Quality of life in pediatric heart transplant recipients: A comparison with children with and without heart disease

Transcription

1 Quality of life in pediatric heart transplant recipients: A comparison with children with and without heart disease Karen Uzark, PhD, CPNP, a Lisa Griffin, BSN, CCTC, b Rose Rodriguez, MSN, c Meg Zamberlan, MS, CPNP, d Paula Murphy, RN, MBA, e Colleen Nasman, BSN, f Joanne Dupuis, MSN, g Sherrie Rodgers, MSN, h Christine A. Limbers, PhD, i and James W. Varni, PhD j From a Children s Hospital Medical Center, Cincinnati, Ohio; b Loma Linda University, Loma Linda, California; c Columbia University Medical Center, New York, New York; d University of Michigan Medical Center, Ann Arbor, Michigan; e St Louis Children s Hospital, St. Louis, Missouri; f Cleveland Clinic Foundation, Cleveland, Ohio; g Children s Hospital of Michigan, Detroit, Michigan; h Children s Memorial Hospital, Chicago, Illinois; i Baylor University, Waco, Texas; and j Texas A & M University, College Station, Texas KEYWORDS: pediatric heart transplant; quality of life; psychosocial impact; congenital heart disease; transplant outcomes; children BACKGROUND: Little is known about the quality of life (QOL) of children with heart disease who undergo life-saving surgery. The aim of this multicenter study was to examine self- and parent-reported QOL outcomes in pediatric heart transplant recipients. METHODS: Pediatric heart transplant recipients/families (n 174) from 7 transplant programs completed the Pediatric Quality of Life Inventory Generic Core Scales and Cardiac Module. Scores for the heart transplant sample were compared with non-transplant patients who had undergone conventional cardiac surgery and with a healthy child sample. Within the cardiac surgery group, heart disease/surgery was further categorized by severity/complexity. RESULTS: Heart transplant recipients were a mean age of years at a mean time posttransplant of years. By both self-report and parent proxy report, mean scores for heart transplant recipients were significantly lower than those in healthy children for physical and psychosocial QOL, including emotional and social functioning (p 0.001), with 31.3% self-reporting significantly impaired psychosocial QOL scores. By self-report, there were no significant differences in emotional and social mean scores between the transplant and cardiac surgery groups. Transplant recipients reported significantly fewer cardiac symptoms than children with cardiac surgery (p 0.01). Their self-reported school functioning scores were not significantly different from children with moderate to severe disease. CONCLUSION: Although pediatric heart transplant recipients experience significant symptomatic improvement, they remain at-risk for impaired psychosocial QOL, similar to children with residual or palliated heart disease. Assessment is needed to identify children at-risk and improve psychosocial outcomes. J Heart Lung Transplant 2012;31: International Society for Heart and Lung Transplantation. All rights reserved. According to data from the International Society for Heart and Lung Transplantation Registry, overall survival after pediatric heart transplantation continues to improve. 1 In addition Reprint requests: Karen Uzark, PhD, CPNP, University of Michigan Mott Children s Hospital, L1242 Women s, SPC 5204, 1500 E Medical Center Dr, Ann Arbor, MI Telephone: Fax: address: karenu@med.umich.edu /$ -see front matter 2012 International Society for Heart and Lung Transplantation. All rights reserved. doi: /j.healun to the preservation of life, however, the goal of transplantation is an improved quality of life (QOL) for pediatric heart transplant recipients. Registry measures of functional status are limited but report that 93% of transplant recipients who survive at least 10 years have no limitations in physical activity. 1 However, little is known about the psychosocial QOL of pediatric survivors of heart transplantation. Approximately 33% of heart transplant recipients have been reported to have behavior problems in the clinical

2 572 The Journal of Heart and Lung Transplantation, Vol 31, No 6, June 2012 range, as reported by their parents. 2 The prevalence of psychosocial problems is reported to be as high as 52% at 12 months after transplant. 3 DeMaso et al 4 reported that approximately 25% of pediatric transplant recipients had emotional difficulties at a median of 9.6 years after transplantation and observed that pediatric heart transplant patients with psychologic difficulties before and after transplantation had more hospitalizations after transplantation. 5 A significant number of children who undergo successful transplantation experience difficulties at school, with educational problems more prevalent in the medium-term after transplant. 