a The department of Child and Adolescent Psychiatry, Erasmus MC Sophia Children's Hospital, The Netherlands
b The department of Cardiology Thoraxcenter, Erasmus MC, The Netherlands
c The department of Cardiothoracic Surgery, Thoraxcenter, Erasmus MC, The Netherlands
Received September 11, 2003; revised April 21, 2004; accepted June 10, 2004 * Corresponding author. EHM van Rijen, Tel.: +31-10-4089755; fax: +31-10-408-9009 (E-mail: vanrijen{at}fsw.eur.nl).
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Abstract |
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Methods and results This study concerns the second psychological and medical follow-up of a cohort of patients operated for congenital heart disease (n=362; age 2046 years). Behavioural and emotional problems were assessed with the Young Adult Self-Report and the Young Adult Behavior Checklist. Medical prediction variables were derived from medical examination and file search. Being female, having low exercise capacity and restrictions imposed by physicians are significant predictors for behavioural and emotional problems as reported by patients themselves. Regarding the scar, personal experiences of patients form a better predictor for later problems than judgement of aesthetical aspects by physicians. Early hospitalisations with reoperations are predictive for behavioural and emotional problems as reported by other informants. The cardiac diagnoses of ventricular septal defect and transposition of the great arteries are associated with higher levels of behavioural and emotional problems.
Conclusion Recent experiences concerning the scar, physical condition and imposed restrictions are the strongest predictors for behavioural and emotional problems as reported by patients themselves.
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Introduction |
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The present study was preceded by a first follow-up conducted in 19891991, which contained a prediction study on behavioural and emotional problems in children and adolescents with congenital heart disease.6 Since all patients had reached adulthood at the time of the second follow-up, we were able to perform a similar prediction study on a large sample of, now all adult, congenital heart disease patients.
Investigating predictors for psychopathology in congenital heart disease adults could be helpful for identifying individuals at risk. Also, aspects in the treatment and support of congenital heart disease adults which need special attention could be specified. The main aim of this study was to determine to what extent variables concerning: (1) biographical status, (2) medical history, (3) first open heart surgery and direct post-operative course, (4) medical course before 1990, (5) medical course after 1990, (6) present contacts with physicians, and (7) present medical status can predict long-term behavioural and emotional problems in adulthood.
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Methods |
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The target population of the present follow-up (20002001) consisted of the 498 patients of the first follow-up. From the 498 patients, we excluded 61 patients who belonged to a miscellaneous diagnostic group, consisting of small numbers of patients with a variety of congenital heart defects (aortic stenosis, discrete subaortic stenosis, atrio-ventricular septal defect, pulmonary atresia, tricuspid atresia, total abnormal pulmonary venous drainage, truncus arteriosus, and further miscellaneous diagnoses). The numbers of patients belonging to each of these miscellaneous diagnoses were excluded, since they were too small (fewer than 5 patients) to allow statistical analyses, consequently making it impossible to draw conclusions, representative for these diagnoses. Therefore we chose to include only the diagnoses with a number of patients large enough to allow statistical analyses. Furthermore, we excluded 11 deceased patients, 26 untraceable patients and 1 patient who had undergone a heart transplant.
Patient sample
Of the remaining 399 patients, 37 refused to participate. Among the participating 362 patients, 10 mentally retarded patients were unable to complete the self-report questionnaire and 3 questionnaires were not usable because of incomplete information, resulting in 349 self-reports. Regarding other-informant reports, of the 362 participating patients, for 1 mentally retarded patient the questionnaire was not applicable, 3 patients did not have a suitable other informant because of language problems, 19 questionnaires were not filled in and 5 questionnaires were not usable because of incomplete information, resulting in 334 other-informant reports. The overall response rate, corrected for deceased persons and persons lost to follow-up was 90.7%, resulting in usable self-reports and other-informant reports for, respectively, 87.5% and 83.7% of the eligible patient sample.
