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Background: Neurocognitive impairment and quality of life are two important long-term challenges for patients with complex CHD. The impact of re-interventions during adolescence and young adulthood on neurocognition and quality of life is not well understood., Methods: In this prospective longitudinal multi-institutional study, patients 13-30 years old with severe CHD referred for surgical or transcatheter pulmonary valve replacement were enrolled. Clinical characteristics were collected, and executive function and quality of life were assessed prior to the planned pulmonary re-intervention. These results were compared to normative data and were compared between treatment strategies., Results: Among 68 patients enrolled from 2016 to 2020, a nearly equal proportion were referred for surgical and transcatheter pulmonary valve replacement (53% versus 47%). Tetralogy of Fallot was the most common diagnosis (59%) and pulmonary re-intervention indications included stenosis (25%), insufficiency (40%), and mixed disease (35%). There were no substantial differences between patients referred for surgical and transcatheter therapy. Executive functioning deficits were evident in 19-31% of patients and quality of life was universally lower compared to normative sample data. However, measures of executive function and quality of life did not differ between the surgical and transcatheter patients., Conclusion: In this patient group, impairments in neurocognitive function and quality of life are common and can be significant. Given similar baseline characteristics, comparing changes in neurocognitive outcomes and quality of life after surgical versus transcatheter pulmonary valve replacement will offer unique insights into how treatment approaches impact these important long-term patient outcomes.

Health-related quality of life (HRQOL) is an important clinical and research trial endpoint in adult heart failure and has been shown to predict mortality and hospitalizations in adult heart failure populations. HRQOL has not been adequately studied in the growing pediatric and young adult heart failure population. This study described HRQOL in adolescents and young adults (AYAs) with heart failure and examined primary disease, sex, race, and other correlates of HRQOL in this sample. Participants in this cross-sectional, single-center study included adolescent and young adults with heart failure and a parent/guardian. Patients and their parent/proxies completed the PedsQL, a well-established measure of HRQOL in pediatric chronic illness populations. HRQOL is impaired in AYAs with heart failure resulting from dilated, hypertrophic, or other cardiomyopathy, congenital heart disease, or post-transplant with rejection/complications. Patients identifying as white endorsed poorer total HRQOL than non-white patients (p = 0.002). Subscale analysis revealed significant correlations between female sex (p = 0.01) and white race (p = 0.01) with poorer self-reported physical functioning. Family income was unrelated to HRQOL. Functional status was strongly associated with total (p = 0.0003) and physical HRQOL (p < 0.0001). Sociodemographic and disease-specific risk and resilience factors specific to HRQOL in AYAs with heart failure include primary cardiac disease, race, sex, and functional status. Building upon extensive work in adult heart failure, utilization, and study of HRQOL as a clinical and research trial outcome is necessary in pediatric heart failure. Developing targeted interventions for those at greatest risk of impaired HRQOL is an important next step., (© 2022. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

Objectives: We previously reported common knowledge deficits and lack of transition readiness in 13- 25-year-olds with congenital or acquired heart disease. The aims of this study were to re-evaluate transition readiness in this cohort at follow-up and to examine the relationship between changes in transition readiness and quality of life (QOL)., Study Design: In this prospective cohort study, patients completed the Transition Readiness Assessment and the Pediatric Quality of Life Inventory using an e-tablet, web-based format at a routine follow-up visit. Changes from initial to follow-up scores were evaluated., Results: Sixty-five percent of patients (106 of 164) completed follow-up assessments at a median age of 18.7 years (IQR, 16.5-21.2 years) at a median follow-up of 1 year. The average perceived knowledge deficit score (percent of items with no knowledge) at follow-up was 18.0 ± 15.2%, which decreased from 24.7 ± 16.5% (P < .0001). On a 100-point scale, the mean score for self-efficacy increased from 71.4 ± 17.0 to 76.7 ± 18.2 (P = .0004) and for self-management increased from 47.9 ± 18.4 to 52.0 ± 20.7 (P = .004). Although physical QOL did not change, the mean psychosocial QOL score increased significantly (P = .02). A decrease in the knowledge deficit score at follow-up was significantly associated with an increased psychosocial QOL score (P = .03). An increase in the self-efficacy score was associated with an increase in psychosocial QOL score (P = .04), especially social QOL (P = .02)., Conclusions: Although deficits in knowledge and self-management skills persist, transition readiness assessment and recognition of deficits can improve transition readiness with improved psychosocial QOL., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Background: Infants who require extracorporeal membrane oxygenation (ECMO) support after a Norwood operation are at increased risk for early and late death compared with patients who do not require ECMO post-Norwood. Little is known about the effect that ECMO post-Norwood has on functional status and quality of life among long-term survivors., Methods: We prospectively evaluated functional status and health-related quality of life in 12 surviving patients (cases) and 19 corresponding patients (controls) from a previous retrospective case-control assessment of long-term survival in patients requiring ECMO post-Norwood. Functional status was assessed with the Vineland Adaptive Behavior Scale-II, and health-related quality of life was assessed with the Pediatric Quality of Life Inventory (PedsQL) core and cardiac modules., Results: There were no differences in demographics, extracardiac or genetic anomalies, or age at follow-up assessment between ECMO cases and non-ECMO controls. The Vineland Adaptive Behavior Scale-II scores were comparable between groups, with both groups demonstrating function in the normal range in all four domains tested. The only difference in PedsQL scores between cases and controls was perceived physical appearance, which was lower among ECMO survivors by both patient and proxy report. PedsQL scores of both groups were comparable to published scores for patients with single-ventricle congenital heart disease but generally lower than scores for the healthy population., Conclusions: The requirement for ECMO support after a Norwood operation does not appear to significantly affect functional status or quality of life among the subset of patients who achieve long-term survival., (Copyright © 2017 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: With improvements in early survival following congenital heart surgery, it has become increasingly important to understand longer-term outcomes; however, routine collection of these data is challenging and remains very limited. We describe the development and initial results of a collaborative programme incorporating standardised longitudinal follow-up into usual care at the Children's Hospital of Philadelphia (CHOP) and University of Michigan (UM)., Methods: We included children undergoing benchmark operations of the Society of Thoracic Surgeons. Considerations regarding personnel, patient/parent engagement, funding, regulatory issues, and annual data collection are described, and initial follow-up rates are reported., Results: The present analysis included 1737 eligible patients undergoing surgery at CHOP from January 2007 to December 2014 and 887 UM patients from January 2010 to December 2014. Overall, follow-up data, of any type, were obtained from 90.8% of patients at CHOP (median follow-up 4.3 years, 92.2% survival) and 98.3% at UM (median follow-up 2.8 years, 92.7% survival), with similar rates across operations and institutions. Most patients lost to follow-up at CHOP had undergone surgery before 2010. Standardised questionnaires assessing burden of disease/quality of life were completed by 80.2% (CHOP) and 78.4% (UM) via phone follow-up. In subsequent pilot testing of an automated e-mail system, 53.4% of eligible patients completed the follow-up questionnaire through this system., Conclusions: Standardised follow-up data can be obtained on the majority of children undergoing benchmark operations. Ongoing efforts to support automated electronic systems and integration with registry data may reduce resource needs, facilitate expansion across centres, and support multi-centre efforts to understand and improve long-term outcomes in this population.

