Baseline characteristics of the study population
| Characteristics | All Fontan (n=96) |
|---|---|
| Male | 52 (53%) |
| Age at initial MELD-XI score (years) | 26 (range 9–51) |
| Primary congenital defect | |
| Tricuspid atresia | 33 (34%) |
| DILV | 22 (23%) |
| HLHS | 1 (1%) |
| Other | 40 (42%) |
| Systemic ventricle | |
| Morphologically left | 67 (70%) |
| Morphologically right | 18 (19%) |
| Biventricular | 11 (11%) |
| Type of Fontan surgery | |
| Lateral tunnel | 40 (42%) |
| RA-PA | 44 (45%) |
| RA-RV | 9 (9%) |
| Extracardiac | 3 (4%) |
| Medications at the time of MELD-XI | |
| Digoxin | 59 (63%) |
| Diuretics | 55 (58%) |
| RAS inhibitor | 65 (70%) |
| Warfarin/ASA | 89 (93%) |
| β-blocker | 24 (27%) |
| Hepatitis serology | |
| Negative | 21 (22%) |
| HCV/HBV | 8 (8%) |
| Undetermined | 67 (70%) |
| History of*: | |
| PLE | 10 (11%) |
| Pulmonary arterial thrombosis/embolism | 2 (2%) |
| Thrombus in the Fontan pathway | 23 (24%) |
| Non-fatal stroke | 10 (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.