6 These studies are limited by small sample size across a wide age span as well as by measurement issues, typically reliant on parental proxy report rather than self-report. However, a comprehensive understanding of QOL in these children is important to optimize health care outcomes. The objective of this study was to assess QOL in pediatric heart transplant recipients as perceived by children and their parents and to compare parental proxy and self-reported QOL in heart transplant recipients vs children with heart disease who underwent non-transplant surgical interventions and with a healthy comparison group. Methods Patient population The study recruited 137 pediatric heart transplant recipients aged 6 to 18 years and parents of 172 children aged 2 to 18 years from 7 pediatric heart transplant programs across the United States. The study was approved by the Institutional Review Board at each institution. Heart transplant recipients/families were approached by the transplant/research coordinator at the time of a routine outpatient follow-up visit, or in one institution (Loma Linda University) where most of the transplant recipients were no longer living locally, recipients were contacted by phone. No family declined participation in the study. The heart disease comparison group was derived from a previous study 7 and consisted of children of the same age who had shared the experience of cardiac surgery. Within the heart disease sample, heart disease severity was categorized as surgically corrected curative (Group 2 mild), surgically treated with significant residua or need for additional surgery (Group 3 moderate), or complex or severe heart disease, uncorrectable or palliated, including single ventricle (Group 4 severe). Group 1 patients were excluded because they had not undergone cardiac surgery. Children in the heart transplant and non-transplant heart disease samples were excluded if they had a major developmental disability or an associated non-cardiac condition unrelated to the transplant that might be expected to affect QOL, or if less than 4 months had passed since their operation. Patients who had renal disease, coronary artery disease, or other complications of transplant were not excluded unless they were experiencing acute symptoms related to these complications at the time of assessment. The healthy comparison sample was derived from the Pediatric Quality of Life Inventory (PedsQL) healthy children database, 8,9 and was age-matched to the heart transplant sample. Procedures and measures To assess health-related QOL, patients and parents completed the Pediatric Quality of Life (PedsQL) Inventory. 10 The 23-item PedsQL 4.0 Generic Core Scales encompass Physical, Emotional, Social, and School. The PedsQL scales consist of parallel child self-report and parent proxy-report formats. Items are reverse-scored and linearly transformed to a0to100scale, so that higher scores indicate better QOL. To create a Psychosocial Health Summary score, the mean is computed as the sum of the items divided by the number of items in the Emotional, Social, and School scales. The reliability and validity of the PedsQL Generic Core Scales has been demonstrated in healthy and patient populations. 8,9 The 27-item PedsQL 3.0 Cardiac Module has 5 scales related to Heart Problems/Symptoms, Perceived Physical Appearance, Treatment Anxiety, Cognitive Problems, and Communication. The Communication Scale is not included for toddlers and young children who do not have the cognitive or language ability to verbalize questions and explanations about the heart. An additional Treatment Barriers scale is imbedded in the module to measure adherence issues for patients who take medications. The validity and reliability of the PedsQL Cardiac Module scales for parent proxy-report for children aged 2 to 18 years and for self-report for children aged 8 to 18 years have been previously reported. 11 The reliability of the Cardiac Module scales was again assessed in the multicenter heart transplant sample reported here and approached or exceeded the reliability standard of 0.70 for group comparison, with coefficients ranging from 0.67 to Statistical analyses Descriptive statistics were generated for demographic and clinical variables and are reported as means and standard deviations (SDs) for continuous variables and frequencies and proportions for categoric variables. Mean PedsQL scale and summary scores were calculated for the pediatric heart transplant sample and comparison groups. PedsQL Generic Core Scales scores for the heart transplant sample were compared with a heart disease non-transplant sample and a healthy child sample of the same age using independent sample t-tests. Differences between PedsQL Generic Core and Cardiac Module Scale scores were also determined across 3 categories of cardiac disease severity and heart transplant using analysis of variance with Tukey post hoc tests. Individual Psychosocial Health Summary scores for heart transplant patients were plotted to determine the frequency of scores 1 SD below the population sample mean, the cutoff score for clinically significant impairment. 9 Individual item analysis with ranking was performed to identify the most significant problems or lowest mean scores within each scale and the frequency of responses. Within the heart transplant group, Psychosocial Health Summary scores were also compared related to primary immunosuppressant (cyclosporine vs tacrolimus) by independent sample t-tests. Pearson correlations were computed to assess the relationship between PedsQL Psychosocial Health Summary scores and continuous variables, including demographics, such as age at assessment, socioeconomic status, age at transplant, and time since transplant.