Given the paucity of data on quality of life (QOL) after ventricular assist device (VAD) implant in children, we sought to describe self-reported QOL in children on VAD support and to identify potential factors associated with impaired QOL. There were 82 children (6-19 y) in the Pediatric Interagency Registry for Mechanical Circulatory Support who completed the generic Pediatric Quality of Life Inventory (PedsQL) +/- a VAD-specific QOL assessment pre-VAD implant (n=18), 3 months (m) post-VAD (n=62) and/or 6 m post VAD (n=38). Their QOL was compared to the general pediatric population, children with a chronic health condition, and children with severe/complex heart disease. Significantly impaired QOL is a score >1 SD below the pediatric population mean. The study group was 59% male, 67% Caucasian, with a primary diagnosis of cardiomyopathy in 82%, and a median age at 1st implant of 14 y (IQR 11-17). At 3 m and 6 m post-VAD, 63% and 81% were outpatient. PedsQL scores were lower than pediatric norms for physical (p<.0001) and psychosocial (p<.01) QOL in pre- and post-VAD groups. In comparison to chronic health condition and severe/complex heart disease groups, PedsQL scores were lower for physical and psychosocial QOL in the pre-VAD group (p<.0001), however psychosocial QOL was not significantly different in post-VAD groups. Physical QOL was significantly impaired in 89%, 71%, and 66% in pre-VAD, 3 m, and 6 m post-VAD groups, respectively. Psychosocial QOL was significantly impaired in 67%, 40%, and 24% in these 3 groups and psychosocial QOL scores were higher in the 3 m post VAD group compared to the pre-VAD group (p=.004). VAD patients were most bothered by their inability to participate in usual play activities. Univariate analysis revealed impaired QOL at 3 m was associated with inotropic support >2 weeks/ongoing post-VAD (p =.04), but was not associated with age at implant, primary diagnosis, hospital length of stay, adverse events or re-hospitalizations within 3 m post-implant, or with discharge status at 3 m post-VAD. Physical QOL is significantly impaired in the majority of patients pre- and post-VAD. However, psychosocial QOL is not significantly impaired in nearly 3/5 patients at 3 m post-VAD. While the study was limited by the small number of patients who had more than one assessment over time, group data suggest VAD implantation may improve psychosocial QOL. [ABSTRACT FROM AUTHOR]

In adult heart failure populations, health-related quality of life (HRQOL) is predictive of changes in health status and outcomes including mortality and hospitalization. Despite a growing population of pediatric and young adult patients with heart failure, HRQOL in this population has not been well described. This study aimed to describe self- and proxy-reported HRQOL in adolescents and young adults (AYA) with heart failure. AYAs (12-24 years) with heart failure or a significant post-heart transplant complication and a caregiver were enrolled at a single center. AYA/parent dyads completed a self- or proxy-reported version of the PedsQL 4.0 Generic Core. Group comparisons based on primary cardiac diagnosis leading to heart failure were conducted. Patient demographic and disease characteristics were examined as correlates of HRQOL. 53 patient-caregiver dyads participated. Patients were 64% male; 76% White. Post-transplant with life threatening complication (26%), single ventricle heart disease (SVHD) (23%), anthracycline induced cardiomyopathy (21%) and dilated cardiomyopathy (DCM) (17%) were the most common diagnoses. Disease burden was high with 42% experiencing extra-cardiac disease and 34% having >11 hospitalizations. AYAs with transplant complications, SVHD, and DCM and their caregivers endorsed significantly impaired total HRQOL when compared to population norms. Conversely, AYAs with anthracycline cardiomyopathy and their caregivers reported normative HRQOL that was significantly better than the other diagnostic groups (p=0.04). Across all diagnostic groups, female sex (p=0.01) and white race (p=0.01) were associated with poorer physical functioning HRQOL. Many AYAs with heart failure and their caregivers report impaired HRQOL, however certain cardiac diagnoses and demographic groups were associated with better reported HRQOL. Further studies are needed to understand mediators of risk for poor HRQOL in this population, as well as the role of potential resiliency focused interventions to improve HRQOL. [ABSTRACT FROM AUTHOR]