Original Article 281
Health Related Quality of life after Corrective Surgery for Congenital Heart Disease Lebensqualität nach operativer Korrektur eines angeborenen Herzfehlers
Affiliations
Key words ▶ health related quality ● ▶ heart defect ● ▶ children ● ▶ congenital ● ▶ parents ● Schlüsselwörter ▶ Lebensqualität ● ▶ Herzfehler ● ▶ Kinder ● ▶ angeboren ● ▶ Eltern ●
Bibliography DOI http://dx.doi.org/ 10.1055/s-0034-1382019 Published online: July 25, 2014 Klin Padiatr 2014; 226: 281–286 © Georg Thieme Verlag KG Stuttgart · New York ISSN 0300-8630 Correspondence Dr. Andreas Heusch Abteilung Pädiatrische Kardiologie/Pulmonologie Helios-Klinikum Wuppertal Heusnerstrasse 40 42238 Wuppertal Germany Tel.: + 49/202/8963 835 Fax: + 49/202/8963 839 andreas.heusch@ helios-kliniken.de
A. Heusch1, G. Calaminus2, J. Kahl3, K. Schmidt4 1
Abteilung Paediatrische Kardiologie/Pulmonologie, Helios-Klinikum Wuppertal, Wuppertal Pediatric Oncology, University Muenster, Muenster, Germany 3 Kinderkardiologische Praxis, Uhlandstrasse, Duesseldorf, Germany 4 Pediatric Cardiology, University Duesseldorf, Duesseldorf, Germany 2
Abstract
Zusammenfassung
After corrective surgery for congenital heart defects (CHD) many patients suffer from residual defects, some with reduced cardiorespiratory capacity and possible impairment of their health related quality of life (HRQoL). The aim of our study is to evaluate, how children after surgery for CHD rate their HRQoL. Method: A standardised questionnaire developed for self-rating in children between 8 and 18 years and dealing with 7 different domains concerning the HRQoL (friends, family, physical functioning, cognition, body image, emotional function and autonomy) was independently answered from patients and their parents during an outpatient visit at their pediatric cardiologist throughout Germany. Results: 173 patients (40 % female, 60 % male, mean age 11.6 years) were interviewed, 167 questionnaires could be evaluated. The mean time interval after surgery was 9.8 ± 3.4 years. Patient had had surgery for complete different types of CHD (ventricular septal defects n = 50, Tetralogy of Fallot n = 51, univentricular heart n = 26, transposition of great arteries n = 40). The results were compared with those of an agematched control group (n = 169). Patients with CHD reported a better HRQoL than the controls for all items (p < 0.01). There was no significant gender specific difference. After puberty, the rating for most items, except of “body image“, had become very similar compared to controls. Parents assessed their children significantly worse in 3 domains (friends, body image and emotion; p < 0.01). Conclusion: Children with congenital heart defects are able to develop coping structures, that enable them to live a normal life from their individual point of view. Integration in psychosocial structures seems to be rather normal when compared to healthy controls. Many patients considered their HRQoL as even better.
Nach operativer Korrektur eines angeborenen Herzfehlers (CHD) bestehen bei vielen Patienten Restdefekte. Bei einigen gehen diese mit einer verminderten kardiorespiratorischen Leistungsfähigkeit und einer möglichen Einschränkung der gesundheitsbezogenen Lebensqualität (HRQoL) einher. Ziel unserer Studie war es, die Selbsteinschätzung der HRQoL bei Kindern nach Korrektur eines angeborenen Herzfehlers zu evaluieren. Methode: Ein Fragebogen, der für die Selbsteinschätzung von Kinder und Jugendlichen von 8–18 Jahren entwickelt wurde und sich mit 7 unterschiedlichen Bereichen der HRQoL befasst (Freunde, Familie, körperliche Fähigkeit, kognitive Leistung, Körperbild, emotionale Fähigkeit und Selbstständigkeit), wurde von Patienten und Eltern unabhängig von einander bei einem Besuch ihres niedergelassenen Kinderkardiologen deutschlandweit beantwortet. Ergebnisse: 173 Patienten ( 40% männlich, 60% weiblich, mittleres Alter 11,6 Jahre) wurden befragt, 167 Fragebogen konnten ausgewertet werden. Der mittlere Abstand zur Operation betrug 9,8 ± 3,4 Jahre. Bei den Patienten waren die unterschiedlichsten Herzfehler korrigiert worden (Ventrikelseptumdefekt n = 50, Fallot‘sche Tetralogie n = 51, Univentrikuläres Herz n = 26, Transposition der großen Gefäße n = 40): Die Ergebnisse wurde mit denen einer Kontrollgruppe vergleichbaren Alters (n=169) verglichen. Patienten mit angeborenem Herzfehler beschrieben in allen Bereichen eine bessere HRQoL als die Kontrollpersonen (p < 0,01). Es bestanden keine geschlechtsspezifischen Unterschiede. Nach der Pubertät wurden die meisten Bereiche mit Ausnahme des Körperbildes ähnlich wie bei Gesunden eingeschätzt. Die elterliche Einschätzung war in 3 Bereichen signifikant schlechter als die der Patienten (Freunde, Körperbild, emotionale Fähigkeit, p < 0,01).
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Heusch A et al. Health Related Quality of … Klin Padiatr 2014; 226: 281–286
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Authors
282 Original Article Zusammenfassung: Kinder mit angeborenem Herzfehler entwickeln Verarbeitungsstrategien, die Ihnen aus eigener Sicht ein normales Leben erlauben. Verglichen mit gesunden Kindern, scheint die Integration in psychosoziale Strukturen weitgehend normal. Viel Patienten schätzen ihre HRQoL sogar besser ein.