3 Uzark et al. Quality of Life in Pediatric Heart Transplant 573 Results Patient characteristics Demographic characteristics of the transplant and comparison groups are reported in Table 1. Transplant recipients at PedsQL completion were a mean age of years and marginally older than the cardiac surgery group, who were a mean age of years (p 0.04). The mean socioeconomic status (SES) based on the Hollingshead 4-factor index indicated on average a middle-class family SES, with no significant differences between heart transplant and non-transplant cardiac groups. There were significantly more Hispanic patients with a concomitant lesser proportion of Caucasian patients in the transplant sample than in the non-transplant cardiac group (p 0.05). Median age at transplant was 2 years (range, 4 days 17.8 years. Mean time since transplant was years, median 5 years (range, years). The mean time since the most recent surgery in the non-transplant heart surgery comparison group was years, not significantly different from the time since heart transplant surgery. Primary transplant immunosuppression was cyclosporine (56%) and tacrolimus (43%). One patient was receiving sirolimus. Heart transplant recipients were taking a mean of 5.4 medications vs 0.57 in the heart surgery group (p ). Comparison of QOL scores across heart surgery and healthy groups Self-report Comparisons of PedsQL mean scores for pediatric heart transplant, cardiac surgery, and healthy children are reported in Table 2. By self-report, overall, physical and psychosocial PedsQL mean scores were significantly lower in pediatric heart transplant recipients than in healthy peers (p 0.001) and in children with curative heart surgery (p 0.01 physical, p 0.05 psychosocial), but were not significantly different from children with moderate (Group 3) or severe/complex (Group 4) heart disease. On the Emotional and Social Scales, mean scores for the heart transplant group were significantly lower than healthy children (p 0.001), but there were no significant differences in emotional and social PedsQL scores between the heart transplant and cardiac surgery groups. Mean scores for School were significantly lower in the heart transplant group than in the healthy children (p 0.01) and in the overall cardiac surgery group (p 0.01) but were not significantly different from patients in the Group 3 or 4 severity category. Comparisons of Cardiac Module Scores for pediatric heart transplant patients and cardiac surgery groups are reported in Table 3. On the Heart Problems/Symptoms scale of the PedsQL Cardiac Module, the heart transplant group had a significantly higher mean PedsQL score (fewer symptoms) than the overall heart surgery group (p 0.01), including Group 3 (p 0.01) and Group 4 patients (p 0.001). Mean Heart Problems/Symptoms Scale scores were not significantly different from patients with mild heart disease (Group 2). There were no differences between transplant and cardiac surgery groups in self-reported Physical Appearance Scale scores. The mean Treatment Anxiety scale score was significantly lower in heart transplant patients than all cardiac groups (overall p 0.01, Group 3 p 0.05) except Group 4. Cognitive problems scale means were significantly lower in heart transplant patients than in the overall cardiac surgery group (p 0.01) and Group 2 patients (p 0.001), but were not different from Group 3 and 4 patients. Lower communication scores were reported in heart transplant patients only in comparison with patients with mild heart disease. Table 1 Participant Characteristics Variables a Heart transplant (n 172) Cardiac group (n 362) Healthy (n 1,664) Characteristics Age, years b Sex Boys 92 (53) 208 (57) 835 (50) Girls 80 (47) 154 (43) 829 (50) Race/ethnicity Caucasian 126 (72) 320 (88) c 1,181 (71) Black 22 (13) 35 (10) 195 (12) Hispanic/Latino 16 (9) 1 ( 1) b 164 (10) Other 5 (3) 6 (2) 124 (7) Missing 3 (2) 0 (0) 0 (0) Socioeconomic status Hollingshead Index Unknown a Continuous data are shown as the mean standard deviation and categoric data as number (%). b p 0.04 compared with heart transplant group by independent sample t-tests. c p 0.05 compared with heart transplant group by chi-square test.

