Cardiol Young 1995; 5:56-62 World Publishers Limited

ISSN 1047-9511

The impact of severe congenital heart disease on physical andpsychosocial functioning in adolescents

Per G. Bjornstad, Ingrid Spurkland and Harald L. LindbergFrom the Departments ofPediatric Cardiology, Child and Adolescent Psychiatry and Thoracic and CardiovascularSurgery, Rikshospitalet, The National Hospital, Oslo

Abstract A study was made of 26 adolescents, aged from 13 to 18 years, with different types of severe congenitalheart 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 psychiatricproblems. Associations were found between physical capacity and psychosocial functioning, and between psychosocialfunctioning and chronic family difficulties. On the other hand, half the patients studied achieved fair function, bothphysically and with regard to their mental health. The degree of reduced physical capacity is an important etiologicalfactor for impaired mental health in patients with congenital heart disease. This knowledge should be taken intoaccount when dealing with this group of patients. A good social network seems to be a protective factor. It willprobably be rewarding to give patients and their families not only an optimal medical follow-up, but alsopsychological, practical and financial support to improve or create such a network.

Key words: Congenital heart disease; physical disability; mental health; psychosocial functioning; adolescents

THE LAST TWO DECADES HAVE WITNESSED MARKEDadvances in all areas of cardiological medicine.Improvements in diagnostic methods, anes-thetic and surgical techniques, as well as postoperativeintensive care, have created a new group of patients:adolescents and young adults with severe congenitalheart disease. Only a few years ago most of these patientswould not have survived beyond infancy or childhood.Now they will require life-long follow-up.

Since the study of Landtman et al1 on psychosocialfunctioning in patients with congenital heart disease,several researchers have investigated this topic. Strangelyenough, a number of studies1'4 find little or no increasein emotional problems. When they do, a consistentrelationship between organic severity and psychosocialfunctioning is lacking. More recent studies,56 however,describe an association between the degree of physicalimpairment and psychosocial functioning. The psy-

The project was financed by The National Hospital, The Child Psychiatric Clinicat 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 diseasedadolescents, nonetheless, has hardly been assessed. Wewanted to address this question from the stance of threehypothesesfirst, that severe congenital heart diseasehas an impact on psychosocial functioning in adoles-cents; second, that the psychosocial functioning isassociated with the degree of physical impairment; andthird, that adolescents with severe congenital heartdisease are more vulnerable to chronic family difficul-ties than those from the general population.

Material

According to the hypotheses, the degree of physicalimpairment, and the possibility of its treatment, areimportant factors that may influence psychosocicaldevelopment, rather than the specific anatomic lesion.All patients aged from 13 to 18 in the register of theNational Hospital with our interpretation of the mostserious form of congenital heart disease were selected. Atotal of 33 patients fulfilled our criteria which were:severe cyanotic heart defect without surgical possibili-ties, with die potential or presence of palliation or withrepair or potential for repair including valvar replacement.

Vol. 5, N o . 1 Bjornstad et al Physical and psychosodal status in adolescents widi congenital heart disease 57

Table 1. Somatic diagnosis and latest type of operation in adolescents with severe congenitalcardiac malformation (n=26).

Diagnosis

Fallot's anomaly with pulmonary atresia or severestenosis requiring a valved conduit

Univentricular atrioventricular connection with orwithout tricuspid atresia

Pulmonary atresia or severe stenosis with DORVor complex transpositions

Ebstein's malformationSevere left-sided valvar disease with valvar replacement

AorticMitral

Total

None

1

-

1

_

-

2

Type of operationsRepair

9

2

2

-

41

18

Palliation

1

4

1

-

_

-

6

Total

10

7

3

1

41

26

DORV: double outlet right ventricle.

The patients included were all considered to havereduced life expectancies. Events proved this suspicionto be well founded. Three patients died within a year ofthe completion of the study. Three patients refused toparticipate. Two patients were excluded because ofadditional physical problems (blindness, chromosomalabnormality) that might have influenced the results.One patient had emigrated. Another one agreed toparticipate, but died from his heart defect during theplanning period of the project. The mean age of theremaining 26 patients (16 boys and 10 girls), was 16.3years. The age and sex distribution is displayed in Figure1. Twenty-one patients had severe cyanotic diseasewhile five had mechanical left-sided valves. Somaticdiagnoses are grouped, and latest types of operationsummarized, in Table 1. The time for surgical interven-

16 17 18;dBoys GirlsJ

Figure 1. Distribution of age and gender of the 26 adolescentsin the study. All of them had different, severe congenital heartdefects. Number of patients on the vertical, age in years on thehorizontal axis.

tion differed widely, but none underwent surgery withi nthe last year before the study. The data on growth andhistory are summarized in Table 2. The hospital dataapply only for The National Hospital, and do notinclude admittance to local hospitals.

