Journal of' Pediatnc Psychology, Vol. 21, No. 3. 1996, pp. 335-352
Behavioral Adjustment of Children with Surgically
Palliated Complex Congenital Heart Disease1
Frank A. Casey
Royal Belfast Hospital for Sick Children
Donald H. Sykes2
Queen's University, Belfast
Brian G. Craig
Royal Belfast Hospital for Sick Children
Rosario Power
Our Lady's Hospital for Sick Children, Dublin
H. Connor Mulholland
Royal Belfast Hospital for Sick Children
Received June 2, 1994; accepted June 21. 1995
Ejcamined the behavioral adjustment at school age of 26 children with surgically
treated complex congenital heart disease compared to that of 26 children who
had been diagnosed as having an innocent murmur. The children with complex
heart disease were rated by their parents as more withdrawn, having more social
problems, and engaging in fewer activities, and by their teachers as more withdrawn. Thefamilies of the children with complex heart disease reported experiencing more stress. Two variables, family strain and exercise tolerance, were strong
predictors of teacher-rated school adjustment in the children with complex heart
'The authors thank Nichola Rooney, Royal Belfast Hospital For Sick Children, for her advice in the
planning of this study. Frank A. Casey held a Research Fellowship supported from trust funds of The
Royal Belfast Hospital For Sick Children.
2
AII correspondence should be addressed to Donald H. Sykes, School of Psychology, Queen's
University, Belfast BT7 INN, Northern Ireland.
335
0146-8693/96/060O-O3351O9.5O/0 O 1996 Pieman PuNiihinj Corporation
336
Casey, Sykes, Craig, Puwer, and Mulholland
disease, with family strain accounting for 33% of the variance, and exercise
tolerance 24%. The impact of the child's chronic condition on the family thus
seems to be a critical factor in the school adjustment of these children, more so
even than the physical limitations imposed on the child by the chronic condition.
KEY WORDS: complex congenital heart disease; behavioral adjustment; exercise tolerance; family
stress.
The impact of chronic physical disease on the psychosocial adjustment of children is of considerable interest, given the continuing improvements in their
medical care and increasing longevity (Drotar, 1981). Reviews of the literature
(Eiser, 1990; Lavigne & Faier-Routman, 1992; Nolan & Pless, 1986; Pless &
Nolan, 1991) have concluded that children with chronic physical disease are at
increased risk for emotional and behavioral maladjustment, with Pless and Nolan
estimating their risk for secondary emotional disturbance to be twice that of their
healthy peers. It has been argued that the risk is general and is not related to
specific diagnostic entities (Pless & Nolan, 1991). However, Lavigne and FaierRoutman (1992), in their meta-analysis of published studies, found considerable
variation between diseases on a measure of overall adjustment. In studies incorporating controls and normative comparisons, they found a group of disorders
with a very large effect size of .75 or more, these included (in decreasing order of
effect size) seizure disorders, inflammatory bowel disease, deafness, cardiac
disorders, and burns. A second group, with a large effect size between .50 and
.74 included cerebral palsy, neurologic (nonseizure) disorders, diabetes, dwarfism, cleft-lip/palate, blindness, pooled disorders, myelomeningocele, and cystic
fibrosis. Disorders with moderate effect sizes between .25 and .49 included
cancer, asthma, juvenile rheumatoid arthritis, and orthopedic disorders. Small
effect sizes, below .24, were noted for renal disorders and precocious puberty.
However, the authors added the cautionary note that the number of studies within
each disorder on which effect sizes could be calculated were small, limiting the
reliability of the effect size comparisons.
In the category cardiac disorders, Lavigne and Faier-Routman (1992) identified only six studies during the period 1928-1989 that met their modest methodological requirements for inclusion in their meta-analysis. Furthermore, in
many of the published studies on congenital heart disease (CHD) the cases
included can be quite heterogeneous in terms of the severity of the CHD, including children with minor lesions having little impact on daily life together with
children with more major defects requiring surgery. In three more recently published articles, two (DeMaso, Beardslee, Silbert, & Fyler, 1990; Utens et al.,
1993) found evidence of greater behavioral impairment in the children with CHD
and one did not (Kramer, Awiszus, Sterzel, van Halteren, & Clausen, 1989).