4 574 The Journal of Heart and Lung Transplantation, Vol 31, No 6, June 2012 Table 2 Pediatric Quality of Life 4.0 Generic Core Scales Scores for Pediatric Heart Transplant Patients and Comparisons with Healthy and Cardiac Samples Variable Heart transplant a Healthy b Cardiac severity PedsQL scale Generic Core Scales Group 2 c Group 3 d Group 4 e Combined f Mean SD Mean SD Mean SD Mean SD Mean SD Mean SD (n 137) (n 1,156) (n 80) (n 98) (n 86) (n 263) Differences Child Self-Report Total Score a b***; a c** Physical Health a b***; a c** Psychosocial Health a b***; a c* Emotional a b*** Social a b*** School a b, c***; a f** (n 172) (n 1648) (n 117) (n 129) (n 116) (n 362) Parent Proxy- Report Total Score a b, c*** Physical Health a b, c***; a e** Psychosocial a b, c***; Health a f* Emotional a b***; a c* Social a b, c***; School a f* a b, c, f***; a d, e* PedsQL, Pediatric Quality of Life; SD, standard deviation. Heart disease severity was categorized as: 2 surgically corrected (curative); 3 surgically treated cardiovascular disease (one or more procedures) with significant residua or need for further surgery; 4 complex or severe cardiovascular disease, uncorrectable or palliated (includes single ventricle). *p 0.05; ** p 0.01;***p a heart transplant; b healthy; c severity group 2; d severity group 3; e severity group 4; f all severity groups combined. As previously noted, 1 SD below the population mean represents a meaningful cutoff for significantly impaired QOL. 9 As reported by pediatric heart transplant recipients, 31.3% had scores 1 SD below the population mean for psychosocial functioning (Figure 1). For the non-transplant heart surgery comparison group, 25.5% of self-reported psychosocial functioning scores were 1 SD below the population mean, not significantly different from the heart transplant group. Parent proxy-report By parent proxy-report (Table 2), heart transplant recipients had significantly lower mean PedsQL Physical Health scores than the healthy group and Group 2 cardiac surgery patients (p 0.001), but scored significantly higher than Group 4 patients (p 0.01). With respect to psychosocial functioning, the mean PedsQL scores were also significantly lower than healthy (p 0.001) and Group 2 patients (p 0.001), but not significantly different from patients with more severe disease. On the Emotional and Social Scales, parent-reported mean scores for the heart transplant group were significantly lower than those for healthy children (p 0.001), similar to the self-reported scores. But unlike the transplant recipients self-reports, parents reported somewhat lower scores for Emotional functioning than Group 2 patients (p 0.05) and lower scores for Social than the Group 2 patients (p 0.001) and the overall cardiac surgery group p 0.05). Mean PedsQL scores for school functioning by parent report were significantly lower in transplant recipients than healthy (p 0.001) and all cardiac surgery groups (overall and Group 2, p 0.001).

