Generalized and specific anxiety in adolescents following heart transplant

Mental health concerns are associated with worse outcomes after adult heart transplant. Illness‐specific anxiety is associated with worsened psychological well‐being after other solid organ transplants but has never been characterized after pediatric heart transplant. This single‐center cross‐sectional study aimed to evaluate illness‐specific and generalized anxiety after heart transplantation in adolescents. A novel 12‐item PHTF, GAD‐7, and the PedsQL were administered. Univariate associations of demographics, clinical features, and medication adherence as measured by immunosuppression standard deviation with the PHTF and GAD‐7 scores were evaluated. Internal consistency and validity of the PHTF were examined. In total, 30 patients participated. The most common illness‐specific fears were retransplantation, rejection, and more generally post‐transplant complications. The PHTF had good internal consistency (Cronbach α = .88). Construct validity was demonstrated between PHTF and GAD‐7 (r = .62) and PedsQL (r = −.54 to −.62). 23% endorsed moderate to severe generalized anxiety symptoms. More severe symptoms were associated with older age at survey (P = .03), older age at listing (P = .01) and having post‐transplant complications (P = .004). Patients with moderate or severe symptoms were more likely to report late immunosuppression doses (P = .004). Illness‐specific and generalized anxiety may be prevalent after pediatric heart transplant. Screening for anxiety in adolescents post‐transplant may identify those at risk for adverse outcomes including non‐adherence. The PHTF is a brief, valid, and reliable instrument identifying illness‐specific anxiety in this population.Peer Reviewedhttps://deepblue.lib.umich.edu/bitstream/2027.42/153669/1/petr13647.pdfhttps://deepblue.lib.umich.edu/bitstream/2027.42/153669/2/petr13647_am.pd

Development of quality metrics for ambulatory pediatric cardiology: Transposition of the great arteries after arterial switch operation

ObjectiveTo develop quality metrics (QMs) for the ambulatory care of patients with transposition of the great arteries following arterial switch operation (TGA/ASO).DesignUnder the auspices of the American College of Cardiology Adult Congenital and Pediatric Cardiology (ACPC) Steering committee, the TGA/ASO team generated candidate QMs related to TGA/ASO ambulatory care. Candidate QMs were submitted to the ACPC Steering Committee and were reviewed for validity and feasibility using individual expert panel member scoring according to the RANDâ UCLA methodology. QMs were then made available for review by the entire ACC ACPC during an â open comment period.â Final approval of each QM was provided by a vote of the ACC ACPC Council.PatientsPatients with TGA who had undergone an ASO were included. Patients with complex transposition were excluded.ResultsTwelve candidate QMs were generated. Seven metrics passed the RANDâ UCLA process. Four passed the â open comment periodâ and were ultimately approved by the Council. These included: (1) at least 1 echocardiogram performed during the first year of life reporting on the function, aortic dimension, degree of neoaortic valve insufficiency, the patency of the systemic and pulmonary outflows, the patency of the branch pulmonary arteries and coronary arteries, (2) neurodevelopmental (ND) assessment after ASO; (3) lipid profile by age 11 years; and (4) documentation of a transition of care plan to an adult congenital heart disease (CHD) provider by 18 years of age.ConclusionsApplication of the RANDâ UCLA methodology and linkage of this methodology to the ACPC approval process led to successful generation of 4 QMs relevant to the care of TGA/ASO pediatric patients in the ambulatory setting. These metrics have now been incorporated into the ACPC Quality Network providing guidance for the care of TGA/ASO patients across 30 CHD centers.Peer Reviewedhttps://deepblue.lib.umich.edu/bitstream/2027.42/142334/1/chd12540_am.pdfhttps://deepblue.lib.umich.edu/bitstream/2027.42/142334/2/chd12540.pd

Reliability and validity of PedsQL for Portuguese children aged 5–7 and 8–12 years

BACKGROUND: Pediatric Quality of Life Inventory (PedsQL) is a measure to assess health-related quality of life (HRQoL) in children and adolescents. It is formed by 23 items adapted to children age and includes a parent proxy report version. With four multidimensional subscales and three summary scores, it measures health as defined by WHO. The concepts measured by this instrument are ‘physical functioning’ (8 items), ‘emotional functioning’ (5 items), ‘social functioning’ (5 items) and ‘school functioning’ (5 items). It also measures a ‘total scale score’ (23 items), a ‘physical health summary score’ (8 items) and a ‘psychosocial health summary score’ (15 items). The aim of this paper is to present the main results of the cultural adaptation and validation of the PedsQL into European Portuguese. METHODS: The Portuguese version was the result of a forward-backward translation process, with a cognitive debriefing analysis, guaranteeing face validity and semantic equivalence. Children aged 5–7 and 8–12 were randomly selected and were asked to fill a socio-demographic data survey and the Portuguese versions of PedsQL and KINDL, another HRQoL measure for children and adolescents. They were divided into three groups, healthy children, children with type I diabetes and children with spina bifida. The reliability was tested for reproducibility (ICC) and internal consistency (Cronbach’s alpha). The construct validity (known-groups discriminant validity) was supported by differences between self-reports from healthy children and children with chronic conditions, and from children with chronic diseases and their parents. The criterion validity was tested after the correlations of the scores obtained by both children and adolescents HRQoL assessment instruments. RESULTS: A total of 179 children and 97 parents were recruited. PedsQL demonstrated good levels of reproducibility (r > 0.95 in all versions) and acceptable levels of internal consistency with Cronbach’s alpha at 0.70 on most scales. Concordance values between children’s and parents’ perceptions ranged between 0.36 and 0.78 and the correlations with KINDL questionnaire were excellent, supporting concurrent validity. CONCLUSIONS: The Portuguese version of the PedsQL demonstrated acceptable psychometric properties for future research and clinical practice for children aged 5–12