If there are relatively few studies of psychosocial adjustment in children
Adjustment in Complex Congenital Heart Disease
337
with CHD, there is a particular dearth of studies reporting on the psychological
adjustment of children with complex cyanotic CHD not amenable to repair. This
group of children, bom with the most severe forms of CHD, usually have hearts
with only a single functional ventricle. There are no surgical procedures that
repair the defect giving them two functional ventricles and a normal circulation
of blood. They therefore differ from patients with transposition of the great
arteries or tetralogy of Fallot who, although also cyanotic in early infancy, can
now be surgically repaired giving a normal circulation and eliminating cyanosis.
Most children with forms of univentricular heart present with cyanosis in the
newborn period. The combined live-birth prevalence of the two most common
forms of univentricular heart, tricuspid atresia and pulmonary atresia, was estimated in the Baltimore-Washington infant study at 1.18 per 10,000 live births
(Ferencz et al., 1985). In previous generations they were considered inoperable,
and most died in early infancy (Keith, Rowe, & Vlad, 1977; Report of the New
England Regional Cardiac Program, 1980). In recent years there has been an
increasing tendency to perform palliative surgery in an attempt to prolong life,
and the management of children with very complex heart disease now constitutes
a significant part of the workload of major pediatric cardiology centers. In most
cases the initial surgery is shunt surgery. This consists of connecting a small
Gortex tube between a systemic artery (usually either the left or right subclavian
artery) and one of the pulmonary arteries, increasing the amount of pulmonary
blood flow, and reducing the degree of, but not eliminating, the cyanosis. Mortality figures for this type of surgery in the neonate are variable, but it should be
less than 20% (Adams, Emannouillides, & Riemenschneider, 1989; Trusler &
Williams, 1980). Published work confirms increased duration of survival after
shunt surgery (Dick, Fyler, & Nadas, 1975). Trusler and Williams (1980) reported a 70% survival rate at 15 years after shunt surgery in patients with tricuspid
atresia, who have functionally a single ventricle. For those surviving through
infancy the goal is to perform further surgery later in childhood of the type first
described by Fontan and Baudet (1971). Although this operation has the potential
to eliminate cyanosis it still leaves the patient with a very abnormal circulation
without a right-sided pumping chamber. The operative mortality for the Fontan
procedure is in the range 7 to 17% (Fontan et al., 1983; Freedom, Benson, &
Smallhorn, 1992; Laks et al., 1984). Medium-term survival for some of the
earliest patients to have this procedure has been reported to be as high as 85%
(Girod, Fontan, DeVille, Ottenkamp, & Choussat, 1987).
Thus, there are now surgical options to achieve survival in these children
with the most complex forms of CHD. However, little has been written about the
quality of life of such survivors, and this is increasingly becoming the focus of
attention for those involved in their care. One study that did include a subsample
of children with univentricular hearts did not report on their psychological outcome separately from that of the other children constituting a cyanotic group with
338
Casty, Sykes, Craig, Power, and MnlboUand
limited physical capacity (Kramer et al., 1989). Contrary to expectations, the
children with cyanotic heart disease were not found to be different in regard to
their behavior (as rated by parents) to a control group of healthy children with a
diagnosis of functional heart murmur.
The aim of the present study was to evaluate the results of palliation in a
group of children with univentricular hearts in terms of both physical outcome
and psychological adjustment to their chronic condition. We recently reported on
the physical outcome for this group (Casey, Craig, & Mulholland, 1994). This
paper focuses on their psychosocial adjustment, and its relationship with measured exercise capacity, this latter being an objective measure of physical outcome. The three areas of functioning evaluated are the behavioral, the academic,
and the social. Multivariate research over the last several decades has provided
convincing evidence that behavioral disturbance in childhood can be categorized
along two broad dimensions, namely, internalizing and externalizing expressions
of dysfunction (Achenbach, 1985; Achenbach & Edelbrock, 1978), with these
two dimensions, particularly the externalizing, having implications for longer
term adjustment (Cicchetti & Toth, 1991; Robins, 1979). Problems in behavioral
adjustment are also linked to academic underachievement, with both domains
(behavioral and academic) predicting later maladjustment (Hinshaw, 1992). The
sociometric status of the child is also of importance, since the withdrawn child
may evidence deficits in peer relations (Rubin, 1985), which peer deficits impact
on later personal adjustment (Parker & Asher, 1987). Little that is definitive is
known concerning the social adjustment of the chronically ill child (Spirito,
DeLawyer, & Stark, 1991), but there is a suggestion that the peer relationships of
children with restrictions placed on their activities may be affected (Spirito et al.,
1991).