5 Uzark et al. Quality of Life in Pediatric Heart Transplant 575 Table 3 Samples Pediatric Quality of Life Cardiac Module Scores for Pediatric Heart Transplant Patients and Comparisons With Cardiac Variable Heart transplant a Cardiac severity b PedsQL Scale Cardiac module Child self-report Symptoms/ Treatment I Physical Appearance Treatment Anxiety Cognitive Problems Group 2 c Group 3 d Group 4 e Combined f Mean SD Mean SD Mean SD Mean SD Mean SD (n 137) (n 80) (n 97) (n 85) (n 259) Differences a d, f**; a e*** a c, f**; a d* a c***; a f** Communication a c** Parent proxy-report Symptoms/ Treatment I (n 169) (n 117) (n 129) (n 115) (n 360) a c*; a d*; a e*; a f** Physical Appearance Treatment Anxiety Cognitive a c** Problems Communication a c* PedsQL, Pediatric Quality of Life; SD, standard deviation. b Heart disease severity was categorized as: 2 surgically corrected (curative); 3 surgically treated cardiovascular disease (one or more procedures) with significant residua or need for further surgery; 4 complex or severe cardiovascular disease, uncorrectable or palliated (includes single ventricle). * p 0.05, ** p 0.01,***p a heart transplant; b healthy; c severity group 2; d severity group 3; e severity group 4; f all severity groups combined. As reported in Table 3, on the Heart Problems/Symptoms scale of the PedsQL cardiac module, the heart transplant group had a significantly higher mean PedsQL score (fewer symptoms) by parent report than all cardiac surgery groups (p 0.05) except for Group 2 patients, where they were significantly lower (p 0.05). There were no significant differences in mean PedsQL scores for physical appearance or treatment anxiety between the transplant group and any cardiac surgery group. Cognitive Problems scale scores were significantly lower in transplant recipients only in comparison with Group 2 patients (p 0.01). As reported by the parents, mean scores for communication were also lower only in comparison with Group 2 patients (p 0.05). Parents of heart transplant recipients and the recipients both reported a similar incidence of impaired psychosocial functioning, 33.7% with scores 1 SD below the population mean. For the non-transplant heart surgery comparison group, 19.6% of parent proxy-reports indicated psychosocial functioning scores were 1 SD below the population mean, which was significantly less frequent than the transplant group (p 0.05). Specific concerns of heart transplant recipients Mean scores for the specific items of the PedsQL Generic Core and Cardiac Module scales were ranked to identify the specific problems that are most frequently perceived by pediatric heart transplant recipients. The frequency of patients reporting often or almost always experiencing a specific problem was examined. With respect to physical functioning or symptoms, the most frequently reported problems were difficulty running (10.2%) or getting out of breath with sports or exercise (14%). Feeling angry often was reported by 11.8% of patients, and 13.2% reported worrying about what was going to happen to them. One in 6 patients (16.3%) reported often or almost always not wanting others to see their scar. The most frequently reported problems with social functioning were getting teased (11.7%) and finding it hard to keep up with others (11.7%). Common school or cognitive problems were related to memory (15.3%), attention (12.6%), missing school to go to the doctor or hospital (12.4%), and trouble solving math problems (20%). One in 7 (13.5%) frequently found it difficult to

6 576 The Journal of Heart and Lung Transplantation, Vol 31, No 6, June 2012 Figure 1 Distribution of self-reported Pediatric Quality of Life Inventory (PedsQL) Psychosocial Summary scores in transplant recipients. The broken line is the cutoff score for at-risk status ( 1 standard deviation below the population mean). tell other (non-medical) people that they had a heart problem/ surgery. Correlates with QOL scores in transplant recipients There were no significant correlations between self-reported psychosocial QOL scores and age at assessment, age at transplant, or time since transplant. Psychosocial Health Summary scores were also not associated with patient sex, race/ethnicity, or SES. There was no difference in self-reported Psychosocial Health Summary scores in patients receiving cyclosporine (mean ) vs tacrolimus (mean ). Selfreported Physical Appearance scores were higher in patients taking cyclosporine (mean ) than in patients taking tacrolimus ( ; p 0.05). By proxy report, the Psychosocial Health Summary score was significantly correlated with a greater number of medications (r 0.174, p 0.02), but not by self-report (r 0.144, p 0.09). School was significantly correlated with Social, by both parent proxy-report (r 0.505, p 0.01) and self-report (r 0.540, p 0.01). High scores on the Heart Problems/Symptoms scale (fewer symptoms) were significantly correlated with higher Psychosocial scores by self-report (r 0.555, p 0.01) and parent proxy-report (r 0.490, p 0.01). In light of observed differences between the perceptions of children and their parents, intraclass correlations (ICC) were examined for each of the Core and Cardiac Module scales. ICCs are designated as 0.40, poor to fair agreement; 0.41 to 0.60, moderate agreement; 0.61 to 0.80, good agreement; and 0.81 to 1.00, excellent agreement. As reported in Table 4, most ICCs for the overall sample were in the moderate range, with the exception of treatment anxiety, where there was good agreement and communication with the lowest agreement (ICC 0.35). Discussion Although as a group pediatric heart transplant recipients reported significantly lower health-related QOL than healthy Table 4 Intraclass Correlation Coefficients between Pediatric Patient Self-Report and Parent Proxy Report for Pediatric Heart Transplant Sample PedsQL scale Parent-child agreement ICCs (years) Total sample Total Generic Score Physical Health Psychosocial Health Emotional Social School Symptoms/Treatment I Physical Appearance Treatment Anxiety Cognitive Problems Communication ICC, intraclass correlation coefficient. ICCs are designated as 0.40 poor to fair agreement, moderate agreement, good agreement, and excellent agreement.

