Prognostic power of cardiopulmonary exercise testing in Fontan patients: a systematic review

OBJECTIVE: Exercise impairment is common in Fontan patients. Our aim is to systematically review previous literature to determine the prognostic value of exercise capacity in older adolescent and adult Fontan patients with respect to late outcome. Additionally, we reviewed the determinants of exercise capacity in Fontan patients and changes in exercise capacity over time. METHODS: PubMed, CINAHL, Embase, The Cochrane Library and Scopus were searched systematically for studies reporting exercise capacity and late outcome such as mortality, cardiac transplantation and hospitalisation. Studies were eligible for inclusion if more than 30 patients were included and mean age was ≥16 years. RESULTS: Four thousand and seven hundred and twenty-two studies were identified by the systematic search. Seven studies fulfilled the inclusion and exclusion criteria. The total number of patients was 1664 adult Fontan patients. There were 149 deaths and 35 heart transplantations. All eligible studies were retrospective cohort studies. The correlation between exercise capacity and late outcome was identified, and HRs were reported. CONCLUSION: In Fontan patients, the best predictors of death and transplantation were a decline in peak VO(2), heart rate variables and exercise oscillatory ventilation. Peak VO(2) was not strongly predictive of mortality or hospitalisation in Fontan patients. Several variables were strong and independent predictors of hospitalisation and morbidity.