Health-Related Quality of Life Outcomes in Children and Adolescents with Congenital Heart Disease Katelyn Mellion, MS1, Karen Uzark, PhD1, Amy Cassedy, PhD2, Dennis Drotar, PhD3, Gil Wernovsky, MD4, Jane W. Newburger, MD, MPH5, Lynn Mahony, MD6, Kathy Mussatto, RN, PhD7, Mitchell Cohen, MD8, Christine Limbers, MS9, and Bradley S. Marino, MD, MPP, MSCE1,10, for the Pediatric Cardiac Quality of Life Inventory Testing Study Consortium* Objectives To compare health-related quality of life (HRQOL) in a group of pediatric patients with congenital heart disease (CHD) and healthy controls and patients with other chronic diseases, and to compare HRQOL among patients with CHD of various severity categories with one another, with controls, and with patients with other chronic diseases. Study design In this cross-sectional survey, t tests were used to compare patient and proxy-reported Pediatric Quality of Life Inventory 4.0 Generic Core Scales (PedsQL) scores (including total, physical health, and psychosocial health summary scores) in children (aged 8-12 years) and adolescents (aged 13-18 years) between controls and (1) a composite CHD population; and (2) patients in each of 3 CHD severity categories: mild (no intervention), biventricle (BV; postintervention), and single ventricle (SV; postpalliation). PedsQL scores among CHD severity categories were compared by ANOVA. PedsQL scores were also compared in the CHD population and children with other chronic diseases without age stratification using t tests. Results There were 1138 (children, n = 625; adolescents, n = 513) and 771 (children, n = 528; adolescents, n = 243) patient and/or proxy reporters in the CHD and healthy control groups, respectively. Total, physical health, and psychosocial health summary scores were lower in the composite CHD, BV, and SV groups compared with controls (P < .0001). There were significant differences among disease severity categories for all scores (P < .01). The composite CHD, BV, and SV groups had similar PedsQL scores as end-stage renal disease, asthma, and obesity populations. Conclusion Children and adolescents with BV and SV CHD have significantly lower HRQOL than healthy controls and similar HRQOL as patients with other chronic pediatric diseases. Interventions targeting both physical and psychosocial domains are needed to improve HRQOL in this high-risk population. (J Pediatr 2014;164:781-8).
H
ealth-related quality of life (HRQOL) is defined as the impact of a specific illness, medical therapy, or health services policy on a patient’s ability to function in various life contexts and draw personal satisfaction from physical, psychological, and social functioning (SoF) perspectives.1 The Pediatric Quality of Life Inventory 4.0 Generic Core Scales (PedsQL) is a reliable, valid generic HRQOL measurement tool that is widely used in pediatric populations to determine HRQOL in patients with chronic diseases and healthy controls.2 The PedsQL can be used to characterize the impact of therapies and disease processes on the HRQOL of children and adolescents with congenital heart disease (CHD) and to compare this impact with that in children and adolescents with other chronic diseases. Given the cognitive and emotional differences between children and adolescents3 and differences in functioning among patients with heart disease (HD) of varying severity,4-12 individuals’ HRQOL may be affected differently, depending on age and disease severity. The effects of these factors on the HRQOL of the pediatric CHD population have not been delineated in a large, geographically
BV CHD EmF ESRD HD HRQOL PCQLI
Biventricle Congenital heart disease Emotional functioning End-stage renal disease Heart disease Health-related quality of life Pediatric Cardiac Quality of Life Inventory
PedsQL PhysHSS PsychHSS ScF SoF SV
Pediatric Quality of Life Inventory 4.0 Generic Core Scales Physical health summary score Psychosocial health summary score School functioning Social functioning Single ventricle
From the 1Division of Cardiology, Department of Pediatrics, 2Department of Biostatistics and Epidemiology, and 3Division of Behavioral and Clinical Psychology, Department of Pediatrics, Cincinnati Children’s Hospital Medical Center, Cincinnati, OH; 4 Division of Cardiology, Department of Pediatrics, The Children’s Hospital of Philadelphia, Philadelphia, PA; 5 Division of Cardiology, Department of Pediatrics, Children’s Hospital Boston, Boston, MA; 6Division of Cardiology, Department of Pediatrics, University of Texas Southwestern Medical Center Dallas, Dallas, TX; 7 Division of Cardiology, Department of Pediatrics, Children’s Hospital of Wisconsin, Milwaukee, WI; 8 Department of Cardiology, Phoenix Children’s Hospital, Phoenix, AZ; 9Department of Psychology and Neuroscience, Baylor University, Waco, TX; and 10 Division of Critical Care Medicine, Department of Pediatrics, Cincinnati Children’s Hospital Medical Center, Cincinnati, OH *A list of Pediatric Cardiac Quality of Life Inventory Testing Study Consortium sites is available at www. jpeds.com (Appendix). Supported by the National Institute of Child Health and Human Development (5-K23-HD048637), the American Heart Association, Pennsylvania/Delaware Affiliate (now Great Rivers Affiliate; beginning Grant-in-Aid 0465467U), the Cincinnati Children’s Hospital Research Foundation, and the Children’s Hospital of Philadelphia Institutional Development Fund. The authors declare no conflicts of interest. Portions of the study were presented as a poster at the American Academy of Pediatrics’ National Conference and Exhibition, San Francisco, CA, October 2-5, 2010. 0022-3476/$ - see front matter. Copyright ª 2014 Mosby Inc. All rights reserved. http://dx.doi.org/10.1016/j.jpeds.2013.11.066
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diverse cohort, however. Two previous studies13,14 assessed HRQOL in pediatric patients with HD; however, relatively small HD samples were used, and findings were mixed. In the present study, we aimed to use a geographically diverse cohort to compare patient and proxy-reported PedsQL scores in children (aged 8-12 years) and adolescents (aged 13-18 years) with CHD with those of sex- and racematched healthy controls. Within each age group, we also compared PedsQL scores among patients with CHD of various severity categories with one another and with controls to determine which severity categories within each age group might benefit from interventions targeting specific HRQOL domains. In addition, PedsQL scores among patients with CHD of various severity categories were compared to those of patients with other chronic diseases. We hypothesized that children and adolescents with CHD would have lower HRQOL than healthy controls, that HRQOL would not be significantly different between children and adolescents with mild CHD and healthy controls, and that children and adolescents with more complex CHD would have significant deficits in each HRQOL domain (total; physical health; psychosocial health; emotional functioning [EmF], SoF, and school functioning [ScF]) compared with healthy controls and patients with less complex disease. We also hypothesized that pediatric patients with complex CHD would have a similar HRQOL as those with other chronic diseases, and that pediatric patients with mild CHD would have a better HRQOL compared with those with other chronic diseases.