Survival Data and Predictors of Functional Outcome an Average of 15 Years after the Fontan Procedure: The Pediatric Heart Network Fontan Cohort

ObjectiveMulticenter longitudinal outcome data for Fontan patients surviving into adulthood are lacking. The aim of this study was to better understand contemporary outcomes in Fontan survivors by collecting follow‐up data in a previously well‐characterized cohort.DesignBaseline data from the Fontan Cross‐Sectional Study (Fontan 1) were previously obtained in 546 Fontan survivors aged 11.9 ± 3.4 years. We assessed current transplant‐free survival status in all subjects 6.8 ± 0.4 years after the Fontan 1 study. Anatomic, clinical, and surgical data were collected along with socioeconomic status and access to health care.ResultsThirty subjects (5%) died or underwent transplantation since Fontan 1. Subjects with both an elevated (>21 pg/mL) brain natriuretic peptide and a low Child Health Questionnaire physical summary score (<44) measured at Fontan 1 were significantly more likely to die or undergo transplant than the remainder, with a hazard ratio of 6.2 (2.9–13.5). Among 516 Fontan survivors, 427 (83%) enrolled in this follow‐up study (Fontan 2) at 18.4 ± 3.4 years of age. Although mean scores on functional health status questionnaires were lower than the general population, individual scores were within the normal range in 78% and 88% of subjects for the Child Health Questionnaire physical and psychosocial summary score, and 97% and 91% for the SF‐36 physical and mental aggregate score, respectively. Since Fontan surgery, 119 (28%) had additional cardiac surgery; 55% of these (n = 66) in the interim between Fontan 1 and Fontan 2. A catheter intervention occurred in 242 (57%); 32% of these (n = 78) after Fontan 1. Arrhythmia requiring treatment developed in 118 (28%) after Fontan surgery; 58% of these (n = 68) since Fontan 1.ConclusionsWe found 95% interim transplant‐free survival for Fontan survivors over an average of 7 years of follow‐up. Continued longitudinal investigation into adulthood is necessary to better understand the determinants of long‐term outcomes and to improve functional health status.Peer Reviewedhttp://deepblue.lib.umich.edu/bitstream/2027.42/110738/1/chd12193.pd

Impaired health-related quality of life in children and adolescents with chronic conditions: a comparative analysis of 10 disease clusters and 33 disease categories/severities utilizing the PedsQL™ 4.0 Generic Core Scales

<p>Abstract</p> <p>Background</p> <p>Advances in biomedical science and technology have resulted in dramatic improvements in the healthcare of pediatric chronic conditions. With enhanced survival, health-related quality of life (HRQOL) issues have become more salient. The objectives of this study were to compare generic HRQOL across ten chronic disease clusters and 33 disease categories/severities from the perspectives of patients and parents. Comparisons were also benchmarked with healthy children data.</p> <p>Methods</p> <p>The analyses were based on over 2,500 pediatric patients from 10 physician-diagnosed disease clusters and 33 disease categories/severities and over 9,500 healthy children utilizing the PedsQL™ 4.0 Generic Core Scales. Patients were recruited from general pediatric clinics, subspecialty clinics, and hospitals.</p> <p>Results</p> <p>Pediatric patients with diabetes, gastrointestinal conditions, cardiac conditions, asthma, obesity, end stage renal disease, psychiatric disorders, cancer, rheumatologic conditions, and cerebral palsy self-reported progressively more impaired overall HRQOL than healthy children, respectively, with medium to large effect sizes. Patients with cerebral palsy self-reported the most impaired HRQOL, while patients with diabetes self-reported the best HRQOL. Parent proxy-reports generally paralleled patient self-report, with several notable differences.</p> <p>Conclusion</p> <p>The results demonstrate differential effects of pediatric chronic conditions on patient HRQOL across diseases clusters, categories, and severities utilizing the PedsQL™ 4.0 Generic Core Scales from the perspectives of pediatric patients and parents. The data contained within this study represents a larger and more diverse population of pediatric patients with chronic conditions than previously reported in the extant literature. The findings contribute important information on the differential effects of pediatric chronic conditions on generic HRQOL from the perspectives of children and parents utilizing the PedsQL™ 4.0 Generic Core Scales. These findings with the PedsQL™ have clinical implications for the healthcare services provided for children with chronic health conditions. Given the degree of reported impairment based on PedsQL™ scores across different pediatric chronic conditions, the need for more efficacious targeted treatments for those pediatric patients with more severely impaired HRQOL is clearly and urgently indicated.</p