Methods

Somatic assessmentAll patients underwent separate clinical examinationsfollowing a standard protocol by a pediatric cardiologistand a cardiac surgeon. The history included a survey ofsocial factors, the judgment of their scars, and theimpressions of the family concerning the medical andsocial support they had been given. General clinicalexamination and routine electrocardiography, chest x-ray and echocardiograms were carried out. A standard-ized bicycle stress test was included. Hematologicalparameters 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 applyonly for The National Hospital and do not include stays inlocal hospitals. Operations exclude non-cardiac surgery.

Parameter

Age (years)Percentiles (height)Percentiles (weight)Days in our hospitalAdmissions hereCatheterizationsOperations

Mean

16.3314780.07.54.72.3

SD

1.7243646.52.71.81.3

Range

13- 1810

58 Cardiology in the Young January 1995

catheterization was performed only when indicated fortreatment or counseling. This was done in 15 patients.

Due to the anatomical heterogeneity (Table 1), he-modynamic or anatomic parameters are not useful indescribing the group. We therefore chose to rely onfunctional parameters. Both the cardiologist and thecardiac surgeon classified twice and independently thephysical capacity of each patient according to the classifi-cation of the New York Heart Association.7 One wasbased on the response of the patients, the second reliedsolely on the information from the parents. Functionalclass was also derived from a standardized bicycle stresstest. The test started with 60 watts for two minutes,increasing with 30 watts every minute until the patientgave up. We divided the maximal performance in wattsendured for more than 29 seconds by the body surfacearea in meters squared. We used cutoff points at 50, 75and 100 watts per meter squared. The class of the NewYork Heart Association thus derived was used in allcalculations.

Psychiatric assessment

All adolescents were interviewed by a child psychiatrist.This interview included the Child Assessment Sched-ule,8 a semistructured interview designed to providepsychiatric diagnoses according to the Diagnostic andStatistical Manual of Mental Disorders.9 It also pro-vides a total score for pathology. Each interview lastedfrom two to four hours and covered details concerningthe cardiac malformation, attitudes, feelings, expecta-tions for the future, and so on.

At least one of each adolescent's parents was given asemistructured interview lasting for two hours. It cov-ered detailed information about previous and presentmedical problems and psychosocial functioning, thesomatic and mental health of the other members of thefamily, and how the family functioned and coped witha disabled child.

The psychosocial functioning was classified accord-ing to the Children's Global Assessment Scale.10"12 Onlyscores on the 10-level interval scale were used.

The mothers were asked to complete the ChildBehavior Checklist13 which covers different areas ofpsychosocial functioning of the adolescent. It consistsof 118 items concerning problems with behavior andemotion and 20 items concerning social competence.

Chronic Family Difficulties1415 were assessed, basedon all available information, including economy, hous-ing, employment, network support and possible maritalor family discord, as well as the mental and social healthof the parents and other members of the family. Carewas taken to avoid the effect of the cardiac malforma-tion 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

assessment, global assessment and chronic family diffi-culties were checked and found satisfactory as reportedelsewhere by Bjornstad, Lindberg & Spurkland.15 So-cioeconomic status was classified according to nationalcriteria.

Statistical procedures

In most instances non-parametric tests were used, be-cause our data only rarely were normally distributed andcontinuous. For correlations the Spearman rank corre-lation coefficient was used, with contingency tables andChi square test used to test binomial variables. Differ-ences in paired observations were assessed with Wilcox-on's rank sum test. Group analysis was performed eitherwith the Kruskall-Wallis test, or Mann-Whitney's l i -test. The parameters were entered into a data base in anIBM 4341 computer. The calculations were made withBMDP Statistical Software, version 1983 (UCLA, LosAngeles, CA, USA). A statistical p-value 0.05 wasconsidered "significant," a value 0.01 "highlysignificant," and lower values "very highly significant."These terms are subsequently used in place of specific p-values in the text.

Ethical aspects

In each case, written informed consent was obtained forthe study and additionally given separately for video-taping of the interviews for the assessment schedule.The study was approved by the ethical committee ofThe National Hospital.

Results

Clinical statusEight patients were visibly cyanotic, while six hadclubbing. One was slightly cyanotic and the rest wereacyanotic. Whereas the mean height and weight were inthe normal range, some patients were very short andmarkedly underweight (Table 2).

Seven patients had scoliosis, but none was in need oftreatment. Some sort of thorax deformity was seen in 11patients, mostly pectus carinatum. Some of these pa-tients complained about the shape of their thorax, butagain there was no indication for treatment. Deficien-cies in the pulses lacking in one or two of the limbs werediscovered in six patients after surgery or catheteriza-tion. No trophic disturbance was discovered in any ofthem, but half of them said, when asked, that they wereoften cold in the specific limb.