The child's adjustment to a chronic illness is mediated, however, not only
by characteristics of the child but also by the reactions of the family to the illness
(Shapiro, 1983). Chronic illness in a child is a source of stress in the family
(Hauenstein, 1990), affecting family functioning (Sholevar & Perkel, 1990). It
has been suggested that normal family patterns and child-rearing practices can
become distorted in families with a chronically ill child. The parents themselves
can be affected (greater fatigue, feelings of malaise, etc.). The child may become
the focus of attention, to the detriment of other family members, with an overinvolved relationship developing between mother and child. Financial hardships
may be experienced, and the family may become more socially isolated (Shapiro, 1983). Aspects of family functioning and the manner in which the family and
family members react to the stress of the chronic illness, can, in rum, affect,
positively or negatively, the adjustment of the child (Hamlett, Pellegrini, & Katz,
1992; Lavigne & Faier-Routman, 1993; Perrin, Ayoub, & Willett, 1993).
Given the evidence that chronic illness in a child can impact negatively on
the child's psychosocial adjustment, with further negative effects on family functioning, the study was designed to evaluate the behavioral, academic, and social
Adjustment in Complex Congenital Heart Disease
339
adjustment achieved by children with complex CHD who had received palliative
surgery, together with the impact of this chronic condition on family functioning.
Little is known concerning the adjustment problems faced by these children,
many of whom continue to experience breathlessness on exertion, with treadmill
exercise testing indicating that the majority cope with moderate functional disability (Casey et al., 1994). The impact of the condition on family functioning
was also studied, in light of the evidence that familial coping reactions to chronic
disease in a child appears to reflect not a generalized response to the increased
stress but rather the particular difficulties faced by families of children with
specific chronic conditions (Eiser & Havermans, 1992). The impact of the condition on family functioning is of interest not only in its own right, however, but
also, as stated above, as a potentially important mediating variable in the child's
adjustment.
More specifically four hypotheses were advanced. First, it was hypothesized that, compared to their nonchronically ill peers, the children with complex
CHD would be at heightened risk for attentional problems and academic underachievement. Complex CHD children are still cyanotic after the palliative surgery and subject to breathlessness, which may have negative consequences for
the effective deployment of their attention. Also, O'Dougherty, Berntson, Boysen, Wright, and Teske (1988) found that children with CHD were prone to
attentional dysfunction (as reflected in poorer performance on a sustained attention task). Problems with academic underachievement in these children are also
to be expected given that inattention is known to be strongly correlated with
academic problems in childhood (Hinshaw, 1992).
Second, it was hypothesized that, because children with restrictions placed
on their activities may have fewer opportunities for social interactions with their
peers (Harper, 1991; Spiritoet al., 1991), complex CHD children would be more
socially withdrawn. The third hypothesis was that families of the children with
complex CHD would report experiencing more stress than the control families,
reflecting not only the chronic nature of the condition (Hauenstein, 1990; Sholevar & Perkel, 1990) but also the fact that the surgery to date was palliative, with
further surgery required in the future. Finally, it was hypothesized that the stress
reaction of the family to the child's condition might itself be a mediating factor
influencing the overall adjustment of the child (Hamlett et al., 1992; Lavigne &
Faier-Routman, 1993; Perrin et al., 1993).
METHOD
Subjects
The study was carried out in the pediatric cardiology department of the
Royal Belfast Hospital for Sick Children, which is the regional referral center
340
Cast* Sykes, Craig, Power, and MulhoUand
managing all children in Northern Ireland with complex CHD. The four criteria
for inclusion in the study were as follow: (a) Of school age; (b) complex cyanotic
CHD not amenable to total repair (i.e., children with univentricular hearts or
variations thereof); (c) absence of any chromosomal or major noncardiovascular
abnormality (Thus children who had required surgery for any other condition
were excluded.); and (4) surgical palliation at least 2 years previously and no
recent hospital admission. From the computerized database of the cardiology
department, 27 eligible patients were identified. Written consent was obtained
for 26 patients (21 male, 5 female). The reason given by the parents in the one
case where consent was withheld was that they felt that the proposed detailed
examination of their child would focus his attention on the illness. The diagnostic
categories of the study group were (a) absent right atrioventricular connection, n
= 8 (31%), (b) absent left atrioventricular connection, n = 9 (35%), (c) double
inlet, left ventricle, n = 5 (19%), and (d) complex double outlet, right ventricle,
n = 4(15%).
The demographic characteristics of the children are presented in Table 1.
The control group comprised 26 children who had attended the pediatric cardiology clinic on one occasion and had been diagnosed as having innocent murmurs.