Methods This study was a cross-sectional survey that used patient data originally collected to test the psychometric properties of the Pediatric Cardiac Quality of Life Inventory (PCQLI). The Institutional Review Board of Cincinnati Children’s Hospital Medical Center approved this study and granted a waiver of consent based on its retrospective nature. The PedsQL data used for the CHD group in this study was provided by patients with CHD and their parents to establish construct validity during the PCQLI validation study.15-17 Patients from 7 large US pediatric cardiac centers (Appendix) contributed to the CHD patient population between November 2004 and December 2008. Eligibility criteria for participation in the PCQLI validation study included CHD or acquired HD, age 8-18 years, fluent in English, and attending an outpatient cardiology visit. Patients who had a significant comorbid medical condition or major developmental delay or who came to the clinic because of an acute change in clinical status were excluded. Proxy reporters were excluded if they were not fluent in English or had a major developmental delay. From the 1605 patient–parent pairs who were included in the PCQLI validation study, those patients with CHD who also had a valid self-reported or proxy-reported PedsQL score were included in the present study. The healthy population used in this study was obtained from the PedsQL healthy 782
Vol. 164, No. 4 children database.2,18 These normative data were obtained from the previously conducted PedsQL initial field test and a statewide State Children’s Health Insurance Program evaluation in California. The PedsQL data for the chronic disease populations were obtained from a previously published database.14 For the PCQLI validation study, patients and proxy reporters were given the PedsQL before a routine outpatient visit. Research personnel obtained patient and parent demographic information and patient clinical information (diagnostic, surgical, and medical history) for the CHD population through parent report and chart review. The original diagnostic category was divided into 2 subgroups: biventricle (BV) CHD and single ventricle (SV) CHD. CHD severity categories included mild CHD (patients with CHD who had not undergone surgical or catheter-based intervention), BV CHD (patients with CHD with BV physiology who had undergone surgical or catheter-based intervention), and SV CHD (patients with CHD with SV physiology who had undergone surgical palliation). The number of cardiac surgeries (defined as sternotomy or thoracotomy) and number of cardiac catheterizations were reported for the BV CHD and SV CHD groups only. The time since the most recent hospitalization was reported for all CHD disease severity categories. For the healthy control population, patient sex and race data for the child and adolescent groups were obtained from the PedsQL healthy children database. The PedsQL, developed by Varni et al, was used to assess HRQOL and has well-documented reliability and validity in the pediatric HD and healthy populations.2,13,14,18 The PedsQL includes 23 items that evaluate physical functioning and psychosocial functioning. The physical health summary score (PhysHSS) includes physical functioning, and the psychosocial health summary score (PsychHSS) includes EmF, SoF, and ScF. The total score incorporates all of these domains. There are separate forms for children aged 8-12 years and adolescents aged 13-18 years as well as their proxy reporters; the content of the items in each form are the same except for appropriate changes to pronouns and language modifications to accommodate developmental differences. All answers are based on a 5-point Likert scale (0, not a problem; 1, almost never a problem; 2, sometimes a problem; 3, often a problem; 4, almost always a problem). Items are reverse-scored and linearly transformed to a scale of 0-100, with higher transformed scores indicating a better HRQOL. The number of cardiac surgeries, number of cardiac catheterizations, and time since the most recent hospitalization were compared in the BV CHD and SV CHD groups using the Wilcoxon rank-sum test. Patient and proxy-reported PedsQL scores (total score, PhysHSS, PsychHSS, EmF, SoF, and ScF) were compared between the composite pediatric CHD group and sex- and race-matched healthy controls in 2 age groups (children [age 8-12 years] and adolescents [age 13-18 years]) and between patients in each of 3 CHD severity categories and healthy controls in both age groups using t tests. Patient and proxy-reported PedsQL scores (total score, PhysHSS, PsychHSS, EmF, SoF, and ScF) were Mellion et al
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Table I. Characteristics of patients with CHD Composite CHD Child
Adolescent
Mild CHD Child
BV CHD
Adolescent
Child
Adolescent
SV CHD Child
Adolescent
Characteristics
(n = 625)
(n = 513) (n = 107; 17%) (n = 97; 19%) (n = 386; 62%) (n =340; 66%) (n = 132; 21%) (n = 76; 15%)
Patient sex male, n (%) Parent sex male, n (%) Patient ethnicity Hispanic, n (%) Parent ethnicity Hispanic, n (%) Patient race, n (%) White African American Other Parent race, n (%) White African American Other Patient age, y, mean SD Parent age, y, mean SD Number of cardiac surgeries, mean SD Number of cardiac catheterizations, mean SD Years since last hospitalization, mean SD
338 (54.1) 85 (13.6) 42 (6.7) 35 (5.6)
301 (58.7) 84 (16.4) 30 (5.8) 25 (4.9)
57 (53.3) 14 (13.1) 13 (12.1) 14 (13.1)
63 (64.9) 12 (12.4) 7 (7.2) 6 (6.2)
202 (52.3) 58 (15.0) 19 (4.9) 12 (3.1)
194 (57.1) 61 (17.9) 18 (5.3) 15 (4.4)
79 (59.8) 13 (9.8) 10 (7.6) 9 (6.8)
44 (57.9) 11 (14.5) 5 (6.6) 4 (5.3)
510 (81.6) 49 (7.8) 66 (10.6)
431 (84.0) 42 (8.2) 40 (7.8)
80 (74.8) 7 (6.5) 20 (18.7)
82 (84.5) 9 (9.3) 6 (6.2)
318 (82.4) 31 (8.0) 37 (9.6)
276 (81.2) 33 (9.7) 31 (9.1)
112 (84.8) 11 (8.3) 9 (6.8)
73 (96.1) 0 (0.0) 3 (3.9)
522 (83.5) 48 (7.7) 55 (8.8) 9.8 1.4 40.8 6.5 1.5 1.4
440 (85.8) 41 (8.0) 32 (6.2) 15.1 1.6 45.1 6.2 1.6 1.6
79 (73.8) 7 (6.5) 21 (19.6) 9.8 1.4 40.5 6.7 NA
80 (82.5) 10 (10.3) 7 (7.2) 15.3 1.6 45.5 6.3 NA
330 (85.5) 31 (8.0) 25 (6.5) 9.8 1.5 40.9 6.3 1.3 1.0
285 (83.8) 31 (9.1) 24 (7.1) 15.2 1.7 45.3 6.2 1.6 1.2
113 (85.6) 10 (7.6) 10 (7.6) 9.6 1.4 40.5 6.8 3.2 0.9
75 (98.7) 0 (0.0) 1 (1.3) 14.8 1.6 43.7 5.6 3.7 1.6
1.6 2.0
1.9 2.2
NA
NA
1.4 1.9
1.8 2.1
3.4 1.8
4.2 2.1
5.2 3.6
6.9 5.6
7.0 3.4
9.6 5.6
5.5 3.7
6.8 5.7
3.8 2.6
5.9 4.3
NA, not applicable.
compared between the composite pediatric CHD population and each of 4 chronic disease groups (end-stage renal disease [ESRD], obesity, asthma, and diabetes), as well as between patients in each of the 3 CHD severity categories and each of the chronic disease groups using t tests. Age group comparisons could not be performed, however, because chronic disease data were not separated into child and adolescent age groups. After Bonferroni correction to adjust for multiple comparisons, P < .00017 was considered statistically significant for all t tests. In addition, patient and proxy-reported PedsQL scores (total score, PhysHSS, PsychHSS, EmF, SoF, and ScF) were compared among the 3 CHD severity categories in both the child and adolescent age groups using 1-way ANOVA with the Tukey post hoc test.