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CHD HRQoL VSD ToF TGA fri fam phys cog bod emo aut
Congenital heart defects Health Related Quality of live Ventricular septal defect Tetralogy of Fallot Transposition of the great arteries Relations to friends Interactions in the families’ Physical capacity Cognitive abilities Patient’s body image Emotional function Aspects of autonomy
Introduction
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During the last decades, optimised surgical techniques as well as improved diagnostic tools and medical treatment have led to an increasing number of long-time survivors after corrective surgery for congenital heart defects (CHD) in childhood. Many patients, however, suffer from residual defects, some with severe impairment of the hemodynamic functional capacity. Several studies have reported a high prevalence of complications in different organ systems [20] or reduced cardio respiratory capacity during follow-up studies [8, 30]. Health related quality of life (HRQoL), which is a subjective selfassessment of the individual well-being, is not only determined by objective data such as functional or laboratory tests. HRQoL is highly affected by the ability of patients and their families to cope with the problems caused by the disease. Therefore, HRQoL is influenced by different aspects which are determined by individual and objective variables [9]. In the present study, we aimed to evaluate how schoolchildren and adolescents rate their HRQoL concerning the most important aspects of daily life after surgery for CHD. Furthermore, we also asked parents to rate their children’s HRQoL, enabling us to compare self- and proxy-reported values.
repair of complete transposition of the great arteries (TGA, atrial or arterial switch), or staged implementation of a Fontan-type circulation for univentricular AV-connection. Surgical procedures in these patients had been performed at different cardiothoracic units throughout Germany. Patients with additional handicaps or known syndromes were not included. Controls were matched according to age and sex from the sample of about 2 000 children and adolescents who had participated in the PedQol evaluation [3, 4]. PedQol evaluation interviews had been conducted at different types of German schools, participation had been voluntary. There were no further selection criteria.
Questionnaire The study centre distributed the questionnaires to the local paediatric cardiologist. After informed consent was obtained, the questionnaire was independently and anonymously answered by the patients and their parents. Younger children were assisted by a receptionist. Subsequently, questionnaires were returned to the study centre for further evaluation. In the first part, the questionnaire asked for demographic data as well as details of the CHD and the surgical procedure according to the classification of the Association for European Paediatric Cardiology [1]. In the second part, the questionnaire asked for HRQoL-items using PedQol, a standardised feedback form that was developed for self-rating in schoolchildren. An adapted form, implying the same questions on patients HRQoL was answered by the parents. PedQol had recently been used for assessment of HRQoL in children with chronic diseases [3, 26]. The PedQol-questions focus on 7 different domains: the relations of the patients to their friends (fri), the interactions in the affected families (fam), the physical capacity (phys), cognitive abilities (cog), the patient’s own body image (bod), their emotional function (emo), and aspects of autonomy (aut). Each domain is explored by a group of different indirect questions (6–12 for each item), and for each question, 1 of the 4 frequency ranks (never, rarely, often, and always) must be chosen as an answer. The patients’ rating was compared to that of healthy schoolchildren (control group), matched for age and gender and to that of their own parents.
Statistics Methods
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Patients and controls 173 Children and adolescents (7–18 years) with CHD after surgery were interviewed during a routine follow-up outpatient visit at their local paediatric cardiologist. 16 medical practices throughout Germany had agreed to recruit patients for the study. Data on response rates is not available; conversations with recruiting doctors suggest that selection was generally independent of patient characteristics and mainly depended on whether time was available. All patients, together with their parents, who agreed to the study and who had gone through one of the following cardiac surgeries were interviewed: repair of a ventricular septal defect (VSD), repair of Tetralogy of Fallot (ToF), Heusch A et al. Health Related Quality of … Klin Padiatr 2014; 226: 281–286
Statistical analysis was performed using commercial software programs (IBM SPSS statistics 18®). Rating of each PedQol domain is reported as mean, standard deviation, median, minimum, maximum and the interquartile area. Rating levels in each domain (never, rarely, often, always) were allocated to increasing negativity (1–4) and were related to a specific percentage range (25 %, 50 %, 75 %, 100 %). From these percentages, mean values for every domain and every patient were calculated and the mean for compared groups were determined. Differences between groups were analysed with the Mann-Whitney-Test or the Kruskal-Wallis-Test where appropriate. P-values < 0.05 were considered to indicate significant differences between groups; p-values < 0.1 were notable trends.
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List of abbreviation
Original Article 283
Comparison between patients and controls was done by a matched pair analysis.
answered the questionnaire as well as 48 of the 52 after correction of Tetralogy of Fallot, 38 of the 40 after TGA correction, and 25 of 26 after Fontan-type operation.
Results
Comparison of patients and controls
Patients Out of 173 patients interviewed, 167 questionnaires were returned completely and could be evaluated further. The mean age was 11.6 years; 101 were male (60 %) and 66 female (40 %) patients. 71 patients were younger than 11 years, 67 were between ▶ Fig. 1). For 11 and 14 years and 29 were older than 14 years (● each domain, percentages of ranking levels were compared with those of age-matched controls. 50 patients had a VSD that was closed surgically, 51 had Tetralogy of Fallot after correction, 26 patients had different types of univentricular AV-connection and a Fontan-type procedure, 40 had complete transposition of the great arteries after corrective surgery (28 arterial switch, 12 atrial switch). The mean time interval after surgery was 9.5 ± 3.1 years. The distribution of sex and age at interview was almost equal in the subgroups of different diagnoses with the exception of the TGA group where more male patients (78 %) were present.
