Adult Fontan Patient Reported Mental and Physical Measures

Introduction: The Fontan is a palliative operation for patients with functionally single ventricles, arising from a heterogeneous group of heart defects. Though it provides a substantial mortality benefit, it is associated with significant morbidity and mortality with increasing age. The number of patients surviving to adulthood is increasing with an estimated incidence of 15-30/1 million. There is a considerable gap of evidence regarding reported physical and mental health for these patients.

Methods: We administered the PROMIS® Global Short Form (v 1.2) to Fontan patients in their scheduled clinic visits during 2017-2018. We also collected relevant demographic and background characteristics. Subsequently the raw PROMIS scores were converted to the standardized T-scores using HealthMeasures Scoring Services, where mean performance is 50 for the general population. We used Cronbach’s alpha to assess reliability, with >0.8 considered good.

Results: A total of 42 patients were included. Median age was 30, 57% were female. Median time from birth to operation was 4.5 years, was due to tricuspid atresia in 17 (40.5%) and double inlet left ventricle in 14 (33%), 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), and T-score for mental health was 53.3, higher than age-group mean (48.5, p<0.001). T-scores showed strong correlation with each other (r=0.7). Correlation of T-scores with age and time to procedure were weak. No association of neither diagnosis nor operation type with T-score was found.

Conclusion: Using patient reported measures can provide insight about patients with complex congenital heart disease. Fontan adult patients view their mental health better compared to the general age group, although their physical health is rated lower.