Results PedsQL scores were available for 1138 patient and/or proxy reporters with CHD (Table I). The CHD child age group comprised 625 patient and/or proxy reporters, 54.1% of whom were male and 81.6% of whom were Caucasian. The CHD adolescent age group comprised 513 patient and/or proxy reporters, 58.7% of whom were male and 84.0% of whom were Caucasian. The sex- and race-matched healthy control group included 771 patient and/or proxy reporters. The healthy child age group had 528 patient/parent respondents, 48.3% of whom were male and 84.7% of whom were Caucasian. The healthy adolescent age group had 243 respondents, 58.4% of whom were male and 87.7% of whom were Caucasian. The original diagnostic categories and specific diagnoses for the CHD population are provided in Table II. Patients in the SV CHD group had a greater number of cardiac surgeries (children, 3.2 vs 1.3, P < .0001; adolescents, 3.7 vs
1.6, P < .0001) and cardiac catheterizations (children, 3.4 vs 1.4, P < .0001; adolescents, 4.2 vs 1.8, P < .0001) compared with those in the BV CHD group. There was a significant difference in the time from most recent hospitalization between the SV CHD and BV CHD groups in the child age group (3.8 vs 5.5; P < .0001), but not in the adolescent age group (5.9 vs 6.8, P = .3037). Patient and proxy-reported PedsQL scores in both the child and adolescent composite CHD groups were significantly lower compared with healthy controls (P < .0001 for all comparisons; Table III). There were no significant differences between children and adolescents with mild CHD and healthy controls with the exception of childreported PhysHSS score (P < .0001). Children and adolescents with BV and SV CHD had lower scores in each HRQOL domain compared with healthy controls (P < .0001 for all comparisons), with the exception of adolescent-reported EmF score in the SV CHD group. Furthermore, children and adolescents with BV and SV CHD had lower scores in each HRQOL domain compared with patients with mild CHD, with the exception of EmF in the adolescent age group. For patient and proxyreported scores in both child and adolescent age groups, differences were seen among the disease severity categories in all HRQOL domains with the exception of adolescentreported EmF score (Table III). In those areas where the ANOVA revealed significant differences among groups, 2way comparisons between groups revealed that SV CHD groups had significantly lower scores compared with both the mild CHD and BV CHD groups in all HRQOL domains, with the exception of adolescent-reported ScF in the BV CHD group. However, in the adolescent age group there were no significant differences between the mild CHD and BV CHD groups in any patient or proxy-
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Table II. Primary diagnoses Diagnoses
Children (n = 625) Adolescents (n = 513) Total (n = 1138)
Original diagnostic category Biventricular CHD SV CHD Septal defects Atrial septal defect (patent foramen ovale, secundum, primum, or sinus venosus) Ventricular septal defect (single or multiple) Atrioventricular septal defect (complete or transitional) Aortopulmonary window Pulmonary venous anomalies Partial anomalous pulmonary venous connection Total anomalous pulmonary venous connection Right heart lesions Tetralogy of Fallot Tetralogy of Fallot with pulmonary atresia Tetralogy of Fallot with absent pulmonary valve Pulmonary atresia with intact ventricular septum Ebstein anomaly Tricuspid stenosis Pulmonary stenosis, valvar Pulmonary artery stenosis, branch Double-chambered right ventricle Other outflow-tract abnormalities in right ventricle Left heart lesions Aortic stenosis Aortic insufficiency Aortic insufficiency and aortic stenosis Mitral stenosis Mitral regurgitation Mitral valve prolapse (without moderate or greater mitral regurgitation) Bicuspid aortic valve (nonobstructive) Other outflow tract abnormalities in left ventricle SV Hypoplastic left heart syndrome Double-inlet left ventricle Double-inlet right ventricle Mitral atresia (not hypoplastic left heart syndrome of double-outlet right ventricle/mitral atresia) Tricuspid atresia Unbalanced atrioventricular canal Right ventricle-aorta with pulmonary atresia Superoinferior ventricle (with or without straddling tricuspid valve) Conotruncal anomalies Transposition of great arteries Double-outlet right ventricle Truncus arteriosus Thoracic arteries and veins Coronary artery anomaly Coarctation of aorta Interrupted aortic arch Patent ductus arteriosus Vascular rings and slings (with or without tracheal stenosis)
reported HRQOL score. In stark contrast, there was a significant difference between the mild CHD and BV CHD groups in all HRQOL domains for child-reported scores and in total, PsychHSS, and SoF scores for proxy reports in the child age group. Patients in the composite CHD group reported similar scores as patients with ESRD, asthma, obesity, and diabetes (Table IV). Proxy-reported PedsQL scores in the composite CHD group were similar to those in the ESRD, obesity, and diabetes groups but significantly higher than those in the asthma group. HRQOL of the mild CHD group was generally similar to that of the diabetes group but better than that of the ESRD and asthma groups according to both patient and proxy reports. HRQOL of 784
625 495 130 132 48 70 11 3 6 3 3 136 52 16 6 15 13 1 29 1 3 0 79 47 4 3 3 6 0 14 2 113 37 18 0 9 22 12 12 3 74 54 11 9 85 8 64 5 7 1
513 439 74 75 33 39 3 0 9 4 5 115 45 9 3 13 10 0 28 4 2 1 105 44 2 8 5 11 8 27 0 72 20 10 3 8 17 7 6 1 61 45 5 11 76 5 62 6 2 1
1138 934 204 207 81 109 14 3 15 7 8 251 97 25 9 28 23 1 57 5 5 1 184 91 6 11 8 17 8 41 2 185 57 28 3 17 39 19 18 4 135 99 16 20 161 13 126 11 9 2
the SV CHD population was generally similar to that of the ESRD, asthma, and obesity groups but worse than that of the diabetes group according to both patient and proxy reports. Interestingly, patient-reported EmF was similar across all comparisons.
