In Fontan patients, maximal aerobic capacity is reduced compared with healthy subjects, with better preservation of submaximal performance. Higher O(2) pulse is associated with better exercise performance, whereas adolescence and male gender are associated with decreased performance compared with healthy subjects.

Background Multicenter longitudinal objective data for Fontan patients surviving into adulthood are lacking. Objectives Describe transplant-free survival and explore relationships between laboratory measures of ventricular performance and functional status over time. Methods Exercise testing, echocardiography, B-type natriuretic peptide (BNP), functional health assessment, and medical history abstraction were repeated 9.4 ± 0.4 years after the Fontan Cross-Sectional Study (Fontan 1) compared to previous values. Cox regression analysis explored risk factors for interim death or cardiac transplantation. Results From the original cohort of 546 subjects, 466 were recontacted and 373 (80%) were enrolled at 21.2 ± 3.5 years of age. Among subjects with paired testing, percent predicted maximum VO2 decreased (69 ± 14 vs. 61 ± 16, p <0.001, n = 95), ejection fraction decreased (58 ± 11 vs. 55 ± 10, p <0.001, n=259), and BNP increased (Median (IQR) 13 (7,25) vs. 18 (9,36) pg/mol, p <0.001, n = 340). At latest follow-up lower Pediatric Quality of Life Inventory (PedsQL) physical summary score was associated with poorer exercise performance (R2 adjusted = 0.20, p <0.001, n = 274). Cumulative complications since the Fontan included additional cardiac surgery (32%), catheter intervention (62%), arrhythmia treatment (32%), thrombosis (12%), and protein losing enteropathy (8%). Since Fontan 1, 54 subjects (10%) have received a heart transplant (n = 23) or died without transplantation (n = 31). The interval risk of death/transplantation was associated with poorer ventricular performance and functional health status assessed at Fontan 1, but was not associated with ventricular morphology, subject age or type of Fontan connection. Conclusions Interim transplant-free survival over 12 years in this Fontan cohort was 90% and was independent of ventricular morphology. Exercise performance decreased and was associated with worse functional health status. Future interventions might focus on preserving exercise capacity. (Clinical Trials Registration #: NCT00132782)

(1990-2008 Nϭ3375) for cases of RCM. Cases were defined as pure when RCM was the only assigned diagnosis. Additional documentation of HCM at any time was used as the criterion for RCM/HCM phenotype. RCM accounted for 4.5% of cases of cardiomyopathy. In 101 (66%), pure RCM was diagnosed; in 51 (34%), there was a mixed phenotype. Age at diagnosis was not different between groups, but 10% of the pure RCM group was diagnosed in infancy versus 24% of the RCM/HCM group. Freedom from death was comparable between groups with 1-, 2-, and 5-year survival of RCM 82%, 80%, and 68% versus RCM/HCM 77%, 74%, and 68%. Transplant-free survival was 48%, 34%, and 22% and 65%, 53%, and 43%, respectively (Pϭ0.011). Independent risk factors at diagnosis for lower transplant-free survival were heart failure (hazard ratio 2.20, Pϭ0.005), lower fractional shortening z score (hazard ratio 1.12 per 1 SD decrease in z score, Pϭ0.014), and higher posterior wall thickness in the RCM/HCM group only (hazard ratio 1.32, PϽ0.001). Overall, outcomes were worse than for all other forms of cardiomyopathy. Conclusions-Transplant-free survival is poor for RCM in childhood. Survival is independent of phenotype; however, the RCM/HCM phenotype has significantly better transplant-free survival. Clinical Trials Registration-URL: http://www.clinicaltrials.gov. Unique Identifier: NCT00005391.