Patient characteristics
Characteristics | Participants (n=73) | P value | |
Age in years, median (range), mean (SD) | 17.62 (4.83–50.24) | 18.9 −8.46 | |
Years post-Fontan, median (range), mean (SD) | 12.93 (0.33–30.13) | 13.52 −6.98 | |
Duration of follow-up in years, mean (SD) | 3.72 (0.61) | ||
Pacemaker present, n (%) | 5 (6.8%) | ||
Male, n (%) | 38 (52.1%) | 0.725 | |
Site of inclusion, n (%) | New South Wales | 49 (67.1%) | <0.030 |
Victoria | 24 (32.9%) | ||
Diagnosis, n (%) | Aortic Atresia | 1 (1.4%) | |
CAVC | 9 (12.3%) | ||
ccTGA | 8 (11.0%) | ||
DILV | 17 (23.3%) | ||
DORV | 6 (8.2%) | ||
HLHS | 5 (6.8%) | ||
Left-AV-valve-atresia | 1 (1.4%) | ||
PA-IVS | 5 (6.8%) | ||
PA-VSD | 2 (2.7%) | ||
TA | 18 (24.7%) | ||
VSD | 1 (1.4%) | ||
Fontan type, n (%) | AP | 9 (12.3%) | <0.001 |
ECC | 50 (68.5%) | ||
LT | 14 (19.2%) | ||
NYHA-classification, n (%) | Class 1 | 57 (78.1%) | |
Class 2 | 13 (17.8%) | ||
Class 3 | 3 (4.1%) | ||
Class 4 | 0 (0%) | ||
Liver panel as percentage of upper limit, mean (SD) | Total bilirubin (%) | 110.00 (74) | |
AST (%) | 82.00 (25) | ||
ALT (%) | 91.00 (30) | ||
GGT (%) | 117.00 (71) | ||
ALP (%) | 53.00 (28) | ||
Severity of liver disease, mean (SD) | Fibro-scan | 17.53 (1.04) | |
Fibro-test score | 0.49 (0.19) | ||
MELD-XI score | 3.78 (4.85) | ||
Events, n (%) | No events | 62 (84.93%) | |
Thrombotic events | 3 (4.11%) | ||
Major bleeding events | 2 (2.74%) | ||
Minor bleeding events | 3 (4.11%) | ||
Missing | 3 (4.11%) |
ALP, alkaline phosphatase; ALT, alanine aminotransferase; AP, atriopulmonary connection; AST, aspartate aminotransferase; AV, atrioventricular; CAVC, complete atrioventricular canal defect; ccTGA, congenitally corrected transposition of the great arteries; DILV, double inlet left ventricle; DORV, double outlet right ventricle; ECC, extracardiac conduit; GGT, gamma-glutamyl transferase; HLHS, hypoplastic left heart syndrome; LT, lateral tunnel; MELD-XI, model for end-stage liver disease excluding international normalised ratio; NYHA, New York Heart Association; PA-IVS, pulmonary atresia with intactventricular septum; PA-VSD, pulmonary atresia with ventricular septal defect; TA, tricuspid atresia; VSD, ventricular septal defect.