Discussion This study is the first to characterize HRQOL in the pediatric CHD population by both age group and disease severity. PedsQL scores of both children and adolescents with mild CHD were not significantly different from those of healthy controls, with the exception of child-reported PhysHSS. Moreover, both children and adolescents with more complex Mellion et al
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Table III. PedsQL scores and comparisons PedsQL score Healthy Scale Child Self-report Total score PhysHSS PsychHSS EmF SoF ScF Proxy report Total score PhysHSS PsychHSS EmF SoF ScF Adolescent Self-report Total score PhysHSS PsychHSS EmF SoF ScF Proxy report Total score PhysHSS PsychHSS EmF SoF ScF
Composite CHD
Mild CHD1
BV CHD2
SV CHD3
Disease severity comparison
n
Mean ± SD
n
Mean ± SD
Among category, P value*
75.8 15.9z 99 81.3 14.1 77.9 17.6z 101 83.4 15.1z 74.7 16.7z 99 80.3 14.8 71.2 20.8z 101 76.4 19.0 79.6 19.8z 101 87.7 14.7 73.3 18.4z 99 76.8 17.2
371 373 371 372 372 371
76.6 15.8z 78.9 17.8z 75.3 16.5z 71.4 21.1z 80.2 19.6z 74.3 18.1z
123 124 123 124 124 123
68.8 15.3z 70.1 16.4z 68.2 16.9z 66.2 20.4z 71.1 20.9z 67.5 18.9z
3 1>2, 3; 2>3 1>3; 2>3 1>2, 3; 2>3 1>3; 2>3
625 625 625 625 625 625
74.7 16.7z 77.8 21.0z 73.1 16.8z 70.0 18.9z 77.4 20.5z 71.9 20.7z
107 107 107 107 107 107
82.7 13.6 85.9 17.7 81.0 14.7 76.0 17.8 87.0 16.9 80.0 18.9
386 386 386 386 386 386
75.1 16.6z 78.5 21.3z 73.2 16.5z 69.6 18.6z 78.1 20.3z 72.0 20.3z
132 132 132 132 132 132
67.3 16.2z 69.0 19.8z 66.4 16.6z 66.4 19.8z 67.7 19.8z 65.1 20.9z
2, 3; 2>3 1>2, 3; 2>3 1>2, 3; 2>3 1>2, 3; 2>3
86.9 11.8 90.2 12.4 85.1 13.5 83.7 17.0 90.2 15.2 81.2 16.2
499 500 499 500 500 499
79.0 15.4z 81.5 17.2z 77.7 16.2z 77.6 20.3z 83.1 17.6z 72.4 20.3z
94 94 94 94 94 94
82.9 15.1 84.9 15.6 81.9 15.9 80.7 19.1 87.8 16.0 77.1 19.5
333 333 333 333 333 333
79.3 14.8z 82.4 16.7z 77.6 15.6z 77.2 20.2z 83.4 16.7z 72.3 20.1z
72 73 72 73 73 72
72.5 16.9z 73.0 19.0z 72.3 17.7z 75.2 21.9 75.7 21.0z 66.6 21.1z
3; 2>3 1>3; 2>3
85.0 12.8 88.6 15.7 83.0 14.1 83.1 16.4 87.6 15.9 78.1 18.4
507 507 507 507 507 507
74.2 17.8z 77.6 21.1z 72.4 18.3z 72.6 20.1z 75.6 23.0z 69.1 22.0z
96 96 96 96 96 96
79.1 17.1 83.2 19.5 76.9 18.1 77.0 19.4 80.5 22.0 73.2 21.3
335 335 335 335 335 335
75.0 17.1z 78.3 20.3z 73.2 17.7z 73.0 20.1z 76.9 21.8z 69.8 22.0z
76 76 76 76 76 76
64.6 18.3z 67.2 23.4z 63.2 18.6z 65.3 19.5z 63.4 25.6z 60.9 21.0z
3 1>3; 2>3 1>3; 2>3 1>3; 2>3 1>3; 2>3
n
Mean ± SD
n
445 443 444 445 444 441
86.2 11.7 90.3 11.6 84.0 13.2 81.6 17.3 87.5 15.3 82.8 15.5
593 598 593 597 597 593
525 525 525 525 524 519
84.9 12.9 89.1 15.3 82.7 13.6 80.9 16.8 86.6 16.1 80.3 17.1
202 202 202 202 202 201 241 241 241 241 241 241
Mean ± SD
n
Mean ± SD
Between category†
1>3; 2>3 1>3
NS, not significant. *These P values represent ANOVA comparisons among the 3 CHD disease severity groups. †1, mild CHD; 2, BV CHD; 3, SV CHD. P < .05 for all comparisons. zRepresents a significant difference for comparisons between a specific CHD group and healthy controls (P < .0001).
CHD had significant deficits in each HRQOL domain except for adolescent-reported EmF, which did not differ between the SV CHD group and healthy controls and did not differ by disease severity category. This study demonstrates that children and adolescents with BV CHD and SV CHD who have undergone surgical or catheter-based intervention are
at increased risk for decreased HRQOL, and that those with SV CHD have significantly diminished HRQOL in both the physical and psychosocial (emotional, social, and school) domains. We also found that the composite CHD population had a similar HRQOL as pediatric patients in other chronic disease populations. However, when evaluated
Table IV. PedsQL scores in the CHD and chronic disease populations Scale
Composite CHDa
Mild CHDb
BV CHDc
SV CHDd
ESRDe
Obesityf
Asthmag
Diabetesh
Self-report Total score PhysHSS PsychHSS EmF SoF ScF Proxy report Total score PhysHSS PsychHSS EmF SoF ScF
n = 1098 77.2 15.7 79.5 17.5 76.0 16.5 74.1 20.8 81.2 18.9 72.9 19.3 n = 1132 74.5 17.2 77.7 21.1 72.8 17.5 71.1 19.5 76.6 21.7 70.6 21.3
n = 195 82.1 14.6 84.1 15.3 81.0 15.3 78.5 19.1 87.8 15.3 76.9 18.3 n = 203 81.0 15.5 84.6 18.6 79.1 16.5 76.4 18.5 83.9 19.7 76.8 20.3
n = 706 77.9 15.4 80.6 17.4 76.4 16.1 74.2 20.8 81.7 18.3 73.3 19.1 n = 721 75.0 16.8 78.5 20.8 73.2 17.0 71.1 19.4 77.5 21.0 70.9 21.1
n = 197 70.2 15.9 71.2 17.4 69.7 17.2 69.5 21.4 72.8 21.0 67.1 19.7 n = 208 66.3 17.0 68.3 21.1 65.2 17.4 66.0 19.7 66.1 22.1 63.5 21.0
n = 85 74.0 15.2 74.7 20.4 73.5 14.8 75.2 18.9 78.5 17.8 66.9 19.2 n = 95 69.6 18.1 71.2 24.2 68.9 17.6 68.7 19.7 73.5 21.0 63.2 21.4
n = 63 74.0 14.2 77.5 17.9 72.1 14.1 68.6 18.5 72.6 18.2 75.0 14.5 n = 63 75.0 14.5 76.3 17.6 73.9 15.3 72.6 17.8 73.5 17.3 76.6 17.0
n = 162 74.9 16.5 76.5 18.0 74.0 18.4 72.9 22.6 78.9 19.8 70.0 21.4 n = 157 68.8 15.9 72.7 18.4 66.8 16.7 64.8 20.2 73.4 18.7 62.4 20.6
n = 300 80.4 12.9 85.9 13.3 77.3 14.6 72.4 19.6 85.6 16.2 74.2 18.1 n = 307 76.6 14.1 82.0 17.2 73.7 15.3 69.2 18.5 81.1 19.3 70.6 19.4
