Table 2

Patient characteristics

CharacteristicsParticipants (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 Wales49 (67.1%)<0.030
Victoria24 (32.9%)
Diagnosis, n (%)Aortic Atresia1 (1.4%)
CAVC9 (12.3%)
ccTGA8 (11.0%)
DILV17 (23.3%)
DORV6 (8.2%)
HLHS5 (6.8%)
Left-AV-valve-atresia1 (1.4%)
PA-IVS5 (6.8%)
PA-VSD2 (2.7%)
TA18 (24.7%)
VSD1 (1.4%)
Fontan type, n (%)AP9 (12.3%)<0.001
ECC50 (68.5%)
LT14 (19.2%)
NYHA-classification, n (%)Class 157 (78.1%)
Class 213 (17.8%)
Class 33 (4.1%)
Class 40 (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-scan17.53 (1.04)
Fibro-test score0.49 (0.19)
MELD-XI score3.78 (4.85)
Events, n (%)No events62 (84.93%)
Thrombotic events3 (4.11%)
Major bleeding events2 (2.74%)
Minor bleeding events3 (4.11%)
Missing3 (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.