The part of the patient sample for which self-reports were available consisted of 188 males and 161 females (mean age: 30 years, age range 2046 years). Numbers of patients belonging to each diagnostic group were: closure of atrial septal defect (ASD; n=93), closure of ventricular septal defect (VSD; n=92), corrective surgery for tetralogy of Fallot (ToF; n=72), Mustard procedure for transposition of the great arteries (TGA; n=55) and surgery for pulmonary stenosis (PS; n=37). The mean ages in these diagnostic groups were 33, 30, 30, 26 and 32 years, respectively. Medical history and physical condition of these patients at the time of the first follow-up912 and second follow-up13 are described elsewhere.
Outcome variables
Psychopathology in young adults with congenital heart disease was assessed with a self-report questionnaire: the Young Adult Self-Report (YASR)14 and a parallel questionnaire to be completed by next of kin, such as a parent or a spouse: the Young Adult Behavior Checklist (YABCL).14
Good reliability and validity of the YASR14 have been confirmed for an earlier Dutch version of the YASR.1517 Ferdinand et al.,15 reported an 18-day test-retest reliability of r=0.89 for the Total Problem Score, and an average Cronbach's alpha across syndromes of 0.84. Wiznitzer et al.,16 and Ferdinand and Verhulst17 demonstrated good discriminative performance of the YASR, compared to other questionnaires, indicating good construct validity. The YASR contains 110 problem items. The response format is 0="not true", 1="somewhat or sometimes true", and 2="very true or often true". The YASR can be scored on 8 syndrome scales: Anxious/Depressed, Withdrawn, Somatic Complaints, Thought Problems, Attention Problems, Intrusive Behaviour, Delinquent Behaviour, and Aggressive Behaviour, and two broad-band groupings of syndromes: Internalising (consisting of the Anxious/Depressed and Withdrawn scales) and Externalising (consisting of Intrusive Behaviour, Delinquent Behaviour and Aggressive Behaviour scales). Internalised problems are experienced more internally (feeling anxious or depressed, withdrawn), whereas externalising problems are more acted out (intrusion, delinquency, aggressiveness). Examples of internalising problem items are: "I feel lonely", and "I am shy or timid". Examples of externalising problem items are: "I try to get a lot of attention", "I lie or cheat", and "I have a hot temper". Reports on these items reflect the patient's standing on behavioural and emotional problems in relation to a normative sample. A Total Problems score is computed by summing the individual item scores. High scores indicate the presence of behavioural and emotional problems and thus are unfavourable; Low scores are favourable.
The YABCL,14 a parallel version of the YASR, is to be completed by an informant who is familiar with the young adult, including a partner, parent or other close relative or friend. The YABCL contains 105 problem items and consists of the same scales as the YASR. Good reliability and validity was reported for the YABCL.14 For the Dutch version of the YABCL, an average Chronbach's alpha across syndromes of 0.81 was found.18
The YASR and YABCL were originally developed for 18- to 30-year olds. However, since normative data were not used in this prediction study, we decided to include the complete patient sample. The problem items seem largely relevant for patients above the age of 30 years and it was thus considered appropriate to administer the YASR and YABCL in the total patient sample. Moreover, successive versions of the YASR and YABCL, namely the ASR and ABCL,19 with extended age ranges from 18 to 59 years, are now available. The ASR and ABCL contain parallel items compared to the YASR and YABCL. For this particular study, only the scales Internalising, Externalising and Total problems of both YASR and YABCL were used.
The YASR and YABCL syndromes were empirically derived by performing principal components/varimax analyses on the problem items derived from clinical practice, in order to identify patterns of co-occurring problems ("bottom up" approach). This is in contrast with the widely used DSM diagnostic constructs, which were formulated using a "top down" approach. Furthermore, the YASR and YABCL are self-report and other informant-report questionnaires, whereas DSM criteria for psychopathology are usually assessed by clinicians, using structured diagnostic interviews. In spite of these differences, high associations were found between YASR Total Problems and DSM-III-R diagnoses in both an American clinical sample20 and a Dutch general population sample,21 with correlation co-efficients of 0.67 and 0.74, respectively.
