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.