P value* b>e, f, g; de, g; c>g; de, g; dg; b>e, g; c>g; dg; b>g, h b>e, g; dg; b>e, g; c>g; d
H
ealth-related quality of life (HRQOL) is defined as the impact of a specific illness, medical therapy, or health services policy on a patient’s ability to function in various life contexts and draw personal satisfaction from physical, psychological, and social functioning (SoF) perspectives.1 The Pediatric Quality of Life Inventory 4.0 Generic Core Scales (PedsQL) is a reliable, valid generic HRQOL measurement tool that is widely used in pediatric populations to determine HRQOL in patients with chronic diseases and healthy controls.2 The PedsQL can be used to characterize the impact of therapies and disease processes on the HRQOL of children and adolescents with congenital heart disease (CHD) and to compare this impact with that in children and adolescents with other chronic diseases. Given the cognitive and emotional differences between children and adolescents3 and differences in functioning among patients with heart disease (HD) of varying severity,4-12 individuals’ HRQOL may be affected differently, depending on age and disease severity. The effects of these factors on the HRQOL of the pediatric CHD population have not been delineated in a large, geographically
BV CHD EmF ESRD HD HRQOL PCQLI
Biventricle Congenital heart disease Emotional functioning End-stage renal disease Heart disease Health-related quality of life Pediatric Cardiac Quality of Life Inventory
PedsQL PhysHSS PsychHSS ScF SoF SV
Pediatric Quality of Life Inventory 4.0 Generic Core Scales Physical health summary score Psychosocial health summary score School functioning Social functioning Single ventricle
From the 1Division of Cardiology, Department of Pediatrics, 2Department of Biostatistics and Epidemiology, and 3Division of Behavioral and Clinical Psychology, Department of Pediatrics, Cincinnati Children’s Hospital Medical Center, Cincinnati, OH; 4 Division of Cardiology, Department of Pediatrics, The Children’s Hospital of Philadelphia, Philadelphia, PA; 5 Division of Cardiology, Department of Pediatrics, Children’s Hospital Boston, Boston, MA; 6Division of Cardiology, Department of Pediatrics, University of Texas Southwestern Medical Center Dallas, Dallas, TX; 7 Division of Cardiology, Department of Pediatrics, Children’s Hospital of Wisconsin, Milwaukee, WI; 8 Department of Cardiology, Phoenix Children’s Hospital, Phoenix, AZ; 9Department of Psychology and Neuroscience, Baylor University, Waco, TX; and 10 Division of Critical Care Medicine, Department of Pediatrics, Cincinnati Children’s Hospital Medical Center, Cincinnati, OH *A list of Pediatric Cardiac Quality of Life Inventory Testing Study Consortium sites is available at www. jpeds.com (Appendix). Supported by the National Institute of Child Health and Human Development (5-K23-HD048637), the American Heart Association, Pennsylvania/Delaware Affiliate (now Great Rivers Affiliate; beginning Grant-in-Aid 0465467U), the Cincinnati Children’s Hospital Research Foundation, and the Children’s Hospital of Philadelphia Institutional Development Fund. The authors declare no conflicts of interest. Portions of the study were presented as a poster at the American Academy of Pediatrics’ National Conference and Exhibition, San Francisco, CA, October 2-5, 2010. 0022-3476/$ - see front matter. Copyright ª 2014 Mosby Inc. All rights reserved. http://dx.doi.org/10.1016/j.jpeds.2013.11.066
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diverse cohort, however. Two previous studies13,14 assessed HRQOL in pediatric patients with HD; however, relatively small HD samples were used, and findings were mixed. In the present study, we aimed to use a geographically diverse cohort to compare patient and proxy-reported PedsQL scores in children (aged 8-12 years) and adolescents (aged 13-18 years) with CHD with those of sex- and racematched healthy controls. Within each age group, we also compared PedsQL scores among patients with CHD of various severity categories with one another and with controls to determine which severity categories within each age group might benefit from interventions targeting specific HRQOL domains. In addition, PedsQL scores among patients with CHD of various severity categories were compared to those of patients with other chronic diseases. We hypothesized that children and adolescents with CHD would have lower HRQOL than healthy controls, that HRQOL would not be significantly different between children and adolescents with mild CHD and healthy controls, and that children and adolescents with more complex CHD would have significant deficits in each HRQOL domain (total; physical health; psychosocial health; emotional functioning [EmF], SoF, and school functioning [ScF]) compared with healthy controls and patients with less complex disease. We also hypothesized that pediatric patients with complex CHD would have a similar HRQOL as those with other chronic diseases, and that pediatric patients with mild CHD would have a better HRQOL compared with those with other chronic diseases.