Prediction variables
In order to examine the predictive value of medical variables on psychopathology in congenital heart disease adults, 7 clusters of prediction variables were chosen on theoretical and/or medical grounds. The relevance of the prediction variables was evaluated based on literature and the clinical experience of both the involved cardiologists as well as the psychologists. Data were partly derived from a retrospective medical file search and medical examination by a cardiologist during the first follow-up (F.M.), and partly derived form medical examination by a cardiologist during the second follow-up (J.R.).
The variable "date first open heart surgery" was converted to the number of days that elapsed since the first patient of this cohort received open heart surgery. This was done to examine accurately the impact of the exact period in which the first surgery took place, since surgical techniques developed at a high pace. The variable "post-operative course" was dichotomised: 0=no complications, 1=with complications. The variable "results operation" was dichotomised: 0=good, 1=moderate or poor. Most dichotomous prediction variables were coded as: 0=favourable or risk absent and 1=unfavourable or risk present, except for the prediction variables "sex" (0=female, 1=male) and 'ECG sinus rhythm' (0=sinus rhythm absent, 1=sinus rhythm present). The maximum exercise capacity (derived from bicycle ergometry) is the percentage from what might be expected considering age, sex and body weight, compared to the normal Dutch population. The operational definition of the variable "Restrictions by scar experienced by patients" was: "Do you feel restricted by the scar?".
Assessment procedures
All patients were traced, approached uniformly and signed an informed consent before participating. During their visit to the Erasmus Medical Center in Rotterdam, patients were interviewed and tested by a psychologist (EvR) and medically examined by a cardiologist. The YASR was filled in by the patient during the psychological examination. Some patients (n=28) who could not visit the hospital for practical or emotional reasons completed the YASR at home and returned it by mail. The YABCL was filled in at home, by someone familiar to the patient (partners, spouses: 64%, parents: 32%, siblings, close friends and others: 4%) and was returned by mail. For patients, who were accompanied during their hospital visit by a familiar person (e.g., parent, spouse), the YABCL was completed in the waiting room.
Statistical Analyses
A three-phase strategy was followed for each of the six outcome measures. Linear regression analysis was applied with only main terms entered into the regression model. Since socio-economic status did not have a main effect on any of the outcome variables, it was not included in the models. In phase 1, each of the separate prediction variables was tested on the YASR and YABCL outcomes (univariate analysis). This was done to explore the predictive quality of each predictor separately. In phase 2, each cluster (i.e., combination) of predictors was related to the YASR and YABCL outcomes (multivariate analysis). The following clusters were used: biographical status, medical history, first open heart surgery and direct post-operative course, medical course before 1990, medical course after 1990, present contacts with physicians and present medical status. Since this phase served as a first, broad selection of predictors, the p-values were set to levels of 0.20 (backward elimination procedure). Variables showing in the regression model, applied to the clusters of prediction variables, were candidate predictors for the final model. In order to correct for sex- and age effects, sex and age were forced into each analysis in phase 1 and 2. The final model in phase 3 contained all significant variables from phase 2 (p-values were set at 0.05, backward elimination procedure). Variables showing significant results in this final model were regarded as the final predictors of YASR and YABCL outcomes. In the tables, the betas presented are standard regression co-efficients which express the strength of the relationship between each prediction variable and the outcome variable. In Table 2, non-standardised regression co-efficients with their standard errors are additionally provided. Plus versus minus indicates, respectively, the positive versus negative direction of the relation between the prediction variable and the outcome variable.
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The linearity assumption was assessed by examining the scatter plots, with the continuous predictors on the x-axis and the dependent variables on the y-axis. The scatter plots showed that no other than linear relationships were to be expected. The assumption of normality of the residuals was examined with histograms of the residuals and normal pp plots of the regression standardised residuals. Both methods illustrated that the assumption of normality was satisfied. The assumption of constant variance of the residuals was assessed by examining scatter plots with the regression standardised residual on the x-axis and the dependent variable on the y-axis. These scatter plots showed that the assumption of constant variance of the residuals was satisfied.