Fourteen patients are on cardiac medication, mostlydigoxin. All patients with mechanical valves are onwarfarin. Some take diuretics or afterload reducingmedication. Three patients, all boys, are smoking ciga-

Vol. 5, N o . 1 BjornstadetaL Physical and psychosocial status in adolescents with congenital heart disease 59

10

8

6

4

2

0

1 1III IV

Boys Girls

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

rettes. Twelve patients are now scheduled for furtherheart surgeryof a palliative nature in two, but correc-tive or as a repeated corrective attempt in 10.

Physical functioningThe information as given by the patients to the cardi-ologist or the cardiac surgeon produced an almostidentical score in the classification of the New YorkHeart Association, with a mean of 1.960.77. Theparental (mostly the mother's) judgment gave a mean of2.31 0.79, and the bicycle stress test a mean of 2.46 1.03.We compared the data using Wilcoxon's test. Thejudgment of the two doctors proved identical. Therewas no significant difference between the maternaljudgment and the score derived from the stress test, but

100-80 70-60 50-40Boys Girls I

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

Table 3. Psychiatric diagnoses in the Diagnostic and StatisticalManual of Mental Disorders9 of adolescents with severe con-genital cardiac malformation (n=26).

Psychiatric diagnosis Boys Girls Total

Overanxious disorderSeparation anxietyMajor depressionDysthymic disorderAttention deficit disorderConduct disorderTotal number of diagnosesDiagnosis in N of patients

41-

21-

84

2212_

1148

631411

2212

a significant overscoring was noted by the response ofthe patients themselves. The distribution of physicalcapacity according to the stress test is shown in Figure2. It is noteworthy that there are no girls in class I, andthat the girls outnumber the boys fourfold in class IV.This difference between boys and girls is statisticallysignificant.

Psychiatric disorders and psychosocial functioningTwelve adolescents (46%) met the criteria for diagnosesin the Diagnostic and Statistical Manual (Table 3). Theresults from children's global assessment scale (Figure3) placed one-third (seven boys, one girl) into the bestgroup. Another third (six boys, three girls) was assessedas functioning reasonably well with only minor tomoderate problems, but the last third (three boys, sixgirls) was seriously dysfunctioning. Significantly, moregirls than boys had impaired psychosocial function.

The mothers scored five of the adolescents (19%,two boys, three girls) to have psychological problems onthe child behavior checklist. They were among thepatients with the most pronounced psychiatric symp-toms.

Socioeconomic statusClassification of the socioeconomic status revealed nodifferences from the population in general.16

Chronic family difficultiesHalf of the families had none or only minor chronicdifficulties (mean 2.82.34). The other half had frommoderate to severe problems. In these cases, the difficul-ties were mainly caused by either somatic and mentalhealth problems in the parents or siblings, or conflictswithin the family.

IntercorrelationsThe intercorrelations between class of the New York

60 Cardiobgy in the Young January 1995

Table 4. Correlation coefficients between five differentvariables on physical and psychosocial function in 26adolescents with severe congenital heart disease.

NYHACGASCFDCAS

CGAS CFD

-0.40- 0.06"-0.66s

* p 0.05; t p 0.001;

CAS

0.46-0.67s0.28"

p 0.0001; n not signi

CBCL

0.24"0.64s

0.55*0.60s

ficant

Abbreviations: NYHA: functional class according to the New YorkHeart 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 assess-ment schedule, the children's global assessment scale,chronic family difficulties and child behavior checklistare listed in Table 4.

Discussion

The most important findings in this study are the highfrequency of major psychiatric problems noted in thisgroup of adolescents with severe congenital heart dis-ease, and the significant correlation found between thepsychosocial function and physical capacity (Table 4).

The psychiatric problems are atypically for age as faras boys are concerned, mostly representing anxiety anddepressive disorders. These seem very likely to be causedby the impact of the life threatening and incapacitatingcardiac malformation itself.

Psychosocial functioning was found to be poor innine patients (35%), and reduced in another nine. Thisis definitely more than is expected in the general popu-lation,17 and demonstrates the need for attention to bepaid also to the mental health in general of this group ofpatients. Moreover, the degree of psychosocial andphysical function correlate significantly. This demon-strates that the more reduced the physical capacity ofthe patient, the higher the risk for developing psychiat-ric disorders. This is in keeping with the findings ofSteinhausen et al,18 Wallander et al19 and Engstrem.20Several previous studies in patients with congenitalheart defects,1"4 nevertheless, found little or no increasein emotional problems, and no consistent relationshipbetween organic severity and psychosocial function.