Methods This study was a cross-sectional survey that used patient data originally collected to test the psychometric properties of the Pediatric Cardiac Quality of Life Inventory (PCQLI). The Institutional Review Board of Cincinnati Children’s Hospital Medical Center approved this study and granted a waiver of consent based on its retrospective nature. The PedsQL data used for the CHD group in this study was provided by patients with CHD and their parents to establish construct validity during the PCQLI validation study.15-17 Patients from 7 large US pediatric cardiac centers (Appendix) contributed to the CHD patient population between November 2004 and December 2008. Eligibility criteria for participation in the PCQLI validation study included CHD or acquired HD, age 8-18 years, fluent in English, and attending an outpatient cardiology visit. Patients who had a significant comorbid medical condition or major developmental delay or who came to the clinic because of an acute change in clinical status were excluded. Proxy reporters were excluded if they were not fluent in English or had a major developmental delay. From the 1605 patient–parent pairs who were included in the PCQLI validation study, those patients with CHD who also had a valid self-reported or proxy-reported PedsQL score were included in the present study. The healthy population used in this study was obtained from the PedsQL healthy 782
Vol. 164, No. 4 children database.2,18 These normative data were obtained from the previously conducted PedsQL initial field test and a statewide State Children’s Health Insurance Program evaluation in California. The PedsQL data for the chronic disease populations were obtained from a previously published database.14 For the PCQLI validation study, patients and proxy reporters were given the PedsQL before a routine outpatient visit. Research personnel obtained patient and parent demographic information and patient clinical information (diagnostic, surgical, and medical history) for the CHD population through parent report and chart review. The original diagnostic category was divided into 2 subgroups: biventricle (BV) CHD and single ventricle (SV) CHD. CHD severity categories included mild CHD (patients with CHD who had not undergone surgical or catheter-based intervention), BV CHD (patients with CHD with BV physiology who had undergone surgical or catheter-based intervention), and SV CHD (patients with CHD with SV physiology who had undergone surgical palliation). The number of cardiac surgeries (defined as sternotomy or thoracotomy) and number of cardiac catheterizations were reported for the BV CHD and SV CHD groups only. The time since the most recent hospitalization was reported for all CHD disease severity categories. For the healthy control population, patient sex and race data for the child and adolescent groups were obtained from the PedsQL healthy children database. The PedsQL, developed by Varni et al, was used to assess HRQOL and has well-documented reliability and validity in the pediatric HD and healthy populations.2,13,14,18 The PedsQL includes 23 items that evaluate physical functioning and psychosocial functioning. The physical health summary score (PhysHSS) includes physical functioning, and the psychosocial health summary score (PsychHSS) includes EmF, SoF, and ScF. The total score incorporates all of these domains. There are separate forms for children aged 8-12 years and adolescents aged 13-18 years as well as their proxy reporters; the content of the items in each form are the same except for appropriate changes to pronouns and language modifications to accommodate developmental differences. All answers are based on a 5-point Likert scale (0, not a problem; 1, almost never a problem; 2, sometimes a problem; 3, often a problem; 4, almost always a problem). Items are reverse-scored and linearly transformed to a scale of 0-100, with higher transformed scores indicating a better HRQOL. The number of cardiac surgeries, number of cardiac catheterizations, and time since the most recent hospitalization were compared in the BV CHD and SV CHD groups using the Wilcoxon rank-sum test. Patient and proxy-reported PedsQL scores (total score, PhysHSS, PsychHSS, EmF, SoF, and ScF) were compared between the composite pediatric CHD group and sex- and race-matched healthy controls in 2 age groups (children [age 8-12 years] and adolescents [age 13-18 years]) and between patients in each of 3 CHD severity categories and healthy controls in both age groups using t tests. Patient and proxy-reported PedsQL scores (total score, PhysHSS, PsychHSS, EmF, SoF, and ScF) were Mellion et al
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Table I. Characteristics of patients with CHD Composite CHD Child
Adolescent
Mild CHD Child
BV CHD
Adolescent
Child
Adolescent
SV CHD Child
Adolescent
Characteristics
(n = 625)
(n = 513) (n = 107; 17%) (n = 97; 19%) (n = 386; 62%) (n =340; 66%) (n = 132; 21%) (n = 76; 15%)
Patient sex male, n (%) Parent sex male, n (%) Patient ethnicity Hispanic, n (%) Parent ethnicity Hispanic, n (%) Patient race, n (%) White African American Other Parent race, n (%) White African American Other Patient age, y, mean SD Parent age, y, mean SD Number of cardiac surgeries, mean SD Number of cardiac catheterizations, mean SD Years since last hospitalization, mean SD
338 (54.1) 85 (13.6) 42 (6.7) 35 (5.6)
301 (58.7) 84 (16.4) 30 (5.8) 25 (4.9)
57 (53.3) 14 (13.1) 13 (12.1) 14 (13.1)
63 (64.9) 12 (12.4) 7 (7.2) 6 (6.2)
202 (52.3) 58 (15.0) 19 (4.9) 12 (3.1)
194 (57.1) 61 (17.9) 18 (5.3) 15 (4.4)
79 (59.8) 13 (9.8) 10 (7.6) 9 (6.8)
44 (57.9) 11 (14.5) 5 (6.6) 4 (5.3)
510 (81.6) 49 (7.8) 66 (10.6)
431 (84.0) 42 (8.2) 40 (7.8)
80 (74.8) 7 (6.5) 20 (18.7)
82 (84.5) 9 (9.3) 6 (6.2)
318 (82.4) 31 (8.0) 37 (9.6)
276 (81.2) 33 (9.7) 31 (9.1)
112 (84.8) 11 (8.3) 9 (6.8)
73 (96.1) 0 (0.0) 3 (3.9)
522 (83.5) 48 (7.7) 55 (8.8) 9.8 1.4 40.8 6.5 1.5 1.4
440 (85.8) 41 (8.0) 32 (6.2) 15.1 1.6 45.1 6.2 1.6 1.6
79 (73.8) 7 (6.5) 21 (19.6) 9.8 1.4 40.5 6.7 NA
80 (82.5) 10 (10.3) 7 (7.2) 15.3 1.6 45.5 6.3 NA
330 (85.5) 31 (8.0) 25 (6.5) 9.8 1.5 40.9 6.3 1.3 1.0
285 (83.8) 31 (9.1) 24 (7.1) 15.2 1.7 45.3 6.2 1.6 1.2
113 (85.6) 10 (7.6) 10 (7.6) 9.6 1.4 40.5 6.8 3.2 0.9
75 (98.7) 0 (0.0) 1 (1.3) 14.8 1.6 43.7 5.6 3.7 1.6
1.6 2.0
1.9 2.2
NA
NA
1.4 1.9
1.8 2.1
3.4 1.8
4.2 2.1
5.2 3.6
6.9 5.6
7.0 3.4
9.6 5.6
5.5 3.7
6.8 5.7
3.8 2.6
5.9 4.3
NA, not applicable.
compared between the composite pediatric CHD population and each of 4 chronic disease groups (end-stage renal disease [ESRD], obesity, asthma, and diabetes), as well as between patients in each of the 3 CHD severity categories and each of the chronic disease groups using t tests. Age group comparisons could not be performed, however, because chronic disease data were not separated into child and adolescent age groups. After Bonferroni correction to adjust for multiple comparisons, P < .00017 was considered statistically significant for all t tests. In addition, patient and proxy-reported PedsQL scores (total score, PhysHSS, PsychHSS, EmF, SoF, and ScF) were compared among the 3 CHD severity categories in both the child and adolescent age groups using 1-way ANOVA with the Tukey post hoc test.