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Results |
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Phase 3: the final model
The results of the final prediction model are presented in Table 2. "Restrictions by scar experienced by patients (sometimes)" was related to higher problem scores on nearly all outcome variables, except for YABCL Internalising. Reoperation(s) before 1990 significantly predicted higher scores on all YABCL scales. Being female significantly predicted higher scores on YASR Internalising and Total problems. Poor physical condition (low maximum exercise capacity) was associated with higher scores on YASR Internalising and Total problems. The diagnostic categories of ventricular septal defect and transposition of the great arteries were associated with higher scores on YASR Externalising. Ventricular septal defect was also associated with higher scores on YABCL Total problems. Restrictions imposed by physician significantly predicted higher scores on YASR Total problems. "Scar judged by physician (moderately healed)", hospitalisations without reoperation(s) before 1990 and having a pace-maker were associated with lower (that is favourable) scores on some outcome variables.
R2 is the percentage of variance in the outcome variable explained by the final predictors. Prediction variables of the YASR outcome explained higher percentages of variance than the prediction variables of the YABCL outcome on the parallel scales.
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Discussion |
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Early hospitalisations
Reoperation before 1990 forms an important predictor in the other informants' report (YABCL) of psychopathology. Remarkably, the medical course across the last decade (after 1990) does not seem to predict psychopathology in congenital heart disease patients, as reported by other informants as well as by patients themselves. An explanation could be that hospitalisations with reoperation in childhood or adolescence have a greater impact for the congenital heart disease patient than the occurrence of such medical experiences in adulthood. However, reoperations before 1990 are not of any importance in the self-report (YASR) of psychopathology. A more likely explanation thus might be that hospitalisations with reoperation performed in childhood or adolescence have a greater impact for others in the environment of the congenital heart disease patient than reoperations which occur in adulthood. In childhood, parents are closely involved in the care of their child and feel particularly responsible when their child is more vulnerable as a result of congenital heart disease. Psychological distress has frequently been observed in parents of children with congenital heart disease.2327 Parents' concerns and anxieties about the development of the child with congenital heart disease might continue into adulthood and might influence the image others (e.g., spouses) have of them as adults. This explanation is supported by a study of DeMaso et al.,4 who found that maternal perceptions were more potent predictors of emotional adjustment in congenital heart disease children, compared to medical severity. It remains unclear why hospitalisations, without reoperation, before 1990 seem less predictive for psychopathology compared to no hospitalisations before 1990.
Sex differences
Being female significantly predicts higher rates of particularly internalising problems, e.g., being anxious or depressed. Previously, higher rates of psychopathology for female patients, compared to male patients, were found in this same cohort. This trend is also seen in the general population.28 However, young congenital heart disease females particularly showed significantly more behavioural and emotional problems than a sex- and age-comparable reference group (van Rijen et al., unpublished data). Being female can thus be considered as a specific predictor for psychopathology in congenital heart disease adults. There are several explanations for these sex differences. Firstly, there is the surgical scar, which is unfavourably situated on the chest. Especially for young females, the scar can therefore be a source of uncertainties or discomfort, for example when entering sexual relationships.22 Secondly, uncertainties regarding abilities of pregnancy and delivery might play a role. For female patients, these issues concern their own bodily functioning and might therefore cause anxiety about their physical condition.22,2931 This is illustrated by the fact that, in this sample, 44.4% of the female patients reported that the congenital heart disease was or had been a limiting factor in the choice of having children.
Maximum exercise capacity
Low maximum exercise capacity also predicts higher rates of particularly internalising problems. In this context, clinical observations show that, during psychological examination, many congenital heart disease patients reported to think of the bicycle ergometry as an unpleasant and fearful experience. For adult congenital heart disease patients, being in poor physical condition might lead to concerns and anxieties about one's health. Health experiences can be very determining for the well-being of patients with congenital heart disease.3234 Fredriksen et al.,35 found a decrease of internalising problems in children and adolescents with congenital heart disease who were submitted to a physical training intervention. Possibly, for congenital heart disease adults, physical training might have the same favourable effect and should be considered in these patients.