Independently of gender, age and type of CHD, patients reported a significantly better HRQoL than controls for all items (p < 0.01), ▶ Fig. 2). especially for the domain “body image” (● When comparing female and male patients, there was no significant gender specific difference in the CHD group. There was a tendency for a more positive rating in the male group for the domains “cognitive abilities”, “body image” and “autonomy” ▶ Fig. 3). In contrast, the control subjects showed a more posi(● tive rating in the female group, which was significantly different in “emotional functioning”, “cognitive abilities”, “physical capacity” and “body image”. CHD-patients had a tendency to rank most items except for “friends” and “autonomy” more negatively with increased age. Especially patients older than 15 years rated the domains “family” (p = 0.043), “physical capacity”, “cognitive abilities” (p < 0.001), and “emotional functioning” (p = 0.036) less positive than younger children. Only the domain “autonomy” was rated ▶ Fig. 4). less negatively by these older CHD-patients (p = 0.014) (●
Parents During the same outpatient visit, 161 parents were interviewed independently; all 50 parents of children after VSD closure
prepubertal (≤ 10 years)
♂ ♀
%
n
16 14 12 10 8 6 4 2 0
fam
37.50 37.50
phys
37.50 37.50
pubertal (11–≤ 14 years)
postpubertal (≥ 15 years)
cog
42
39
20
bod
29
28
9
45.00 43.75 35.00 30.00 37.50 37.50
emo
45.83 41.67
aut 0
20
40 % Median female
7
8
9
10
11
12
13
14
15
16
17
fam
37.50
phys
37.50
43.75
P= 0.17
43.75
P=0.025
bod
aut
40 % Median
Healthy control
bod
p< 0.001
emo
p= 0.036
aut
p= 0.014
P < 0.01 P=0.005
45.00
20
p= 0.043
cog
40.00 37.50
emo
fam
P< 0.01
50.00
30.00
male
phys
50.00 45.00
cog
80
fri
43.75 40.00
fri
60
Fig. 3 Median of ranking of Quality of Life in patients with CHD. Comparison of 99 male vs. 67 female patients. High value indicates negative ranking (absent values are due to missing ranking).
18
Fig. 1 Demographic data and age distribution of the patient group.
0
40.00 40.00
fri
55.00
60
P < 0.01
0
20
80
CHD patients
Fig. 2 Median of ranking of Quality of Life in patients with CHD compared to healthy controls. High value indicates negative ranking. Statistical significance is given with p-values.
≥15 years
40 % Median 11–≤14 years
60
80
P< 0.05 (Kruskall-Wallis) ≤10 years
Fig. 4 Median of ranking of Quality of Life in 167 patients with CHD according to the age groups. High value indicates negative ranking (absent values are due to missing ranking).
Heusch A et al. Health Related Quality of … Klin Padiatr 2014; 226: 281–286
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284 Original Article
years). In the controls, especially “emotional function” in females and “autonomy” in males were rated worse. In the older patients ( > 15 years), females after puberty rated the domains “autonomy”, “cognitive abilities”, and “family” worse than males, while in the controls females rated “body image” significantly ▶ Fig. 6a, b). worse than patients and healthy males. (●
In CHD-patients after puberty, the rating for most items had become very similar compared to controls. In particular, the domain “body image” was rated higher after puberty by all CHD▶ Fig. 5). patients than by the controls (p < 0.01) (● Looking at the gender specific differences in the various age groups, there were no differences in the younger patients ( < 10
Parents rating When comparing the rating of parents and their affected children, parents rated worse in all domains except “physical capacity”. The difference was significant in the 3 domains “friends”, ▶ Fig. 7). In the patients with “body image”, and “emotion” (● Fontan-circulation and after Fallot-repair, the largest differences occurred between the ratings of patients and those of their parents.
40.00 40.00
fam
43.75
phys
43.75 43.75
50.00
50.00 50.00
cog
60.00
bod
P< 0.01
35.00
Discussion
45.00 43.75
emo
▼
55.00
aut
45.83
20
0
40 % Median
Healthy control
60
The main intention of cardiac surgery in patients with congenital heart defects is to allow long-time survival and to improve the quality of life. After corrective surgery for congenital heart defects, however, many patients may experience functional residual deficits especially those with complex defects [27]. In some of these patients residual deficits may lead to reduced cardio respiratory capacity [30]. Furthermore, the neurological outcome of children after surgery for heart defects may be affected; some children show various deficiencies including learning dis-
80
p< 0.05 (Mann-Whitney) CHD patients
Fig. 5 Median of ranking of Quality of Life in patients with CHD compared to healthy controls: 29 patients ≥ 15 years. High value indicates negative ranking.
a
15 years 40.00 37.50
50
43.75 40.00 45.00
aut
60
0
10
20
% Median
30 % Median
p< 0.05 (Mann-Whitney)
female
male
Heusch A et al. Health Related Quality of … Klin Padiatr 2014; 226: 281–286
40
50
60
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fri
40.00
fri fam
37.50
phys
37.50 37.50
50.00
p< 0.01
43.75
45.00
cog
50.00 30.00
bod
p=0.007
37.50 37.50
emo
43.75
p< 0.01
45.00 45.83
aut 0
20
40 % Median Parent‘s rating
60 80 p< 0.05 (Mann-Whitney) Selfrating
Fig. 7 Median of ranking of Quality of Life in 167 CHD parents and their children.