North et al21 may have shown a possible explanationfor such discrepancies. Studying 138 reports on psychi-atric factors in ulcerous colitis, they found that 131 ofthem contained serious deficiencies in research meth-odology.

The mothers make accurate judgments of the physi-

cal capacity of their children. The old adage of thepediatrician "listen to the mother" has again beendemonstrated. But even the mothers underestimate theprevalence of psychopathology. This is in line with theobservation that parents are often less aware of theinternal affective state of their children.23

The distribution of physical capacity shown in Figure2 is significantly different in boys and girls. A markedpreponderance of females was particularly obvious inclass IV of the New York Heart Association (four offive). Even when excluding patients with left-sidedartificial valves (all boys, and all well functioning), westill find an obvious tendency towards poorer physicalcapacity in the girls (p=0.06). There is no cardiacexplanation for this difference between boys and girls,which we consider to be intriguing.

A significant overrepresentation of girls is also foundamong the patients with psychiatric problems. Becauseof the small number of patients in our study thispreponderance may or may not be accidental. If thefinding is not accidental, it represents an observationthat has been overlooked elsewhere. A possible explana-tion for the overrepresentation of girls in the less favor-able groups physically and mentally may be the signifi-cant correlation between physical and psychosocialfunction. It seems valid to assume, that (over)protectionand restrictions imposed on patients with severe heartdisease may be a greater risk for girls than for boys. Thefemale social role may represent a vulnerability factorfor developing psychosocial problems through reducedphysical training, whereas the social environment ofmales could represent a protective factor by demandingparticipation in physical activity and exercise. Thiscould overrule some of the restrictions implied byparents, teachers, doctors, and so on, and have animpact not only on the physical condition of thepatients. In that case, the difference between the sexes inboth aspects may, at least in part, be caused by adifferent degree of physical activity and training, whichshould lead us to encourage such physical activity,especially in girls.

It has been suggested1'3 that the degree of psychopa-thology and reduced psychosocial functioning may bedue to a direct harmful effect of physical incapacity andrestrictions, or a result of influence of parental anxietyand overprotection. Our data give additional supportfor this view. These reasons may also partly representthe link to reduced physical capacity. Organic cerebraldysfunction, either from the cyanotic cardiac lesionitself or from repeated open heart surgery, cannot be ruledout, but this question was not addtessed in this study.

Engstf0m20 also found in another group of chroni-cally ill patients that girls generally had higher pathol-ogy scores on most of the Child Behavior Checklistsubscales, particularly the subscales for somatic com-

Vol. 5, N o . 1 Bjornstadetah Physical and psychosocial status in adolescents with congenital heart disease 61

plaints, depression and anxious-obsessive symptoms.Due to the small size of our samples, nonetheless, weshould be careful with our conclusions. Despite thesecaveats, we think that our two first hypotheses havebeen confirmed. But it would be worthwhile to makefurther studies to assess more clearly if the differencebetween the sexes holds true.

We can easily imagine that a severe, life threateningcardiac lesion has a major influence on mental health.The association between psychosocial functioning andenvironmental long-standing strain (chronic familydifficulties) has been documented,1422 and further con-firmed in this study. The correlation between class inthe New York Heart Association grading and chronicfamily difficulties was not significant, indicating that amajor influence from the cardiac malformation itselfwas avoided in our assessment (Table 4). On the otherhand, a very highly significant correlation was foundbetween family difficulties and the score on the globalassessment scale, thus indicating the impact of familydifficulties on psychosocial function, thereby confirm-ing our third hypothesis.

We are pleased to find, nonetheless, that half of ourpatients with these severely malformed hearts are func-tioning in class I or II of the scale of the New York HeartAssociation. This certainly is a merit of modern cardi-ology and cardiac surgery. The patients will probablyhave reduced life expectancy, but half of them display aphysical capacity enabling them to lead reasonably goodlives.

The finding that eight of our 26 patients (31 %) are incompletely normal mental health made us look forprotecting factors in these patients. Some were found.First, a link was observed to good physical capacity, anargument in favor for early repair, if possible, or pallia-tions without delay. Adequate physical activities andtraining should also be encouraged for all patients,particularly the girls. Second, the adverse effect ofchronic family difficulties on psychosocial develop-ment, leads us to emphasize the importance of consid-ering the whole family. This includes not only parents,but also other family members, friends of parents andpatient, and the school, organizations, clubs, churchesand 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 feelconfident that the increasing interest in protective factors24will lead to future studies focusing on these aspects.

Our study indicates that, whereas the somatic parthas been taken care of satisfactorily, too little attentionhas been paid to psychological consequences. Increasedefforts within this, often underestimated, area mightimprove not only mental health in this group of pa-tients, but influence the total quality of life. Optimal

treatment of patients with congenital heart diseaserequires somatic, as well as psychological and family-related, care and follow-up.

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