Cardiac diagnosis
Patients who underwent surgery for ventricular septal defect and transposition of the great arteries have a higher risk of developing particularly externalising problems, e.g., intrusive and aggressive behaviour. It is striking that the diagnostic category transposition of the great arteries is more prominently present as significant predictor in phase 1 and 2 compared to the final model in phase 3. Since transposition of the great arteries can be considered as a severe condition with far-reaching consequences for the adult patient's life (Roos et al., unpublished data), it is probable that they also have high ratings on the other prediction variables. Correction for the other prediction variables in the final model might explain why transposition of the great arteries has receded more to background. This would imply that factors associated with the diagnosis transposition of the great arteries, such as lower maximum exercise capacity, rather than the diagnosis itself, are predictive for psychopathology at adult age. In a study of children with cyanotic heart defects, similar results were found by DeMaso et al.36 They stated that the diagnosis of a severe cyanotic heart defect does not appear to make a child more likely to have emotional disorders in the absence of other factors, such as impairment of the central nervous system. Higher ratings of externalising problems in patients with ventricular septal defect and transposition of the great arteries might be related to overcompensation of restrictions experienced as a result of the congenital heart disease, since risky and defiant behaviour have been reported during psychological examination.
Restrictions imposed by physician
Restrictions imposed by a physician (for example concerning physical activity, life style, reproductive issues) form a significant predictor for overall problems as reported by patients themselves. Besides restrictions that are imposed by a physician, restrictions that congenital heart disease patients themselves experience subjectively, as a result of uncertainties or misconceptions, might also play a role in the occurrence of behavioural and emotional problems.
Pace-maker
Remarkably, having a pace-maker (which was the case for 30 patients in the sample) was a significant predictor for less externalising problems as reported by others close to the patient. Alpern et al.,37 found overall normal psychosocial adaptation of children with cardiac pace-makers. Little is known about the adjustment of adult congenital heart disease patients with pace-makers. Alpern et al.,37 found that the self-image of young pace-maker patients is not different from that of peers, while peers assume that children and adolescents with pace-makers are more anxious, depressed, embarrassed, socially isolated, or diminished in their sense of self-worth. Similar assumptions might underlie the low externalising behaviour as reported by the others close to the patient.
Different informants
It is striking that some predictors of psychopathology in congenital heart disease adults are informant-specific. Predictors that are exclusively predictive for psychopathology as reported by the patients themselves, project the recent personal situation of the patient (maximum exercise capacity, restrictions imposed by physician). Predictors that are exclusively predictive for psychopathology as reported by others, mostly project the early course of hospitalisations. The level of psychopathology reported by congenital heart disease patients thus has a stronger relation with the recent medical status and relevant subjective experiences compared to the level of psychopathology reported by the others close to the patient. In the psychological evaluation of congenital heart disease patients, it therefore seems particularly important to carefully take the role of different informants into account.
Limitations
It could be argued that, as a maximum of 12% of the variance is explained, psychopathology is only marginally determined by medical factors. The predictors indicated in Table 2 however kept their significance throughout a three-phase model. The predictive value of these medical variables could therefore be considered small, but certainly persistant and significant. Taking into account the complete medical course from birth till the present from which the medical variables were derived, their predictive values can be considered remarkable.
The strength of our model is that the final predictors remained significant during all three phases. Since this study can be considered as a first, broad, explorative study, we did not apply conservative corrections for multiple testing. Therefore, false positive results cannot be excluded. Future research should therefore examine the predictors found in this study.
For most medical predictors, the cause-effect relationship is fairly obvious. One could argue however that this relationship is not so obvious for the variables representing the present medical status. Four out of these six variables however, are objective medical measures (medication for the heart, pacemaker, maximum exercise capacity and sinus rhythm), and thus can be considered appropriate candidate predictors. The variable "scar judged by the physician" is very unlikely to be predicted in reverse order by the patient's status of psychopathology. In this respect, the most questionable predictor would be "restrictions by scar experienced by patients". In this study, it was assumed that experiences of restrictions of the scar predict the patient's psychopathology, but the reverse relation cannot be excluded.
The unique longitudinal measurements enabled us to evaluate the effects of life-span medical experiences on psychopathology at adult age in this now all adult cohort, representing the overall first generation of adults with congenital heart disease. The results offer possibilities to improve patient care and are therefore of clinical significance.
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Conclusion |
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Acknowledgments |
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References |
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