abilities, behavioural abnormalities, attention deficits and hyperactivity [38, 39]. Children with cyanotic cardiac defects seem to be at higher risk for dysfunction in speech and language [12]. The reduction of cardio respiratory capacity or mental abilities may impair the patients’ quality of life. On the other hand, quality of life is influenced by several other factors that relate to subjective impressions of individual patients and their parents. Several reports have been published recently that touch upon these issues, but research concept and measurement of HRQoL is questionable in most of them [23]. In our study, we used a standardized questionnaire, which was developed for school children and adolescents aged 8-18 years, who suffer from chronic disease [3]. The questionnaire deals with different aspects of daily life. As the questionnaire was handed out to patients and controls during a regular visit at their outpatient clinics located all over Germany, the sample was drawn from multiple centres and any direct influence of a single treating centre was eliminated. Furthermore, the questionnaire was not specifically designed for children with cardiac disease as the “Pediatric Cardiac Quality of Life Inventory” [19], and therefore, allows comparison to other chronic diseases. Recently, a new questionnaire for pre-school children was developed, now allowing measurement of the HRQoL in children 3-7 years [29]. In addition to validated instruments, multicentre studies are essential [33]. Our data demonstrate that patients after corrective surgery for congenital heart disease generally rate their HRQoL better than the control group of healthy children and adolescents. There are many studies with different research design dealing with HRQoL in patients with CHD during the last years. However, the results are ambiguous. In a large study with 404 adults, Moons also reported a higher self-rating of quality of life in the patients group [24]. A population-based study in Finland showed a high quality of life and high level of education even in patients who were operated in early periods of cardiac surgery [32]. A multicentre nationwide German study showed positive rankings after repair of Tetralogy of Fallot, even though objective exercise capacity was reduced [25]. Other studies show no difference in self-assessment if school and physical performance were reduced [13]. Costello et al. found normal mean psychological scores, even in young cardiac patients after membrane oxygenation (ECMO) [7]. According to
the positive self-assessment of HRQoL, Menteer et al. found no increased incidence of mood disorders in paediatric patients with heart failure and after transplantation [19]. Berghammer et. al reported on a small group of young adults with congenital heart disease who experienced ambivalence in viewing themselves in different domains of HRQoL [2]. Lower quality of life after surgery for CHD was reported in other studies [15, 35], especially in adults [18] but also in younger children [10]. Spijkerboer et al. showed lower scores for motor, cognitive, and emotional functioning in a cohort similar to our patients that were compared to healthy controls [34]. Other studies in paediatric patients with non-cardiac chronic disease (childhood cancer, diabetic patient), similar to our findings, reported better or at least equal ratings for the patients in comparison to the controls [4, 28]. However, these positive ratings of patients after surgery for CHD on all tested subscales tend to decrease with increasing age. Our findings, as well as other data [28], underline that quality of life is a dynamic process. We cannot explain, whether this is a consequence of changing hemodynamic, negative experiences or a more realistic view of life. In an interview study with adult patients between 25 and 40 years, Claessens described patients’ difficulties with their feeling of “being different” [6]. A Taiwanese study found significantly worse rankings of female adults, whereas men’s ranking was equal to normal. In a cohort of German adults after surgery for CHD, Rose pointed out that especially psychological factors lead to diminished quality of life in higher age [31]. This fact emphasizes that psychological support and efforts to enable normal social integration are very important for these patients. These efforts should start timely to prevent problems during early adolescence. They enable the patients to build up coping structures, which allow normal social and professional life in adulthood [37]. Besides personal peculiarity, Health related Quality is affected by many social factors. The socio-economic status of the families, especially the family’s income, has a great impact as shown by Cassedy et al. [5]. Also the parental education, the occupational status, and the different medical and social services as well as national peculiarities are important factors. The different populations and the various instruments used for measurement of HRQoL could be one reason for the contrary results reported in various papers. Parents rated their children’s HRQoL worse, especially in emotional and social domains; on the other hand, the voting for “physical capacity“ and “cognitive abilities“ were homogenous. Similarly Goldbeck, Lambert and Eagleson confirmed a limited parent-child agreement which predominately reflects different subjective viewpoints [11, 17]. Stress of parents during education of children with CHD is well known [35], and this fact may contribute to the worse ranking by parents in our cohort. The different point of parent’s view has to be considered in questionnaire dealing with HRQoL of younger infants. The coping mechanism is very similar in patients with CHD and reference groups [36]. Moons explains this phenomenon with a stronger sense of coherence of the patients. He postulates that this could be an important target for intervention to improve patients’ quality of life [22].
Conclusion Children with congenital heart defects are able to develop coping structures during their childhood and adolescence that enable them to live a normal life from their individual point of view. Heusch A et al. Health Related Quality of … Klin Padiatr 2014; 226: 281–286
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Original Article 285
Integration in psychosocial structures seems to be rather normal when compared to healthy controls. Patients considered their HRQoL even better than the controls. This observation is in line with other studies in groups of children with chronic disease and children with cancer. Psychosocial support is needed to link the different views of children and their parents’views regarding the HRQoL and to avoid negative family interactions. Accompanying family-centred programs can help to improve HRQoL, as demonstrated using the example of a neonatal intensive care unit [14]. Such support would also facilitate a normal development of care and emotional structures as well as autonomy in patients after surgery for CHD.