Results PedsQL scores were available for 1138 patient and/or proxy reporters with CHD (Table I). The CHD child age group comprised 625 patient and/or proxy reporters, 54.1% of whom were male and 81.6% of whom were Caucasian. The CHD adolescent age group comprised 513 patient and/or proxy reporters, 58.7% of whom were male and 84.0% of whom were Caucasian. The sex- and race-matched healthy control group included 771 patient and/or proxy reporters. The healthy child age group had 528 patient/parent respondents, 48.3% of whom were male and 84.7% of whom were Caucasian. The healthy adolescent age group had 243 respondents, 58.4% of whom were male and 87.7% of whom were Caucasian. The original diagnostic categories and specific diagnoses for the CHD population are provided in Table II. Patients in the SV CHD group had a greater number of cardiac surgeries (children, 3.2 vs 1.3, P < .0001; adolescents, 3.7 vs
1.6, P < .0001) and cardiac catheterizations (children, 3.4 vs 1.4, P < .0001; adolescents, 4.2 vs 1.8, P < .0001) compared with those in the BV CHD group. There was a significant difference in the time from most recent hospitalization between the SV CHD and BV CHD groups in the child age group (3.8 vs 5.5; P < .0001), but not in the adolescent age group (5.9 vs 6.8, P = .3037). Patient and proxy-reported PedsQL scores in both the child and adolescent composite CHD groups were significantly lower compared with healthy controls (P < .0001 for all comparisons; Table III). There were no significant differences between children and adolescents with mild CHD and healthy controls with the exception of childreported PhysHSS score (P < .0001). Children and adolescents with BV and SV CHD had lower scores in each HRQOL domain compared with healthy controls (P < .0001 for all comparisons), with the exception of adolescent-reported EmF score in the SV CHD group. Furthermore, children and adolescents with BV and SV CHD had lower scores in each HRQOL domain compared with patients with mild CHD, with the exception of EmF in the adolescent age group. For patient and proxyreported scores in both child and adolescent age groups, differences were seen among the disease severity categories in all HRQOL domains with the exception of adolescentreported EmF score (Table III). In those areas where the ANOVA revealed significant differences among groups, 2way comparisons between groups revealed that SV CHD groups had significantly lower scores compared with both the mild CHD and BV CHD groups in all HRQOL domains, with the exception of adolescent-reported ScF in the BV CHD group. However, in the adolescent age group there were no significant differences between the mild CHD and BV CHD groups in any patient or proxy-
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Table II. Primary diagnoses Diagnoses
Children (n = 625) Adolescents (n = 513) Total (n = 1138)
Original diagnostic category Biventricular CHD SV CHD Septal defects Atrial septal defect (patent foramen ovale, secundum, primum, or sinus venosus) Ventricular septal defect (single or multiple) Atrioventricular septal defect (complete or transitional) Aortopulmonary window Pulmonary venous anomalies Partial anomalous pulmonary venous connection Total anomalous pulmonary venous connection Right heart lesions Tetralogy of Fallot Tetralogy of Fallot with pulmonary atresia Tetralogy of Fallot with absent pulmonary valve Pulmonary atresia with intact ventricular septum Ebstein anomaly Tricuspid stenosis Pulmonary stenosis, valvar Pulmonary artery stenosis, branch Double-chambered right ventricle Other outflow-tract abnormalities in right ventricle Left heart lesions Aortic stenosis Aortic insufficiency Aortic insufficiency and aortic stenosis Mitral stenosis Mitral regurgitation Mitral valve prolapse (without moderate or greater mitral regurgitation) Bicuspid aortic valve (nonobstructive) Other outflow tract abnormalities in left ventricle SV Hypoplastic left heart syndrome Double-inlet left ventricle Double-inlet right ventricle Mitral atresia (not hypoplastic left heart syndrome of double-outlet right ventricle/mitral atresia) Tricuspid atresia Unbalanced atrioventricular canal Right ventricle-aorta with pulmonary atresia Superoinferior ventricle (with or without straddling tricuspid valve) Conotruncal anomalies Transposition of great arteries Double-outlet right ventricle Truncus arteriosus Thoracic arteries and veins Coronary artery anomaly Coarctation of aorta Interrupted aortic arch Patent ductus arteriosus Vascular rings and slings (with or without tracheal stenosis)
reported HRQOL score. In stark contrast, there was a significant difference between the mild CHD and BV CHD groups in all HRQOL domains for child-reported scores and in total, PsychHSS, and SoF scores for proxy reports in the child age group. Patients in the composite CHD group reported similar scores as patients with ESRD, asthma, obesity, and diabetes (Table IV). Proxy-reported PedsQL scores in the composite CHD group were similar to those in the ESRD, obesity, and diabetes groups but significantly higher than those in the asthma group. HRQOL of the mild CHD group was generally similar to that of the diabetes group but better than that of the ESRD and asthma groups according to both patient and proxy reports. HRQOL of 784
625 495 130 132 48 70 11 3 6 3 3 136 52 16 6 15 13 1 29 1 3 0 79 47 4 3 3 6 0 14 2 113 37 18 0 9 22 12 12 3 74 54 11 9 85 8 64 5 7 1
513 439 74 75 33 39 3 0 9 4 5 115 45 9 3 13 10 0 28 4 2 1 105 44 2 8 5 11 8 27 0 72 20 10 3 8 17 7 6 1 61 45 5 11 76 5 62 6 2 1
1138 934 204 207 81 109 14 3 15 7 8 251 97 25 9 28 23 1 57 5 5 1 184 91 6 11 8 17 8 41 2 185 57 28 3 17 39 19 18 4 135 99 16 20 161 13 126 11 9 2
the SV CHD population was generally similar to that of the ESRD, asthma, and obesity groups but worse than that of the diabetes group according to both patient and proxy reports. Interestingly, patient-reported EmF was similar across all comparisons.