Such children have been chosen by some researchers as appropriate control
comparisons when studying children with CHD (e.g., Kramer et al., 1989;
Wright & Nolan, 1994). None of the control children had been subject to surgical
interventions. The two groups were matched for age, /(50) = 0.22, ns, and
gender (exactly). Social status as measured by occupation (Registrar General,
1980) showed a statistically nonsignificant trend, x ^ 3 , N = 52) = 4.31, ns, for
the parents of the children with complex CHD to be of lower socioeconomic
status. All of the control children were in full-time attendance at school. Of the
Table I. Demographic Information: Complex CHD and Controls
Measure
Complex CHD
Controls
Sample n
Gender % male
Age (years ± SD)°
SES*
Professional/intermediate*1
Skilled
Semi/unskilled
Housewife
School attendance1'
Full-time
Three-quarters
Half-lime
Home tutored
26
81
8.78 ± 3.26
26
81
8.62 ± 1.97
5(19)
11 (42)
7(27)
3(12)
10 (38)
12 (46)
3(12)
1 (4)
17 (65)
4(15)
3(12)
2(8)
26 (100)
0
0
0
"Range: 4.4-16.5 years Complex; 5.2-15.8 years Controls
'Percentages in parenthesis.
Adjustment in Complex Congenital Heart Disease
341
children with complex CHD, 17 (65%) were in full-time attendance, 4(15%) were
attending 3/« time, 3 (12%) were attending 'h time, and 2 (8%) were receiving
home tutoring. In the case of the 2 children receiving home tutoring, 1 was the
most severely ill child in the group who could not have attended school at that time,
whereas the other was not attending school due to maternal anxiety, physical
assessment indicating that he was well enough to go to school. Less than full-time
attendance at school was not related to diagnosis or to functional status. Typically,
these children attended school each day, but finished earlier than their peers,
parents having been advised by their physician to encourage the child to remain at
school as long as the child could do so comfortably. None of the children from
either the control or complex group had repeated a year at school. The children
were considered to be of normal cognitive ability, since with the exception of 1
child who was attending a school for the physically handicapped, and the 2
children not attending school, the remainder were in attendance at regular schools.
Only 1 (complex) child was noted to have neurological abnormalities.
Measures
Behavioral, Academic, and Social Adjustment. Behavioral adjustment was
assessed with the parent- and teacher-rated forms of the Child Behavior Checklist
(Achenbach, 1991a, 1991b). These empirically derived and reliable checklists
provide, within each form, narrow-band measures of adjustment (Thought Problems, Attention Problems, etc.), two broad-band measures of behavioral disturbance (Internalizing and Externalizing), and a total behavior problem score. In
both forms of the checklist, there are two narrow-band measures of social adjustment, namely, Withdrawn and Social Problems. It would have been preferable to
have sociometric status measured by peer assessment. However, this was not
possible, and the Withdrawn and Social Problems factors of the Child Behavior
Checklist are used instead as indicators, to some degree, of the child's social
adjustment. In the teacher-rated form there are additionally five measures of
school adjustment (academic performance, working, behaving, learning, and
happy), and in the parent-rated form, three measures of social competence,
namely, activities, social, and school. Multivariate analysis of variance (MANOVA) from the Statistical Package for the Social Sciences (SPSS, 1990) was
used to analyze the relative performance of the two groups of children (complex
and control) in respect of their behavioral, social and academic standing, since
the multiple dependent measures are correlated.
Family Functioning. The impact of the child's medical condition on the
family was assessed with the Impact on Family Scale (Stein & Riessman, 1980).
The scale focuses on changes in the normative behavior of the family as a
consequence of the child's illness, with negative influences on family relationships
"conceptualized in terms of losses: financial burden, restrictions in social life,
decreased interaction with significant others, less time for other family members.
342
Casey, Sykes, Craig, Power, and Mulholland
and increased subjective distress and strain" (Stein & Riessman, 1980, p. 466).
The scale provides four measures, namely, financial strain, familial/social strain,
personal strain, and mastery (the latter, mastery, not scored since many of its items
were not applicable to the families of the control children). Such a measure was
included given the evidence that a child's adjustment may be mediated to a degree
by family reactions to the illness (DeMaso et al., 1991).
Physical Functioning. The functional physical ability of each child was
assessed with an objective measure of exercise tolerance, using a symptomlimited graded treadmill exercise test (Bruce, Kusimi, & Hosmer, 1973), providing a maximum exercise time (minutes) for each child. The relationship between
family strain, exercise time, and the dependent measures was assessed using
multiple regression.