Contributor’s Statement
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Initiation and planning of the study, check of the returning questionnaires, selection and interpretation of the data, main writer: A. Heusch Planning of the study and the questionnaires, statistical work, selection and interpretation of the data, review of the manuscript: G. Calaminus Initiation and planning of the study, coordination with the pediatric surgeries: J. Kahl Planning of the study, mentoring during all phases, review of the manuscript: K. Schmidt
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286 Original Article
Health Related Quality of life after Corrective Surgery for Congenital Heart Disease Lebensqualität nach operativer Korrektur eines angeborenen Herzfehlers
Affiliations
Key words ▶ health related quality ● ▶ heart defect ● ▶ children ● ▶ congenital ● ▶ parents ● Schlüsselwörter ▶ Lebensqualität ● ▶ Herzfehler ● ▶ Kinder ● ▶ angeboren ● ▶ Eltern ●
Bibliography DOI http://dx.doi.org/ 10.1055/s-0034-1382019 Published online: July 25, 2014 Klin Padiatr 2014; 226: 281–286 © Georg Thieme Verlag KG Stuttgart · New York ISSN 0300-8630 Correspondence Dr. Andreas Heusch Abteilung Pädiatrische Kardiologie/Pulmonologie Helios-Klinikum Wuppertal Heusnerstrasse 40 42238 Wuppertal Germany Tel.: + 49/202/8963 835 Fax: + 49/202/8963 839 andreas.heusch@ helios-kliniken.de
A. Heusch1, G. Calaminus2, J. Kahl3, K. Schmidt4 1
Abteilung Paediatrische Kardiologie/Pulmonologie, Helios-Klinikum Wuppertal, Wuppertal Pediatric Oncology, University Muenster, Muenster, Germany 3 Kinderkardiologische Praxis, Uhlandstrasse, Duesseldorf, Germany 4 Pediatric Cardiology, University Duesseldorf, Duesseldorf, Germany 2
Abstract
Zusammenfassung
After corrective surgery for congenital heart defects (CHD) many patients suffer from residual defects, some with reduced cardiorespiratory capacity and possible impairment of their health related quality of life (HRQoL). The aim of our study is to evaluate, how children after surgery for CHD rate their HRQoL. Method: A standardised questionnaire developed for self-rating in children between 8 and 18 years and dealing with 7 different domains concerning the HRQoL (friends, family, physical functioning, cognition, body image, emotional function and autonomy) was independently answered from patients and their parents during an outpatient visit at their pediatric cardiologist throughout Germany. Results: 173 patients (40 % female, 60 % male, mean age 11.6 years) were interviewed, 167 questionnaires could be evaluated. The mean time interval after surgery was 9.8 ± 3.4 years. Patient had had surgery for complete different types of CHD (ventricular septal defects n = 50, Tetralogy of Fallot n = 51, univentricular heart n = 26, transposition of great arteries n = 40). The results were compared with those of an agematched control group (n = 169). Patients with CHD reported a better HRQoL than the controls for all items (p < 0.01). There was no significant gender specific difference. After puberty, the rating for most items, except of “body image“, had become very similar compared to controls. Parents assessed their children significantly worse in 3 domains (friends, body image and emotion; p < 0.01). Conclusion: Children with congenital heart defects are able to develop coping structures, that enable them to live a normal life from their individual point of view. Integration in psychosocial structures seems to be rather normal when compared to healthy controls. Many patients considered their HRQoL as even better.
Nach operativer Korrektur eines angeborenen Herzfehlers (CHD) bestehen bei vielen Patienten Restdefekte. Bei einigen gehen diese mit einer verminderten kardiorespiratorischen Leistungsfähigkeit und einer möglichen Einschränkung der gesundheitsbezogenen Lebensqualität (HRQoL) einher. Ziel unserer Studie war es, die Selbsteinschätzung der HRQoL bei Kindern nach Korrektur eines angeborenen Herzfehlers zu evaluieren. Methode: Ein Fragebogen, der für die Selbsteinschätzung von Kinder und Jugendlichen von 8–18 Jahren entwickelt wurde und sich mit 7 unterschiedlichen Bereichen der HRQoL befasst (Freunde, Familie, körperliche Fähigkeit, kognitive Leistung, Körperbild, emotionale Fähigkeit und Selbstständigkeit), wurde von Patienten und Eltern unabhängig von einander bei einem Besuch ihres niedergelassenen Kinderkardiologen deutschlandweit beantwortet. Ergebnisse: 173 Patienten ( 40% männlich, 60% weiblich, mittleres Alter 11,6 Jahre) wurden befragt, 167 Fragebogen konnten ausgewertet werden. Der mittlere Abstand zur Operation betrug 9,8 ± 3,4 Jahre. Bei den Patienten waren die unterschiedlichsten Herzfehler korrigiert worden (Ventrikelseptumdefekt n = 50, Fallot‘sche Tetralogie n = 51, Univentrikuläres Herz n = 26, Transposition der großen Gefäße n = 40): Die Ergebnisse wurde mit denen einer Kontrollgruppe vergleichbaren Alters (n=169) verglichen. Patienten mit angeborenem Herzfehler beschrieben in allen Bereichen eine bessere HRQoL als die Kontrollpersonen (p < 0,01). Es bestanden keine geschlechtsspezifischen Unterschiede. Nach der Pubertät wurden die meisten Bereiche mit Ausnahme des Körperbildes ähnlich wie bei Gesunden eingeschätzt. Die elterliche Einschätzung war in 3 Bereichen signifikant schlechter als die der Patienten (Freunde, Körperbild, emotionale Fähigkeit, p < 0,01).
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Heusch A et al. Health Related Quality of … Klin Padiatr 2014; 226: 281–286
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Authors
282 Original Article Zusammenfassung: Kinder mit angeborenem Herzfehler entwickeln Verarbeitungsstrategien, die Ihnen aus eigener Sicht ein normales Leben erlauben. Verglichen mit gesunden Kindern, scheint die Integration in psychosoziale Strukturen weitgehend normal. Viel Patienten schätzen ihre HRQoL sogar besser ein.
