Abstract 12767: Trajectory of Exercise Capacity in Childhood and Its Impact on Their Adult Pathophysiology in Patients After the Fontan Operation

Abstract

Background: Preserved exercise capacity in childhood may predict better long-term outcome in patients after the Fontan operation. Purpose: This study was to elucidate an impact of trajectory of exercise capacity in childhood on the long-term exercise capacity and non-cardiac multi-organ function. Method and Results: Since 1990, consecutive 197 Fontan patients had undergone two serial cardiopulmonary exercise testing (CPX) at the age of 8±2 and 14±2 years and we measured the change in %-predicted peak oxygen uptake (dPVO 2 : %). Of these, 94 patients had performed CPX in the adulthood (24±4 years). Our patients were subdivided into 2 groups; those with positive dPVO 2 (n=113, group I) and those with negative dPVO 2 (n=84, group D). Early era at operation, heterotaxy, protein losing enteropathy, high PVO 2 and plasma brain natriuretic peptide levels (BNP) at 1 st CPX had independent negative impact on dPVO 2 (p<0.05-0.0001). Group I showed significantly higher PVO 2 and lower BNP in their adulthood, while 49 clinical events, including 9 deaths occurred after 2 nd CPX. When compared with group I, group D had high hazard ratio of 2.1 and 6.5 for morbidity and mortality, respectively (p<0.05 for both); however, there were no differences in high prevalence of impaired glucose tolerance, liver MELD XI score, or 24-hour creatinine clearance between two groups in their adulthood. Conclusions: The trajectory of exercise capacity in childhood is improving in current Fontan children which is associated with better prognosis, higher PVO 2 and lower BNP in the adulthood. However, the non-cardiac multi-organ dysfunction may progress regardless of the trajectory.