Procedure
Having identified the 26 patients with complex CHD from the computerized
database, a list of patients with innocent murmurs who were scheduled to attend
upcoming outpatient clinics was obtained from the same database. Patients with
complex CHD were then matched with the first patient on the outpatients list who
was (a) the same gender, and (b) an age within ± 6 months of that of the complex
patient. Following identification from the computerized database of eligible subjects, the parents were contacted by letter, and the informed consent of both
parent and child was sought, with the nature and purpose of the study fully
explained in the initial contact letter. An appointment was made for each child to
be seen in the pediatric cardiology department. While the physical assessment
was being carried out, the parent accompanying the child (invariably the mother)
was asked to complete the Child Behavior Checklist and the Impact on Family
Scale. Parents had been told that contact with the child's school would be
necessary, and details of the school and teachers names were elicited during the
clinic session. The teacher-rated form of the Child Behavior Checklist and an
accompanying letter were posted to the principal of the child's school, requesting
that the child's teacher complete the checklist, and return it in the stamped,
addressed envelope. Where there were delays in returning the questionnaire,
reminder telephone calls were made.
RESULTS
Teacher-Rated Adjustment
Although there was a trend for the children with complex CHD to be rated
by their teachers as performing less well academically, F(l, 43) = 2.392, p =
Adjustment in Complex Congenital Heart Disease
343
.13, and working less hard, F(l, 43) = 2.535, p = .12, than their controls, the
multivariate F on the five measures of school adjustment was not significant,
F(5, 39) = 1.435, ns, indicating a similar level of school adjustment in the two
groups of children. However, this positive result needs to be qualified slightly by
the finding of a higher incidence of children with complex CHD performing
academically in the clinical or borderline clinical range (T scores less than 41)
compared to their controls (i.e., 11 vs. 5, x2 = 3.45, p = .063). MANOVA on
the eight behavioral factors produced a significant multivariate F. F(8, 38) =
3.552, p = .004, with univariate Fs (Table II) identifying two of the eight factors
as discriminating between the two groups, namely, Withdrawn and Somatic
Complaints. These results remained when less than full-time attenders at school
were excluded. However, it is known that behavior checklists such as the CBCL
can pose problems for interpretation when used with children with chronic physical illnesses (Perrin, Stein, & Drotar, 1991). One of the problems applies to the
two factors, Withdrawn and Somatic Complaints. The item "underactive" is one
of the items defining the Withdrawn factor, and three items, "dizzy," "tired," and
"headaches" load on Somatic Complaints, four items on which many more of the
children with complex CHD are rated positively than the control children. Given
that these positive ratings possibly reflect the childrens' medical condition, it has
the effect of artificially raising their scores on these factors. When the factors are
rescored, leaving out the four items, the multivariate F (and the univariate Fs
also) ceases to be significant, F(8, 38) = 1.050, ns.
With the two broad-band measures of behavioral adjustment, Internalizing
and Externalizing, there was a significant multivariate F, F(2, 44) = 3.330, p =
.045, with the complex CHD children being rated higher on the Internalizing
dimension, F{\, 45) = 4.921, p = .03. However, if the Internalizing score is
calculated without the four items specified above, the multivariate F ceases to be
Table II. Teacher-Rated Behavioral Adjustment. Comparison of Children with Complex CHD
with Their Controls
Complex CHD
Behavioral factor
Withdrawn
Somatic complaints
Anxious/depressed
Social problems
Thought problems
Attention problems
Delinquent behavior
Aggressive behavior
Control
M
SD
M
SD
F
P
56.67
61.13
53.63
55.67
51.67
55 75
53.00
53.25
5.49
9.21
5.95
6.35
4.72
6.63
5.15
5.90
52.30
52.96
53.30
53.96
50.00
53.61
54.13
53.91
4.06
5.41
5.12
5.59
0.00
5.29
5.47
5.27
9.522
13.576
0.039
0.958
2.871
1.490
0.532
0.165
.003
001
ns
ns
ns
ns
ns
ns
344
Casey, Sykes, Craig, Power, and Mulholland
significant, F(2, 44) = 0.709, ns. The two groups of children did not differ in
regard to the single overall scale score, f(45) = 1.38, ns.