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CHD HRQoL VSD ToF TGA fri fam phys cog bod emo aut
Congenital heart defects Health Related Quality of live Ventricular septal defect Tetralogy of Fallot Transposition of the great arteries Relations to friends Interactions in the families’ Physical capacity Cognitive abilities Patient’s body image Emotional function Aspects of autonomy
Introduction
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During the last decades, optimised surgical techniques as well as improved diagnostic tools and medical treatment have led to an increasing number of long-time survivors after corrective surgery for congenital heart defects (CHD) in childhood. Many patients, however, suffer from residual defects, some with severe impairment of the hemodynamic functional capacity. Several studies have reported a high prevalence of complications in different organ systems [20] or reduced cardio respiratory capacity during follow-up studies [8, 30]. Health related quality of life (HRQoL), which is a subjective selfassessment of the individual well-being, is not only determined by objective data such as functional or laboratory tests. HRQoL is highly affected by the ability of patients and their families to cope with the problems caused by the disease. Therefore, HRQoL is influenced by different aspects which are determined by individual and objective variables [9]. In the present study, we aimed to evaluate how schoolchildren and adolescents rate their HRQoL concerning the most important aspects of daily life after surgery for CHD. Furthermore, we also asked parents to rate their children’s HRQoL, enabling us to compare self- and proxy-reported values.
repair of complete transposition of the great arteries (TGA, atrial or arterial switch), or staged implementation of a Fontan-type circulation for univentricular AV-connection. Surgical procedures in these patients had been performed at different cardiothoracic units throughout Germany. Patients with additional handicaps or known syndromes were not included. Controls were matched according to age and sex from the sample of about 2 000 children and adolescents who had participated in the PedQol evaluation [3, 4]. PedQol evaluation interviews had been conducted at different types of German schools, participation had been voluntary. There were no further selection criteria.
Questionnaire The study centre distributed the questionnaires to the local paediatric cardiologist. After informed consent was obtained, the questionnaire was independently and anonymously answered by the patients and their parents. Younger children were assisted by a receptionist. Subsequently, questionnaires were returned to the study centre for further evaluation. In the first part, the questionnaire asked for demographic data as well as details of the CHD and the surgical procedure according to the classification of the Association for European Paediatric Cardiology [1]. In the second part, the questionnaire asked for HRQoL-items using PedQol, a standardised feedback form that was developed for self-rating in schoolchildren. An adapted form, implying the same questions on patients HRQoL was answered by the parents. PedQol had recently been used for assessment of HRQoL in children with chronic diseases [3, 26]. The PedQol-questions focus on 7 different domains: the relations of the patients to their friends (fri), the interactions in the affected families (fam), the physical capacity (phys), cognitive abilities (cog), the patient’s own body image (bod), their emotional function (emo), and aspects of autonomy (aut). Each domain is explored by a group of different indirect questions (6–12 for each item), and for each question, 1 of the 4 frequency ranks (never, rarely, often, and always) must be chosen as an answer. The patients’ rating was compared to that of healthy schoolchildren (control group), matched for age and gender and to that of their own parents.
Statistics Methods
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Patients and controls 173 Children and adolescents (7–18 years) with CHD after surgery were interviewed during a routine follow-up outpatient visit at their local paediatric cardiologist. 16 medical practices throughout Germany had agreed to recruit patients for the study. Data on response rates is not available; conversations with recruiting doctors suggest that selection was generally independent of patient characteristics and mainly depended on whether time was available. All patients, together with their parents, who agreed to the study and who had gone through one of the following cardiac surgeries were interviewed: repair of a ventricular septal defect (VSD), repair of Tetralogy of Fallot (ToF), Heusch A et al. Health Related Quality of … Klin Padiatr 2014; 226: 281–286
Statistical analysis was performed using commercial software programs (IBM SPSS statistics 18®). Rating of each PedQol domain is reported as mean, standard deviation, median, minimum, maximum and the interquartile area. Rating levels in each domain (never, rarely, often, always) were allocated to increasing negativity (1–4) and were related to a specific percentage range (25 %, 50 %, 75 %, 100 %). From these percentages, mean values for every domain and every patient were calculated and the mean for compared groups were determined. Differences between groups were analysed with the Mann-Whitney-Test or the Kruskal-Wallis-Test where appropriate. P-values < 0.05 were considered to indicate significant differences between groups; p-values < 0.1 were notable trends.
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List of abbreviation
Original Article 283
Comparison between patients and controls was done by a matched pair analysis.
answered the questionnaire as well as 48 of the 52 after correction of Tetralogy of Fallot, 38 of the 40 after TGA correction, and 25 of 26 after Fontan-type operation.
Results
Comparison of patients and controls
Patients Out of 173 patients interviewed, 167 questionnaires were returned completely and could be evaluated further. The mean age was 11.6 years; 101 were male (60 %) and 66 female (40 %) patients. 71 patients were younger than 11 years, 67 were between ▶ Fig. 1). For 11 and 14 years and 29 were older than 14 years (● each domain, percentages of ranking levels were compared with those of age-matched controls. 50 patients had a VSD that was closed surgically, 51 had Tetralogy of Fallot after correction, 26 patients had different types of univentricular AV-connection and a Fontan-type procedure, 40 had complete transposition of the great arteries after corrective surgery (28 arterial switch, 12 atrial switch). The mean time interval after surgery was 9.5 ± 3.1 years. The distribution of sex and age at interview was almost equal in the subgroups of different diagnoses with the exception of the TGA group where more male patients (78 %) were present.
