acyanotic. Whereas the mean height and weight were in
the normal range, some patients were very short and
markedly underweight (Table 2).
Seven patients had scoliosis, but none was in need of
treatment. Some sort of thorax deformity was seen in 11
patients, mostly pectus carinatum. Some of these patients complained about the shape of their thorax, but
again there was no indication for treatment. Deficiencies in the pulses lacking in one or two of the limbs were
discovered in six patients after surgery or catheterization. No trophic disturbance was discovered in any of
them, but half of them said, when asked, that they were
often cold in the specific limb.
Fourteen patients are on cardiac medication, mostly
digoxin. All patients with mechanical valves are on
warfarin. Some take diuretics or afterload reducing

medication. Three patients, all boys, are smoking ciga-

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8
6
4

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BjornstadetaL Physical and psychosocial status in adolescents with congenital heart disease

2
0

III

Table 3. Psychiatric diagnoses in the Diagnostic and Statistical


Manual of Mental Disorders9 of adolescents with severe congenital cardiac malformation (n=26).

Boys

Girls

Total

Overanxious disorder
Separation anxiety
Major depression
Dysthymic disorder
Attention deficit disorder
Conduct disorder

4
1
2
1
-

2
2
1
2
_
1

6
3
1
4
1
1

Total number of diagnoses

14

22

Diagnosis in N of patients

12

Psychiatric diagnosis

1
IV

Boys Girls
Figure 2. Physical capacity according to the classification of
the New York Heart Association7 of adolescents with serious
congenital heart defect. Number of patients on the vertical,
functional class on the horizontal axis.

rettes. Twelve patients are now scheduled for further


heart surgeryof a palliative nature in two, but corrective or as a repeated corrective attempt in 10.
Physical functioning
The information as given by the patients to the cardiologist or the cardiac surgeon produced an almost
identical score in the classification of the New York
Heart Association, with a mean of 1.960.77. The
parental (mostly the mother's) judgment gave a mean of
2.31 0.79, and the bicycle stress test a mean of2.46 1.03.
We compared the data using Wilcoxon's test. The
judgment of the two doctors proved identical. There
was no significant difference between the maternal
judgment and the score derived from the stress test, but

a significant overscoring was noted by the response of


the patients themselves. The distribution of physical
capacity according to the stress test is shown in Figure
2. It is noteworthy that there are no girls in class I, and
that the girls outnumber the boys fourfold in class IV.
This difference between boys and girls is statistically
significant.
Psychiatric disorders and psychosocialfunctioning
Twelve adolescents (46%) met the criteria for diagnoses
in the Diagnostic and Statistical Manual (Table 3). The
results from children's global assessment scale (Figure
3) placed one-third (seven boys, one girl) into the best
group. Another third (six boys, three girls) was assessed
as functioning reasonably well with only minor to
moderate problems, but the last third (three boys, six
girls) was seriously dysfunctioning. Significantly, more
girls than boys had impaired psychosocial function.
The mothers scored five of the adolescents (19%,
two boys, three girls) to have psychological problems on
the child behavior checklist. They were among the
patients with the most pronounced psychiatric symptoms.
Socioeconomic status
Classification of the socioeconomic status revealed no
differences from the population in general.16
Chronic family difficulties

100-80

70-60

50-40

Boys Girls I
Figure 3. Psychosocialfiinctioning according to childrens'
global assessment scale10 of 26 adolescents with severe
congenital heart defect. Number of patients on the vertical,
functional score on the horizontal axis.

Half of the families had none or only minor chronic


difficulties (mean 2.82.34). The other half had from
moderate to severe problems. In these cases, the difficulties were mainly caused by either somatic and mental
health problems in the parents or siblings, or conflicts
within the family.
Intercorrelations
The intercorrelations between class of the New York

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Cardiobgy in the Young

Table 4. Correlation coefficients between five different


variables on physical and psychosocial function in 26
adolescents with severe congenital heart disease.

NYHA
CGAS

CFD
CAS

CGAS

CFD

CAS

CBCL

-0.40-

0.06"
-0.66s

0.460.67s

0.24"
0.64s
0.55*
0.60s

0.28"

* p 0.05; t p 0.001; p 0.0001; n not signi ficant

Abbreviations: NYHA: functional class according to the New York


Heart Association; CGAS: Children's Global Assessment Scale; CAS:
Child Assessment Schedule; CFD: Chronic Family Difficulties;
CBCL: Child Behavior Checklist.

Heart Association and the scores on the child assessment schedule, the children's global assessment scale,
chronic family difficulties and child behavior checklist
are listed in Table 4.
Discussion
The most important findings in this study are the high
frequency of major psychiatric problems noted in this
group of adolescents with severe congenital heart disease, and the significant correlation found between the
psychosocial function and physical capacity (Table 4).
The psychiatric problems are atypically for age as far
as boys are concerned, mostly representing anxiety and
depressive disorders. These seem very likely to be caused
by the impact of the life threatening and incapacitating
cardiac malformation itself.
Psychosocial functioning was found to be poor in
nine patients (35%), and reduced in another nine. This
is definitely more than is expected in the general population,17 and demonstrates the need for attention to be
paid also to the mental health in general of this group of
patients. Moreover, the degree of psychosocial and
physical function correlate significantly. This demonstrates that the more reduced the physical capacity of
the patient, the higher the risk for developing psychiatric disorders. This is in keeping with the findings of
Steinhausen et al,18 Wallander et al19 and Engstrem.20
Several previous studies in patients with congenital
heart defects,1"4 nevertheless, found little or no increase
in emotional problems, and no consistent relationship
between organic severity and psychosocial function.
North et al21 may have shown a possible explanation
for such discrepancies. Studying 138 reports on psychiatric factors in ulcerous colitis, they found that 131 of
them contained serious deficiencies in research methodology.
The mothers make accurate judgments of the physi-