Central Nervous System Changes in Pediatric Heart Failure: A Volumetric Study

Autonomic dysfunction, mood disturbances, and memory deficits appear in pediatric and adult heart failure (HF). Brain areas controlling these functions show injury in adult HF patients, many of whom have comorbid cerebrovascular disease. We examined whether similar brain pathology develops in pediatric subjects without such comorbidities. In this study, high-resolution T1 brain magnetic resonance images were collected from seven severe HF subjects age (age 8–18 years [mean 13]; left ventricular shortening 9 to 19% [median 14%]) and seven age-matched healthy controls (age 8–18 years [mean 13]). After segmentation into gray matter (GM), white matter, and cerebrospinal fluid (CSF), regional volume loss between groups was determined by voxel-based morphometry. GM volume loss appeared on all HF scans, but ischemic changes and infarcts were absent. HF subjects showed greater CSF volume than controls (mean ± SD 0.30 ± 0.04 vs. 0.25 ± 0.04 l, P = 0.03), but total intracranial volume was identical (1.39 ± 0.11 vs. 1.39 ± 0.09 l, P = NS). Regional GM volume reduction appeared in the right and left posterior hippocampus, bilateral mid-insulae, and the superior medial frontal gyrus and mid-cingulate cortex of HF subjects (threshold P < 0.001). No volume-loss sites appeared in control brains. We conclude that pediatric HF patients show brain GM loss in areas similar to those of adult HF subjects. Substantial changes emerged in sites that regulate autonomic function as well as mood, personality and short-term memory. In the absence of thromboembolic disease and many comorbid conditions found in adult HF patients, pediatric HF patients show significant, focal GM volume loss, which may coincide with the multiple neurologic and psychological changes observed in patients with HF

Self-reported health experiences of children living with congenital heart defects: Including patient-reported outcomes in a national cohort study

Background: Understanding children’s views about living with congenital heart defects (CHDs) is fundamental to supporting their successful participation in daily life, school and peer relationships. As an adjunct to a health and quality of life outcomes questionnaire, we asked school-age children who survived infant heart procedures to describe their experiences of living with CHDs. Methods: In a UK-wide cohort study, children aged 10 to 14 years with CHDs self-completed postal questionnaires that included an open question about having a ‘heart problem’. We compared the characteristics of children with more and less severe cardiac diagnoses and, through collaborative inductive content analysis, investigated the subjective experiences and coping strategies described by children in both clinical severity groups. Results: Text and/or drawings were returned by 436 children (246 boys [56%], mean age 12.1 years [SD 1.0; range 10–14]); 313 had less severe (LS) and 123 more severe (MS) cardiac diagnoses. At the most recent hospital visit, a higher proportion of the MS group were underweight (more than two standard deviations below the mean for age) or cyanosed (underweight: MS 20.0%, LS 9.9%; cyanosed: MS 26.2%, LS 3.5%). Children in the MS group described concerns about social isolation and feeling ‘different’, whereas children with less severe diagnoses often characterised their CHD as ‘not a big thing’. Some coping strategies were common to both severity groups, including managing health information to avoid social exclusion, however only children in the LS group considered their CHD ‘in the past’ or experienced a sense of survivorship. Conclusions: Children’s reported experiences were not dependent on their cardiac diagnosis, although there were clear qualitative differences by clinical severity group. Children’s concerns emphasised social participation and our findings imply a need to shift the clinical focus from monitoring cardiac function to optimising participation. We highlight the potential for informing and evaluating clinical practice and service provision through seeking patient-reported outcomes in paediatric care

Measuring quality of life in Duchenne muscular dystrophy : a systematic review of the content and structural validity of commonly used instruments

Duchenne muscular dystrophy (DMD) is an inherited X-linked neuromuscular disorder. A number of questionnaires are available to assess quality of life in DMD, but there are concerns about their validity. This systematic review aimed to appraise critically the content and structural validity of quality of life instruments for DMD. Five databases (EMBASE, MEDLINE, CINAHL, PsycINFO, and Cochrane Library) were searched, with supplementary searches in Google Scholar. We included articles with evidence on the content and/or structural validity of quality of life instruments in DMD, and/or instrument development. Evidence was evaluated against the Consensus-based Standards for the selection of health Measurement INstruments (COSMIN) criteria. Fifty five articles featured a questionnaire assessing quality of life in DMD. Forty instruments were extracted and 26 underwent assessment. Forty-one articles contained evidence on content or structural validity (including 37 development papers). Most instruments demonstrated low quality evidence and unsatisfactory or inconsistent validity in DMD, with the majority not featuring direct validation studies in this population. Only KIDSCREEN received an adequate rating for instrument design and a satisfactory result for content validity based on its development, yet, like the majority of PROMs, the measure has not been directly validated for use in DMD. Further research is needed on the validity of quality of life instruments in DMD, including content and structural validity studies in this population