Independently of gender, age and type of CHD, patients reported a significantly better HRQoL than controls for all items (p < 0.01), ▶ Fig. 2). especially for the domain “body image” (● When comparing female and male patients, there was no significant gender specific difference in the CHD group. There was a tendency for a more positive rating in the male group for the domains “cognitive abilities”, “body image” and “autonomy” ▶ Fig. 3). In contrast, the control subjects showed a more posi(● tive rating in the female group, which was significantly different in “emotional functioning”, “cognitive abilities”, “physical capacity” and “body image”. CHD-patients had a tendency to rank most items except for “friends” and “autonomy” more negatively with increased age. Especially patients older than 15 years rated the domains “family” (p = 0.043), “physical capacity”, “cognitive abilities” (p < 0.001), and “emotional functioning” (p = 0.036) less positive than younger children. Only the domain “autonomy” was rated ▶ Fig. 4). less negatively by these older CHD-patients (p = 0.014) (●
Parents During the same outpatient visit, 161 parents were interviewed independently; all 50 parents of children after VSD closure
prepubertal (≤ 10 years)
♂ ♀
%
n
16 14 12 10 8 6 4 2 0
fam
37.50 37.50
phys
37.50 37.50
pubertal (11–≤ 14 years)
postpubertal (≥ 15 years)
cog
42
39
20
bod
29
28
9
45.00 43.75 35.00 30.00 37.50 37.50
emo
45.83 41.67
aut 0
20
40 % Median female
7
8
9
10
11
12
13
14
15
16
17
fam
37.50
phys
37.50
43.75
P= 0.17
43.75
P=0.025
bod
aut
40 % Median
Healthy control
bod
p< 0.001
emo
p= 0.036
aut
p= 0.014
P < 0.01 P=0.005
45.00
20
p= 0.043
cog
40.00 37.50
emo
fam
P< 0.01
50.00
30.00
male
phys
50.00 45.00
cog
80
fri
43.75 40.00
fri
60
Fig. 3 Median of ranking of Quality of Life in patients with CHD. Comparison of 99 male vs. 67 female patients. High value indicates negative ranking (absent values are due to missing ranking).
18
Fig. 1 Demographic data and age distribution of the patient group.
0
40.00 40.00
fri
55.00
60
P < 0.01
0
20
80
CHD patients
Fig. 2 Median of ranking of Quality of Life in patients with CHD compared to healthy controls. High value indicates negative ranking. Statistical significance is given with p-values.
≥15 years
40 % Median 11–≤14 years
60
80
P< 0.05 (Kruskall-Wallis) ≤10 years
Fig. 4 Median of ranking of Quality of Life in 167 patients with CHD according to the age groups. High value indicates negative ranking (absent values are due to missing ranking).
Heusch A et al. Health Related Quality of … Klin Padiatr 2014; 226: 281–286
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284 Original Article
years). In the controls, especially “emotional function” in females and “autonomy” in males were rated worse. In the older patients ( > 15 years), females after puberty rated the domains “autonomy”, “cognitive abilities”, and “family” worse than males, while in the controls females rated “body image” significantly ▶ Fig. 6a, b). worse than patients and healthy males. (●
In CHD-patients after puberty, the rating for most items had become very similar compared to controls. In particular, the domain “body image” was rated higher after puberty by all CHD▶ Fig. 5). patients than by the controls (p < 0.01) (● Looking at the gender specific differences in the various age groups, there were no differences in the younger patients ( < 10
Parents rating When comparing the rating of parents and their affected children, parents rated worse in all domains except “physical capacity”. The difference was significant in the 3 domains “friends”, ▶ Fig. 7). In the patients with “body image”, and “emotion” (● Fontan-circulation and after Fallot-repair, the largest differences occurred between the ratings of patients and those of their parents.
40.00 40.00
fam
43.75
phys
43.75 43.75
50.00
50.00 50.00
cog
60.00
bod
P< 0.01
35.00
Discussion
45.00 43.75
emo
▼
55.00
aut
45.83
20
0
40 % Median
Healthy control
60
The main intention of cardiac surgery in patients with congenital heart defects is to allow long-time survival and to improve the quality of life. After corrective surgery for congenital heart defects, however, many patients may experience functional residual deficits especially those with complex defects [27]. In some of these patients residual deficits may lead to reduced cardio respiratory capacity [30]. Furthermore, the neurological outcome of children after surgery for heart defects may be affected; some children show various deficiencies including learning dis-
80
p< 0.05 (Mann-Whitney) CHD patients
Fig. 5 Median of ranking of Quality of Life in patients with CHD compared to healthy controls: 29 patients ≥ 15 years. High value indicates negative ranking.
a
15 years 40.00 37.50
50
43.75 40.00 45.00
aut
60
0
10
20
% Median
30 % Median
p< 0.05 (Mann-Whitney)
female
male
Heusch A et al. Health Related Quality of … Klin Padiatr 2014; 226: 281–286
40
50
60
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fri
40.00
fri fam
37.50
phys
37.50 37.50
50.00
p< 0.01
43.75
45.00
cog
50.00 30.00
bod
p=0.007
37.50 37.50
emo
43.75
p< 0.01
45.00 45.83
aut 0
20
40 % Median Parent‘s rating
60 80 p< 0.05 (Mann-Whitney) Selfrating
Fig. 7 Median of ranking of Quality of Life in 167 CHD parents and their children.