Parent-Rated Adjustment
MANOVA on the three social competence scales, Activities, Social, and
School, produced a significant multivariate F, F(3, 43) = 4.199, p = .011, the
children with complex CHD being rated as less engaged in activities compared to
the control children, univariate F(l, 45) = 9.762, p = .003. MANOVA on the
eight behavioral factors produced a significant multivariate F, F(8, 42) = 3.318,
p = .005, with three of the factors identified as significant at the univariate level:
Withdrawn, Somatic Complaints, and Social Problems (Table 111). The Withdrawn and Social Problems factors remained significant when children not in
full-time attendance at school were excluded from the analysis, F(l, 40) =
6.568, p = .014, and F(l, 40) = 4.815, p = .03, respectively, but Somatic
Complaints did not, F(l, 40) = 2.819, ns. When the Withdrawn and Somatic
Complaints factors are rescored without the four items, the multivariate F is
reduced but remains significant, F(8, 42) = 2.376, p = .033, with Somatic
Complaints no longer significant, F(l, 49) = 1.891, ns, and Withdrawn reduced
to the statistical borderline, F(l, 49) = 3.365, p = .073.
With the two broad-band measures of behavioral adjustment, Internalizing
and Externalizing, there was a barely significant multivariate F, F(2, 48) =
3.077, p = .055, with the complex CHD children being rated higher on the
Internalizing dimension, F(l, 49) = 6.188,/;= .016. The multivariate F ceased
to be significant when the Internalizing factor was rescored without the four
items, F(2, 48) = 1.680, ns. The children with complex CHD scored higher than
their controls on the single overall scale score, t{49) = 2.48, p = .017: M
Table III. Parent-Rated Behavioral Adjuslment. Comparison of Children with Complex CHD
with Their Controls
Complex CHD
Behavioral factor
Withdrawn
Somatic complaints
Anxious/depression
Social problems
Thought problems
Attention problems
Delinquent behavior
Aggressive behavior
Control
M
SD
M
SD
F
P
58.19
66.73
56.65
60.69
51.65
59.15
56.00
55.77
8.31
11.56
9.05
9.26
3.09
7.37
7.49
7.93
53.52
57.44
55.24
54.84
50.56
58.36
52.96
52.92
6.04
7.51
7.29
9.42
1.94
11.07
5.62
6.04
5.235
11.486
0.375
5.010
2.278
0.092
2.673
2.071
.026
.001
ns
.03
ns
ns
ns
ns
Adjustment in Complex Congenital Heart Disease
345
complex = 57.85, SD = 10.36; M controls = 50.28, SD = 11.39, which effect
was reduced but remained significant when the four items were excluded, f(49)
= 2.01, p = .05.
Exercise Tolerance
There was, as expected, a considerable difference in exercise time (measured in minutes) between the two groups, F(\, 49) = 94.26, p < .001: M
complex = 7.12, SD = 1.86; M controls = 12.04, SD = 1.76.
Impact on the Family
MANOVA on the three subscales produced a highly significant multivariate
F, F(i, 48) = I7.191,p < .001, with univariate tests indicating that the families
of the target children experienced more financial strain, greater familial/social
stress, and greater personal strain (Table IV).
Behavioral Adjustment, Family Strain, and Exercise Tolerance
Exercise tolerance is an objective indicator, to a degree, of the functional
physical ability of the child. Impact on the family is a subjective indicator of the
stress experienced by the family as a consequence of the child's cardiac condition. These two measures clearly differentiate the children with complex CHD
from the control children. An important question is whether these measures
affect the behavioral adjustment of the children. One would expect family stress
and parent-rated behavioral adjustment to be related to some degree, since both
are rated by the one informant, the mother. However, the three measures, family
stress, exercise tolerance, and teacher-rated behavioral adjustment, are independent of each other in terms of their source. Within the group of children with
complex CHD, family strain (the mean of three of the Impact on Family measures, i.e., financial, familial/social, and personal strain) and exercise tolerance
Table IV. Impact on Family Scale: Comparison of Children with Complex CHD
with Their Controls
Complex CHD
Subscale
Financial strain
Familial/social stress
Personal strain
Control
M
SD
M
SD
F
P
58.19
57.85
64.08
14.23
13.95
14.11
36.00
36.23
36.92
13.84
12 98
13.54
32.51
33.45
50.11
<.00l
<00l
<.00l
346
Casey, Sykes, Craig, Power, and Mulholland
Table V. School and Behavioral Adjustment of Children with Complex CHD:
Correlations with Family Strain and Exercise Tolerance
Family strain
Exercise tolerance
School adjustment (n = 23)
Mean school performance
Working
Behaving
Learning
Happy
-.524"
-.569*
-.466°
-.620*
-.315
.437°
.435°
.482°
.476°
.522°
Behavioral adjustment (n = 24)
Withdrawn (teacher-rated)
Somatic complaints (teacher-rated)
Withdrawn (parent-rated)
Somatic complaints (parent-rated)
Social problems (parent-rated)
.268
.178
-.202
.288
.162
.153
044
.108
.206
.329
"p :s .05.