7 Uzark et al. Quality of Life in Pediatric Heart Transplant 577 children, most seem to have a good QOL without significant impairment, as perceived by themselves and their parents. However, a significant minority, approximately 1 in 3 heart transplant recipients, perceived significantly impaired psychosocial functioning. This is consistent with previous reports of pathologic psychologic functioning (27%), 4 significant behavior problems (33%), and impaired social competence ( 50%), 2 and symptoms of psychologic distress in 20% to 24% of pediatric heart transplant recipients. 12 Compared with their peers who had undergone conventional cardiac surgery, heart transplant recipients perceived significantly fewer symptoms, especially in contrast to children with surgery for moderate to severe heart disease. Because an important goal of transplantation is not only to prolong life but also to relieve symptoms and allow the child to be as active as peers, the data suggest we have achieved that goal for most of these children. However, self-reported psychosocial QOL, including emotional and social functioning, was not significantly different from their peers after conventional cardiac surgery, especially those with moderate to severe disease. School functioning was also similar to children with moderate to severe disease. By parent report, psychosocial QOL was also worse in transplant recipients than reported by the cardiac surgery group with curative surgery and not different from peers with more severe disease. In one previous report that compared psychosocial outcome of 23 heart and 21 heart-lung transplant recipients with a group who underwent conventional cardiac surgery, the prevalence of psychiatric disorder in the non-transplant surgery group decreased from 26% to 6.5%, whereas 18.5% of the transplant recipients still had a psychiatric disorder 12 months after transplant, not significantly changed from 26% before transplant. 3 Data regarding the type or complexity of cardiac surgery is not provided by these investigators. In pediatric liver transplant recipients, health-related QOL was also lower than that reported for healthy children but similar to that for children with other chronic illnesses. 13 Some heart transplant recipients in our study identified specific concerns related to inability to do physical activities, finding it hard to keep up with peers and getting teased. In recent qualitative studies by Green et al, 14,15 the most important theme was the ability to do the activities other kids did, normal kid stuff. In their study, 5 of 11 heart transplant recipients reported being victims of teasing and bullying related to their scar or, more frequently, medication side effects such as hirsutism and gingival hyperplasia. 14 Interestingly we found no difference in perceived physical appearance between transplant and non-transplant surgery patients or between patients taking tacrolimus and patients taking cyclosporine, a drug with noted cosmetic side effects. Green et al 15 also described a common theme among adolescents was feeling resentful, consistent with the anger reported by heart transplant recipients in our study. Resentment about the medical regimen and being treated differently from others, defining them as not normal, resulted in attempts to hide their regimen from others, not wanting others to know. 15 Difficulty talking to others, a problem identified in our study, was previously found to be an important coping strategy associated with psychosocial adaptation. 16 Communication of feelings and fears to others may be especially important, as approximately 1 in 8 transplant recipients worried about what was going to happen to them, consistent with the findings of Brosig et al 17 describing concerns about organ rejection and life expectancy. Wray et al 18 also found that some heart transplant recipients were anxious about their health, and posttraumatic stress disorder has been reported in 16% of adolescent organ transplant recipients 19 and in nearly 40% of parents of pediatric heart transplant recipients. 20 School and cognitive problems observed in our study have been reported by others, including increased risk of developmental or cognitive deficits and increased absence from school Interestingly, the observed trouble solving math problems in our study is also consistent with objective measurement of arithmetic scores in heart transplant recipients reported by others. 