Discussion This study is the first to characterize HRQOL in the pediatric CHD population by both age group and disease severity. PedsQL scores of both children and adolescents with mild CHD were not significantly different from those of healthy controls, with the exception of child-reported PhysHSS. Moreover, both children and adolescents with more complex Mellion et al
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Table III. PedsQL scores and comparisons PedsQL score Healthy Scale Child Self-report Total score PhysHSS PsychHSS EmF SoF ScF Proxy report Total score PhysHSS PsychHSS EmF SoF ScF Adolescent Self-report Total score PhysHSS PsychHSS EmF SoF ScF Proxy report Total score PhysHSS PsychHSS EmF SoF ScF
Composite CHD
Mild CHD1
BV CHD2
SV CHD3
Disease severity comparison
n
Mean ± SD
n
Mean ± SD
Among category, P value*
75.8 15.9z 99 81.3 14.1 77.9 17.6z 101 83.4 15.1z 74.7 16.7z 99 80.3 14.8 71.2 20.8z 101 76.4 19.0 79.6 19.8z 101 87.7 14.7 73.3 18.4z 99 76.8 17.2
371 373 371 372 372 371
76.6 15.8z 78.9 17.8z 75.3 16.5z 71.4 21.1z 80.2 19.6z 74.3 18.1z
123 124 123 124 124 123
68.8 15.3z 70.1 16.4z 68.2 16.9z 66.2 20.4z 71.1 20.9z 67.5 18.9z
3 1>2, 3; 2>3 1>3; 2>3 1>2, 3; 2>3 1>3; 2>3
625 625 625 625 625 625
74.7 16.7z 77.8 21.0z 73.1 16.8z 70.0 18.9z 77.4 20.5z 71.9 20.7z
107 107 107 107 107 107
82.7 13.6 85.9 17.7 81.0 14.7 76.0 17.8 87.0 16.9 80.0 18.9
386 386 386 386 386 386
75.1 16.6z 78.5 21.3z 73.2 16.5z 69.6 18.6z 78.1 20.3z 72.0 20.3z
132 132 132 132 132 132
67.3 16.2z 69.0 19.8z 66.4 16.6z 66.4 19.8z 67.7 19.8z 65.1 20.9z
2, 3; 2>3 1>2, 3; 2>3 1>2, 3; 2>3 1>2, 3; 2>3
86.9 11.8 90.2 12.4 85.1 13.5 83.7 17.0 90.2 15.2 81.2 16.2
499 500 499 500 500 499
79.0 15.4z 81.5 17.2z 77.7 16.2z 77.6 20.3z 83.1 17.6z 72.4 20.3z
94 94 94 94 94 94
82.9 15.1 84.9 15.6 81.9 15.9 80.7 19.1 87.8 16.0 77.1 19.5
333 333 333 333 333 333
79.3 14.8z 82.4 16.7z 77.6 15.6z 77.2 20.2z 83.4 16.7z 72.3 20.1z
72 73 72 73 73 72
72.5 16.9z 73.0 19.0z 72.3 17.7z 75.2 21.9 75.7 21.0z 66.6 21.1z
3; 2>3 1>3; 2>3
85.0 12.8 88.6 15.7 83.0 14.1 83.1 16.4 87.6 15.9 78.1 18.4
507 507 507 507 507 507
74.2 17.8z 77.6 21.1z 72.4 18.3z 72.6 20.1z 75.6 23.0z 69.1 22.0z
96 96 96 96 96 96
79.1 17.1 83.2 19.5 76.9 18.1 77.0 19.4 80.5 22.0 73.2 21.3
335 335 335 335 335 335
75.0 17.1z 78.3 20.3z 73.2 17.7z 73.0 20.1z 76.9 21.8z 69.8 22.0z
76 76 76 76 76 76
64.6 18.3z 67.2 23.4z 63.2 18.6z 65.3 19.5z 63.4 25.6z 60.9 21.0z
3 1>3; 2>3 1>3; 2>3 1>3; 2>3 1>3; 2>3
n
Mean ± SD
n
445 443 444 445 444 441
86.2 11.7 90.3 11.6 84.0 13.2 81.6 17.3 87.5 15.3 82.8 15.5
593 598 593 597 597 593
525 525 525 525 524 519
84.9 12.9 89.1 15.3 82.7 13.6 80.9 16.8 86.6 16.1 80.3 17.1
202 202 202 202 202 201 241 241 241 241 241 241
Mean ± SD
n
Mean ± SD
Between category†
1>3; 2>3 1>3
NS, not significant. *These P values represent ANOVA comparisons among the 3 CHD disease severity groups. †1, mild CHD; 2, BV CHD; 3, SV CHD. P < .05 for all comparisons. zRepresents a significant difference for comparisons between a specific CHD group and healthy controls (P < .0001).
CHD had significant deficits in each HRQOL domain except for adolescent-reported EmF, which did not differ between the SV CHD group and healthy controls and did not differ by disease severity category. This study demonstrates that children and adolescents with BV CHD and SV CHD who have undergone surgical or catheter-based intervention are
at increased risk for decreased HRQOL, and that those with SV CHD have significantly diminished HRQOL in both the physical and psychosocial (emotional, social, and school) domains. We also found that the composite CHD population had a similar HRQOL as pediatric patients in other chronic disease populations. However, when evaluated
Table IV. PedsQL scores in the CHD and chronic disease populations Scale
Composite CHDa
Mild CHDb
BV CHDc
SV CHDd
ESRDe
Obesityf
Asthmag
Diabetesh
Self-report Total score PhysHSS PsychHSS EmF SoF ScF Proxy report Total score PhysHSS PsychHSS EmF SoF ScF
n = 1098 77.2 15.7 79.5 17.5 76.0 16.5 74.1 20.8 81.2 18.9 72.9 19.3 n = 1132 74.5 17.2 77.7 21.1 72.8 17.5 71.1 19.5 76.6 21.7 70.6 21.3
n = 195 82.1 14.6 84.1 15.3 81.0 15.3 78.5 19.1 87.8 15.3 76.9 18.3 n = 203 81.0 15.5 84.6 18.6 79.1 16.5 76.4 18.5 83.9 19.7 76.8 20.3
n = 706 77.9 15.4 80.6 17.4 76.4 16.1 74.2 20.8 81.7 18.3 73.3 19.1 n = 721 75.0 16.8 78.5 20.8 73.2 17.0 71.1 19.4 77.5 21.0 70.9 21.1
n = 197 70.2 15.9 71.2 17.4 69.7 17.2 69.5 21.4 72.8 21.0 67.1 19.7 n = 208 66.3 17.0 68.3 21.1 65.2 17.4 66.0 19.7 66.1 22.1 63.5 21.0
n = 85 74.0 15.2 74.7 20.4 73.5 14.8 75.2 18.9 78.5 17.8 66.9 19.2 n = 95 69.6 18.1 71.2 24.2 68.9 17.6 68.7 19.7 73.5 21.0 63.2 21.4
n = 63 74.0 14.2 77.5 17.9 72.1 14.1 68.6 18.5 72.6 18.2 75.0 14.5 n = 63 75.0 14.5 76.3 17.6 73.9 15.3 72.6 17.8 73.5 17.3 76.6 17.0
n = 162 74.9 16.5 76.5 18.0 74.0 18.4 72.9 22.6 78.9 19.8 70.0 21.4 n = 157 68.8 15.9 72.7 18.4 66.8 16.7 64.8 20.2 73.4 18.7 62.4 20.6
n = 300 80.4 12.9 85.9 13.3 77.3 14.6 72.4 19.6 85.6 16.2 74.2 18.1 n = 307 76.6 14.1 82.0 17.2 73.7 15.3 69.2 18.5 81.1 19.3 70.6 19.4
P value* b>e, f, g; de, g; c>g; de, g; dg; b>e, g; c>g; dg; b>g, h b>e, g; dg; b>e, g; c>g; d