abilities, behavioural abnormalities, attention deficits and hyperactivity [38, 39]. Children with cyanotic cardiac defects seem to be at higher risk for dysfunction in speech and language [12]. The reduction of cardio respiratory capacity or mental abilities may impair the patients’ quality of life. On the other hand, quality of life is influenced by several other factors that relate to subjective impressions of individual patients and their parents. Several reports have been published recently that touch upon these issues, but research concept and measurement of HRQoL is questionable in most of them [23]. In our study, we used a standardized questionnaire, which was developed for school children and adolescents aged 8-18 years, who suffer from chronic disease [3]. The questionnaire deals with different aspects of daily life. As the questionnaire was handed out to patients and controls during a regular visit at their outpatient clinics located all over Germany, the sample was drawn from multiple centres and any direct influence of a single treating centre was eliminated. Furthermore, the questionnaire was not specifically designed for children with cardiac disease as the “Pediatric Cardiac Quality of Life Inventory” [19], and therefore, allows comparison to other chronic diseases. Recently, a new questionnaire for pre-school children was developed, now allowing measurement of the HRQoL in children 3-7 years [29]. In addition to validated instruments, multicentre studies are essential [33]. Our data demonstrate that patients after corrective surgery for congenital heart disease generally rate their HRQoL better than the control group of healthy children and adolescents. There are many studies with different research design dealing with HRQoL in patients with CHD during the last years. However, the results are ambiguous. In a large study with 404 adults, Moons also reported a higher self-rating of quality of life in the patients group [24]. A population-based study in Finland showed a high quality of life and high level of education even in patients who were operated in early periods of cardiac surgery [32]. A multicentre nationwide German study showed positive rankings after repair of Tetralogy of Fallot, even though objective exercise capacity was reduced [25]. Other studies show no difference in self-assessment if school and physical performance were reduced [13]. Costello et al. found normal mean psychological scores, even in young cardiac patients after membrane oxygenation (ECMO) [7]. According to
the positive self-assessment of HRQoL, Menteer et al. found no increased incidence of mood disorders in paediatric patients with heart failure and after transplantation [19]. Berghammer et. al reported on a small group of young adults with congenital heart disease who experienced ambivalence in viewing themselves in different domains of HRQoL [2]. Lower quality of life after surgery for CHD was reported in other studies [15, 35], especially in adults [18] but also in younger children [10]. Spijkerboer et al. showed lower scores for motor, cognitive, and emotional functioning in a cohort similar to our patients that were compared to healthy controls [34]. Other studies in paediatric patients with non-cardiac chronic disease (childhood cancer, diabetic patient), similar to our findings, reported better or at least equal ratings for the patients in comparison to the controls [4, 28]. However, these positive ratings of patients after surgery for CHD on all tested subscales tend to decrease with increasing age. Our findings, as well as other data [28], underline that quality of life is a dynamic process. We cannot explain, whether this is a consequence of changing hemodynamic, negative experiences or a more realistic view of life. In an interview study with adult patients between 25 and 40 years, Claessens described patients’ difficulties with their feeling of “being different” [6]. A Taiwanese study found significantly worse rankings of female adults, whereas men’s ranking was equal to normal. In a cohort of German adults after surgery for CHD, Rose pointed out that especially psychological factors lead to diminished quality of life in higher age [31]. This fact emphasizes that psychological support and efforts to enable normal social integration are very important for these patients. These efforts should start timely to prevent problems during early adolescence. They enable the patients to build up coping structures, which allow normal social and professional life in adulthood [37]. Besides personal peculiarity, Health related Quality is affected by many social factors. The socio-economic status of the families, especially the family’s income, has a great impact as shown by Cassedy et al. [5]. Also the parental education, the occupational status, and the different medical and social services as well as national peculiarities are important factors. The different populations and the various instruments used for measurement of HRQoL could be one reason for the contrary results reported in various papers. Parents rated their children’s HRQoL worse, especially in emotional and social domains; on the other hand, the voting for “physical capacity“ and “cognitive abilities“ were homogenous. Similarly Goldbeck, Lambert and Eagleson confirmed a limited parent-child agreement which predominately reflects different subjective viewpoints [11, 17]. Stress of parents during education of children with CHD is well known [35], and this fact may contribute to the worse ranking by parents in our cohort. The different point of parent’s view has to be considered in questionnaire dealing with HRQoL of younger infants. The coping mechanism is very similar in patients with CHD and reference groups [36]. Moons explains this phenomenon with a stronger sense of coherence of the patients. He postulates that this could be an important target for intervention to improve patients’ quality of life [22].
Conclusion Children with congenital heart defects are able to develop coping structures during their childhood and adolescence that enable them to live a normal life from their individual point of view. Heusch A et al. Health Related Quality of … Klin Padiatr 2014; 226: 281–286
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Integration in psychosocial structures seems to be rather normal when compared to healthy controls. Patients considered their HRQoL even better than the controls. This observation is in line with other studies in groups of children with chronic disease and children with cancer. Psychosocial support is needed to link the different views of children and their parents’views regarding the HRQoL and to avoid negative family interactions. Accompanying family-centred programs can help to improve HRQoL, as demonstrated using the example of a neonatal intensive care unit [14]. Such support would also facilitate a normal development of care and emotional structures as well as autonomy in patients after surgery for CHD.
Contributor’s Statement
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Initiation and planning of the study, check of the returning questionnaires, selection and interpretation of the data, main writer: A. Heusch Planning of the study and the questionnaires, statistical work, selection and interpretation of the data, review of the manuscript: G. Calaminus Initiation and planning of the study, coordination with the pediatric surgeries: J. Kahl Planning of the study, mentoring during all phases, review of the manuscript: K. Schmidt
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