2,17 Identification of developmental or cognitive deficits after transplant is important to design interventions to optimize school functioning. Psychosocial QOL was not significantly correlated with any sociodemographic variables, including age at transplant, time since transplant, or socioeconomic status in our study. The findings of Serrano-Ikkos et al 3 suggest that older age has a negative effect on long-term psychosocial functioning; however, psychosocial outcomes were not related to age or time since transplant according to other reports. 4,17,18 Wray et al 2 assessed heart transplant recipients at 12 months and 3 years after transplant and found stability in psychologic functioning over time. Although psychosocial QOL was significantly correlated with symptoms, symptoms were uncommon in our study. Previous reports in pediatric heart transplant recipients have not found medical factors or severity to be significantly correlated with psychologic functioning. 2,4 Post-transplant psychologic functioning, however, has been significantly correlated with family functioning, 4,16,24 which was not evaluated in the current study. Finally, the significant relationship between school functioning and social QOL further emphasizes the need for school interventions as well as avoidance of school absence as much as possible to optimize QOL outcomes after transplant. The study has some limitations. The healthy children from the PedsQL database and the non-transplant heart surgery sample from a single institution were not necessarily representative of the same patient population as the transplant patients. Study coordinators did not track information about the children who were deemed ineligible for participation in the study, but report that exclusions were infrequent and primarily because of neurocognitive deficits. Unfortunately, we did not collect information on pre-transplant diagnosis that might affect a child s perceptions after transplant, although the relevance of this variable is unclear because conflicting findings have been reported. 2,5,18,25 There was no pre-transplant assessment, and children were only assessed once at varied intervals after transplant. Other studies have shown improvement in physical and psychosocial functioning after transplant compared with before transplant. 3,18,26

8 578 The Journal of Heart and Lung Transplantation, Vol 31, No 6, June 2012 The strengths of our study include a larger sample size, inclusion of multiple transplant centers, and the use of a multidimensional, generic, and disease-specific validated instrument with normative data across a wide age span with both parent proxy and child self-reports. In summary, although pediatric heart transplant recipients may experience significant symptomatic improvement, they remain at risk for impaired psychosocial QOL similar to children with residual or palliated heart disease. Risk factors for poor psychosocial outcomes need to be identified. Immunosuppressive medications, the surgical scar, and the uncertainty of the future are unavoidable realities for the transplant recipient and can create stress for the entire family. The findings emphasize the importance of routine QOL assessment in these young patients to identify those at risk. The PedsQL, used in this study, is brief and easy to administer in the outpatient setting and includes both parent and child report. Review of the responses can guide counseling and referral. Avoidance of unnecessary restriction of physical and social activities by parents or others may help promote normalization for the child and adolescent after transplant, leading to better psychosocial QOL. Discussion of fears and strategies to help facilitate coping with the stresses of life after transplant, such as promoting peer communication or referral for psychologic counseling, may also prove beneficial. Future research is needed to identify risk factors leading to poor psychosocial outcomes and to evaluate the effect of interventions to improve QOL for pediatric heart transplant recipients. Disclosure statement Dr. Varni holds the copyright and the trademark for the PedsQL and receives financial compensation from the Mapi Research Trust, a non-profit research institute that charges distribution fees to for-profit companies that use the Pediatric Quality of Life Inventory. He did not receive compensation for the current