Introduction: The Fontan procedure is a palliative operation for patients with single functional ventricles, arising from a heterogeneous group of heart defects. There is a considerable gap in evidence regarding the self-reported physical and mental health of these patients surviving to adulthood. Methods and Results: We administered the PROMIS® Global Short Form (v 1.2) to Fontan patients during their scheduled clinic visits during 2017−2018. The raw PROMIS scores were subsequently converted to standardized T-scores, where the mean performance was 50 for the general population. We used Cronbach’s alpha to assess reliability, with >0.8 considered good. A total of 42 patients were included. The median age was 30 (IQR: 24−34) years and 59% (95% CI: 43−74%) were female. The median time from birth to operation was 4.5 (IQR: 3−8) years, with 55% having an extracardiac Fontan. The questionnaire had good internal reliability with an alpha of 0.87. Seventy-one percent of respondents rated their overall health as “excellent” or “good”. The mean T-score for physical health was 46.6, lower than the age-group mean (51.6, p < 0.001). The mean T-score for mental health was 53.3, higher than the age-group mean (48.5, p < 0.001). T-scores showed strong correlation with each other (r = 0.7) and weak correlation with age and time from procedure. There was no association of T-score with diagnosis or operation type. Conclusions: Adult Fontan patients report better mental health despite worse reporting physical health compared with the age group means. Patient-reported measures can provide clinically meaningful insights about the care of patients with complex congenital heart disease.
Keywords: Fontan; adult congenital heart disease; patient-reported outcomes.