January 1995

cal capacity of their children. The old adage of the


pediatrician "listen to the mother" has again been
demonstrated. But even the mothers underestimate the
prevalence of psychopathology. This is in line with the
observation that parents are often less aware of the
internal affective state of their children.23
The distribution of physical capacity shown in Figure
2 is significantly different in boys and girls. A marked
preponderance of females was particularly obvious in
class IV of the New York Heart Association (four of
five). Even when excluding patients with left-sided
artificial valves (all boys, and all well functioning), we
still find an obvious tendency towards poorer physical
capacity in the girls (p=0.06). There is no cardiac
explanation for this difference between boys and girls,
which we consider to be intriguing.
A significant overrepresentation of girls is also found
among the patients with psychiatric problems. Because
of the small number of patients in our study this
preponderance may or may not be accidental. If the
finding is not accidental, it represents an observation
that has been overlooked elsewhere. A possible explanation for the overrepresentation of girls in the less favorable groups physically and mentally may be the significant correlation between physical and psychosocial
function. It seems valid to assume, that (over)protection
and restrictions imposed on patients with severe heart
disease may be a greater risk for girls than for boys. The
female social role may represent a vulnerability factor
for developing psychosocial problems through reduced
physical training, whereas the social environment of
males could represent a protective factor by demanding
participation in physical activity and exercise. This
could overrule some of the restrictions implied by
parents, teachers, doctors, and so on, and have an
impact not only on the physical condition of the
patients. In that case, the difference between the sexes in
both aspects may, at least in part, be caused by a
different degree of physical activity and training, which
should lead us to encourage such physical activity,
especially in girls.
It has been suggested1'3 that the degree of psychopathology and reduced psychosocial functioning may be
due to a direct harmful effect of physical incapacity and
restrictions, or a result of influence of parental anxiety
and overprotection. Our data give additional support
for this view. These reasons may also partly represent
the link to reduced physical capacity. Organic cerebral
dysfunction, either from the cyanotic cardiac lesion
itself or from repeated open heart surgery, cannot be ruled
out, but this question was not addtessed in this study.
Engstf0m20 also found in another group of chronically ill patients that girls generally had higher pathology scores on most of the Child Behavior Checklist
subscales, particularly the subscales for somatic com-

Vol. 5, N o . 1

Bjornstadetah Physical and psychosocial status in adolescents with congenital heart disease

plaints, depression and anxious-obsessive symptoms.


Due to the small size of our samples, nonetheless, we
should be careful with our conclusions. Despite these
caveats, we think that our two first hypotheses have
been confirmed. But it would be worthwhile to make
further studies to assess more clearly if the difference
between the sexes holds true.
We can easily imagine that a severe, life threatening
cardiac lesion has a major influence on mental health.
The association between psychosocial functioning and
environmental long-standing strain (chronic family
difficulties) has been documented, 1422 and further confirmed in this study. The correlation between class in
the New York Heart Association grading and chronic
family difficulties was not significant, indicating that a
major influence from the cardiac malformation itself
was avoided in our assessment (Table 4). On the other
hand, a very highly significant correlation was found
between family difficulties and the score on the global
assessment scale, thus indicating the impact of family
difficulties on psychosocial function, thereby confirming our third hypothesis.
We are pleased to find, nonetheless, that half of our
patients with these severely malformed hearts are functioning in class I or II of the scale of the New York Heart
Association. This certainly is a merit of modern cardiology and cardiac surgery. The patients will probably
have reduced life expectancy, but half of them display a
physical capacity enabling them to lead reasonably good
lives.
The finding that eight of our 26 patients (31 %) are in
completely normal mental health made us look for
protecting factors in these patients. Some were found.
First, a link was observed to good physical capacity, an
argument in favor for early repair, if possible, or palliations without delay. Adequate physical activities and
training should also be encouraged for all patients,
particularly the girls. Second, the adverse effect of
chronic family difficulties on psychosocial development, leads us to emphasize the importance of considering the whole family. This includes not only parents,
but also other family members, friends of parents and
patient, and the school, organizations, clubs, churches
and so on. A good social network seems to be protective.
This knowledge should be used to give psychological,
practical and financial support to patients and families,
and to improve or create a functioning network. We feel
confident that the increasing interest in protective factors24
will lead to future studies focusing on these aspects.
Our study indicates that, whereas the somatic part
has been taken care of satisfactorily, too little attention
has been paid to psychological consequences. Increased
efforts within this, often underestimated, area might
improve not only mental health in this group of patients, but influence the total quality of life. Optimal

61

treatment of patients with congenital heart disease


requires somatic, as well as psychological and familyrelated, care and follow-up.
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