Primary Disease, Sex, and Racial Differences in Health-Related Quality of Life in Adolescents and Young Adults with Heart Failure.

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.

Impact of the COVID-19 pandemic on CHD care and emotional wellbeing.

BACKGROUND: Understanding the impact of the COVID-19 pandemic on paediatric non-COVID-19-related care, as well as patient and caregiver concerns and stressors, is critical for informing healthcare delivery. It was hypothesised that high care disruptions and psychological stress would be observed among paediatric and adult CHD patients in the early phase of the pandemic. METHODS: A cross-sectional, international, electronic survey study was completed. Eligible participants included parents of children with acquired or CHD, adults with CHD, or caregivers of adults with CHD. RESULTS: A total of 1220 participants from 25 countries completed the survey from 16 April to 4 May, 2020. Cardiac care disruption was significant with 38% reporting delays in pre-pandemic scheduled cardiac surgeries and 46% experiencing postponed cardiac clinic visits. The majority of respondents (75%) endorsed moderate to high concern about the patient with heart disease becoming ill from COVID-19. Worry about returning for in-person care was significantly greater than worry of harm to patient due to postponed care. Clinically significant psychological stress was high across the sample including children (50%), adults with CHD (42%), and caregivers (42%). CONCLUSIONS: The early phase of the COVID-19 pandemic contributed to considerable disruptions in cardiac care for patients with paediatric and adult CHD. COVID-19-related fears are notable with potential to impact willingness to return to in-person care. Psychological stress is also very high necessitating intervention. Further study of the impact of delays in care on clinical outcomes is warranted.

Design and initial results of a programme for routine standardised longitudinal follow-up after congenital heart surgery.

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.