project. None of the remaining authors has a financial relationship with a commercial entity that has an interest in the subject of the presented manuscript or other conflicts of interest to disclose. References 1. Kirk R, DK, Edwards LB, Aurora P, et al. Registry of the International Society for Heart and Lung Transplantation: thirteenth official pediatric heart transplantation report J Heart Lung Transplant 2010; 29: Wray J, Radley-Smith R. Longitudinal assessment of psychological functioning in children after heart or heart-lung transplantation. J Heart Lung Transplant 2006;25: Serrano-Ikkos E, Lask B, Whitehead B, Rees P, Graham P. Heart or heart-lung transplantation: psychosocial outcome. Pediatr Transplant 1999;3: DeMaso DR, Douglas Kelley S, Bastardi H, O Brien P, Blume ED. The longitudinal impact of psychological functioning, medical severity, and family functioning in pediatric heart transplantation. J Heart Lung Transplant 2004;23: DeMaso DR, Twente AW, Spratt EG, O Brien P. Impact of psychologic functioning, medical severity, and family functioning in pediatric heart transplantation. J Heart Lung Transplant 1995;14: Wray J, Long T, Radley-Smith R, Yacoub M. Returning to school after heart or heart-lung transplantation: how well do children adjust? Transplantation 2001;72: Uzark K, Jones K, Slusher J, Limbers C, Burwinkle TM, Varni JW. Quality of life in children with heart disease as perceived by children and parents. Pediatrics 2008;121:e Varni JW, Seid M, Kurtin PS. PedsQL 4.0: Reliability and validity of the Pediatric Quality of Life Inventory version 4.0 Generic Core Scales in healthy and patient populations. Med Care 2001;39: Varni JW, Burwinkle TM, Seid M, Skarr D. The PedsQL 4.0 as a pediatric population health measure: feasibility, reliability, and validity. Ambul Pediatr 2003;3: Varni JW, Seid M, Rode CA. The PedsQL: measurement model for the Pediatric Quality of Life Inventory. Med Care 1999;37: Uzark K, Jones K, Burwinkle TM, Varni, JW. The Pediatric Quality of Life Inventory in Children with Heart Disease. Prog Pediatr Cardiol 2003;18/2: Todaro JF, Fennell EB, Sears SF, Rodrigue JR, Roche AK. Review: cognitive and psychological outcomes in pediatric heart transplantation. J Ped Psych 2000:25: Bucuvalas JC, Britto M, Krug S, et al. Health-related quality of life in pediatric liver transplant reicpients: a single-center study. Liver Transpl 2003;9: Green A, McSweeney J, Ainley K, Bryant J. In my shoes: children s quality of life after transplantation. Prog Transpl 2007;17: Green A, Meaux J, Huett A, Ainley K. It has its ups and downs : adolescents quality of life after heart transplantation. Prog Transplant 2011;21: Uzark KC, Sauer S, Lawrence K, Miller J, Addonizio L, Crowley DC. The psychosocial impact of pediatric heart transplantation. J Heart Lung Transplant 1992;11: Brosig C, Hintermeyer M, Zlotocha J, Behrens D, Mao J. An exploratory study of the cognitive, academic, and behavioral functioning of pediatric cardiothoracic transplant recipients. Prog Transplant 2006; 16: Wray J, Radley-Smith R. Depression in pediatric patients before and 1 year after heart or heart-lung transplantation. J Heart Lung Transplant 2004:23: Mintzer LL, Stuber ML, Seacord D, Castaneda M, Mesrkhani V, Glover D. Traumatic stress symptoms in adolescent organ transplant recipients. Pediatrics 2005;115: Farley LM, DeMaso DR, D Angelo ED, et al. Parenting stress and parental post-traumatic stress disorder in families after pediatric heart transplantation. J Heart Lung Transplant 2007;26: Uzark K, Beebe D, Spicer R. Neurodevelopmental outcomes in pediatric heart transplant recipients. J Heart Lung Transplant 2009;28: Wray J, Radley-Smith, R. Developmental and behavioral status of infants and young children awaiting heart or heart-lung transplantation. Pediatrics 2004;113: Wray J, Pot-Mees C, Radley-Smith, Zeitlin H, Yacoub M. Cognitive function and behavioural status in paediatric heart and heart lung transplant recipients: the Harefield experience. BMJ 1994;309: Hirshfeld AB, Kahle AL, Clark BJ 3rd, Bridges ND. Parent-reported health status after pediatric thoracic organ transplant. J Heart Lung Transplant 2004;23: Nixon PA, Morris KA. Quality of life in pediatric heart, heart-lung, and lung transplant recipients. Int J Sports Med 2000;21(Suppl 2): S Wray J, Radley-Smith R. Prospective evaluation of pediatric heart and heart-lung recipients. J Pediatr Psychol 2007;32: