Health-related quality-of-life for people with a Fontan circulation, their parents, and siblings

AIMS: The Fontan procedure has revolutionized care for individuals born with single-ventricle heart disease, with over 80% of patients surviving into adulthood. This study aimed to estimate the long-term health-related quality-of-life (HRQoL) experienced by patients and families. METHODS AND RESULTS: Patients, parents, and siblings were recruited through the Australian and New Zealand Fontan Registry and self-reported their HRQoL the Child Health Utility 9D index for children and EuroQol 5-Dimensions for adults. Responses included 125 patients, 69 siblings, and 144 parents. Child patients reported more sadness, challenges with school and homework, and problems joining in activities, resulting in a lower HRQoL summary score compared with the general child population (0.74 vs. 0.80, P = 0.03). Adult patients reported more problems with anxiety and depression, usual activities, and self-care. While 60% of adult patients reported a summary score comparable with the general population, the 40% of adult patients who perceived their heart condition as serious reported lower HRQoL (0.77 vs. 0.90, P = 0.01). Child siblings experienced more tiredness and annoyance, while adult siblings reported more anxiety and/or depression; however, total HRQoL summary scores did not differ from the general population. Parents' HRQoL was comparable with the general population. CONCLUSION: Overall, child patients, and adult patients who perceived their heart condition as serious, reported lower HRQoL than the general population. Siblings reported concerns in specific HRQoL domains. The reported utility scores can serve as references for the Fontan population and their family members, as well as formal inputs for economic evaluations of interventions to assist with resource allocation decisions.