Table 1

Baseline characteristics of the study population

CharacteristicsAll Fontan (n=96)
Male52 (53%)
Age at initial MELD-XI score (years)26 (range 9–51)
Primary congenital defect
 Tricuspid atresia33 (34%)
 DILV22 (23%)
 HLHS1 (1%)
 Other40 (42%)
Systemic ventricle
 Morphologically left67 (70%)
 Morphologically right18 (19%)
 Biventricular11 (11%)
Type of Fontan surgery
 Lateral tunnel40 (42%)
 RA-PA44 (45%)
 RA-RV9 (9%)
 Extracardiac3 (4%)
Medications at the time of MELD-XI
 Digoxin59 (63%)
 Diuretics55 (58%)
 RAS inhibitor65 (70%)
 Warfarin/ASA89 (93%)
 β-blocker24 (27%)
Hepatitis serology
 Negative21 (22%)
 HCV/HBV8 (8%)
 Undetermined67 (70%)
History of*:
 PLE10 (11%)
 Pulmonary arterial thrombosis/embolism2 (2%)
 Thrombus in the Fontan pathway23 (24%)
 Non-fatal stroke10 (11%)
Pre-Fontan haemodynamics:
 CI (L/min/m2)2.85±1.3
 Systemic venous pressure (mm Hg)13±6.3
Fontan haemodynamics (n=50, see text for details)
 CI (L/min/m2)2.4±0.7
 Fontan pathway pressure (mm Hg)17±6
 Capillary pulmonary wedge pressure (mm Hg)11±5
 Pulmonary vascular resistance (iWU)2.2±1.4
  • *Complication of Fontan circulation occurred before first MELD-XI score measurement.

  • DILV, double inlet left ventricle; HBV, hepatitis B virus; HCV, hepatitis C virus; HLHS, hypoplastic left heart syndrome; iWU, indexed Wood Unit; MELD-XI, Model for End-stage Liver Disease eXcluding INR; PA, pulmonary artery; PLE, protein-losing enteropathy; RA, right atrium; RAS, renin-angiotensin system; RV, right ventricle.