b
p =s .01.
were found to be related to teacher-rated measures of school functioning but not
to the two teacher-rated behavioral factors that distinguished the targets from
their controls (i.e., Withdrawn and Somatic Complaints) nor to the three parentrated behavioral factors that distinguished between the groups (i.e., Withdrawn,
Somatic Complaints, and Social Problems) (Table V). There was no relationship
between family strain, exercise tolerance, and the behavioral measures in the
control group. Taking the children with complex CHD together, entering family
strain and exercise tolerance (which are not themselves correlated: r = —. 12) as
two predictors in a multiple regression equation with a summary measure of
school adjustment (Total Adaptive T score) as the dependent variable, produced a
significant multiple correlation, R = .753, F(2, 20) = 13.056, p = .0002,
accounting for 57% of the variance in school adjustment. Family Strain determined 33% and exercise tolerance 24% of the variance, respectively (Table VI).
Table VI. Summary of Stepwise Regression Analysis for Variables Predicting
the School Adjustment of Children with Complex CHD
Variables entered
Step 1
Family strain
Step 2
Family strain and exercise tolerance
R2
df
F
P
0.326
1
10.166
.004
0.566
1
11.071
.003
2, 20
13.056
.0002
Adjustment in Complex Congenital Heart Disease
347
DISCUSSION
The findings from this study provide some support for certain of the hypotheses put forward in the introduction but not for others. Children with complex
CHD who have had palliative surgery are not rated by their teachers or their
parents as more inattentive when compared to their peer controls, a finding that is
not compatible with the suggestion of O'Dougherty et al. (1988) that children
with CHD are prone to attentional problems. Academically, the children with
complex CHD are only marginally impaired compared to their controls, although
more of them score in the clinical or borderline clinical range, thus providing
only modest support for the finding of Wright and Nolan (1994) of poorer school
performance in children with cyanotic heart disease. They are seen by parents
and teachers as more withdrawn, with parents also viewing their children as
having more social problems and engaging in fewer activities. However, this
perception of the children with complex CHD being more withdrawn seems to
reflect unduly the single item, underactive. When it is excluded, the teacherrated effect ceases to be significant, and the parent-rated effect is reduced to the
statistical borderline. The only other CBCL factor that differentiated them from
their control peers was Somatic Complaints. But this factor was reduced to
nonsignificance when the three items, dizzy, tired, and headaches, were excluded. There was support for the third and fourth hypotheses. The families of
the children with complex CHD reported experiencing more stress as a consequence of their child's condition, scoring considerably higher than the families of
the control children in regard to financial, familial/social, and personal strain.
The familial stress response seems to mediate at least some of the child's adjustment, in that the measure of family strain predicted some 33% of the variance in
the school adjustment of the children with complex CHD.
The finding of a higher incidence of children with complex CHD performing academically in the clinical or borderline clinical range may reflect central
nervous system impairment and/or a deficit in intelligence, both having been
noted in children with transposition of the great arteries, or with tetralogy of
Fallot, who had been surgically treated (DeMaso et al., 1990), with lower intelligence being found in cyanotic as opposed to acyanotic CHD children (Aram,
Ekelman, Ben-Shachar, & Levinsohn, 1985). Not having a measure of intelligence in the present study (there were not the trained personnel with the time to
carry out the assessments) this possibility cannot be tested. The failure to measure intelligence, however, poses a problem for the interpretation of the regression analysis. In that analysis, a substantial 57% of the variance in a global
measure of school adjustment was determined by family strain and exercise
tolerance. If either or both of these variables are correlated with intelligence, and
if intelligence is itself correlated with the dependent variable, school adjustment,
then there is a specification error in the regression analysis (Lewis-Beck, 1990),
348
Casey, Sykes, Craig, Power, and Mulbollajtd
which precludes definitive statements being made regarding the role of family
strain and exercise tolerance in determining school adjustment. It is possible that
intelligence and exercise tolerance are correlated, given that lower levels of
exercise tolerance may reflect more severe cyanosis. Nonetheless, it is also
possible that exercise tolerance, a measure of the heart's efficiency in providing
the required oxygen for performance, might affect school adjustment directly.
Similarly, not only might the observed link between exercise tolerance and
school adjustment be due to the mediating variable of intelligence, but so also
might intelligence be the reason for the high correlation between family strain
and school adjustment. For example, reduced child intelligence could both place
a strain on the family and account for poor school adjustment. However, this
explanation seems less likely in the present study, given that parents' ratings of
the school competence of complex CHD children were not significantly different
from those given by the parents of the control children. The question then arises
as to why a measure of family strain should have such a large impact on the
child's school adjustment (33% of the variance). Further analyses revealed that
the relationship between the measure of family strain and the child's adjustment
at school was mainly a function of the subscale, familial/social strain, which
measures the impact of the sick child on the family (it includes such items as, "I
don't have much time left over for other family members after caring for my
child," "Because of the illness we are not able to travel very far from home,"
"Our family gives up things because of my child's illness," etc.). In families
scoring high on this subscale, the child's physical condition seems to be having a
major negative influence on normal family functioning. That being so, it implies
that the sick child is a focus and recipient of a disproportionate amount of family
(parental) attention. The net effect could be that the usual parental disciplinary,
child management, practices would not apply in the same degree to the sick
child, with the child being pampered by the parents, as Linde (1982) has suggested. A lower level of parental discipline would produce a child with fewer
self-regulatory skills and social competencies, with the child adopting to some
degree an invalid role, which would impact negatively on school adjustment.
Despite the group differences that were identified, it can be argued that,
overall, the behavioral adjustment and physical status of this sample of children
with complex CHD are encouraging. The children were for the greater part rated
as behaviorally no different to their peers. When they were rated more negatively
than their peers (more withdrawn, more social problems) their scores were far
from placing them within the range of children referred to child guidance facilities. Academically, they were not doing quite as well as their peers, but again the
difference was not large. Of particular interest is the finding that a significant
degree of the variation in school adjustment may be mediated by parental reactions to the child's condition rather than reflecting factors intrinsic to the medical
condition itself. Physically (Casey et al., 1994), the majority of the children in
the present study were functioning at a moderate, yet clinically significant, level
Adjustment in Complex Congenital Heart Disease
349
of physical disability (the predominant somatic symptom being breathlessness).
The mean maximum exercise time for the complex group was 7.12 minutes
(compared to 12.04 minutes for the controls), which is the equivalent of a
walking distance of 343 meters. This is a substantially greater distance than that
estimated by the parents, 70% of whom reported a walking distance of less than
100 meters as within the capability of their child (in all, 80% of the parents of the
children with complex CHD underestimated their child's exercise tolerance).
This misperception of the child's physical capabilities may be an additional
factor influencing the adjustment of the child, for parental perceptions of the
severity of the illness rather than actual severity (Offord, Cross, Andrews, &
Aponte, 1982), and parental perceptions of the restrictions of the disease (Eiser,
Havermans, Pancer, & Eiser, 1992), have been found to be associated with
poorer adjustment. Although direct comparisons of the magnitude of the effect of
this chronic condition on children versus parents cannot be made, nonetheless the
data suggest that the stress may be greater for parents than for the children
themselves, at least within the age range studied. Families of children with
complex CHD report considerable strain, yet the children themselves are rated,
by teachers and parents, for the most part as no different than their peers, and
when they are rated as different, the difference is far from a magnitude that would
be clinically significant.
As with other chronic conditions (Brown, Doepke, & Kaslow, 1993), families of children with congenital heart disease would probably benefit from a
comprehensive evaluation and intervention program, designed to assess aspects
of family structure known to impact on parenting (Hamlett et al., 1992; Kxonenberger & Thompson, 1992; Speechley & Noh, 1992), to educate parents as to the
actual capabilities of their child, and to offer guidance as to appropriate parenting
practices that would reduce the likelihood of the child adopting an invalid role.
Although there have been few rigorously evaluated behavioral family interventions with chronically ill children, the evidence nonetheless suggests that they
show promise for helping the child to adjust and the family to adapt to the
chronic condition (Finney & Bonner, 1992). Such family interventions, targeting
specific parameters of family functioning thought to impact on the child's adjustment, and if they also incorporated measures of physical functioning (exercise
tolerance) and cognitive ability (IQ), would help to resolve issues relating to the
directionality of effect, that is, whether the family response affects the child's
school adjustment or vice versa.
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