Baseline characteristics of patients
| Number of patients or mean (SD, range) | |
|---|---|
| 2-D, two dimensional; DILV, double inlet left ventricle; FEV1, forced expiratory volume in 1 s; INR, international normalised ratio; MELD, model of end-stage liver disease; MPA, main pulmonary artery; NYHA, New York Heart Association; SVEDP, systemic ventricular end-diastolic pressure; VC, vital capacity. | |
| Age (years) | 24.6 (8, 15.8–43.4) |
| Time since Fontan (years) | 14.1 (4.9, 6.9–26.4) |
| Sex (F/M) | 3/9 |
| Diagnosis: tricuspid atresia/DILV | 7/5 |
| Alcohol consumption: 0/100–250/>250 g/week | 7/4/1 |
| NYHA class: I/II/III/IV | 1/7/3/1 |
| Exercise tolerance test | |
| Workload (metabolic equivalents) | 6.5 (2.3, 4–11) |
| Maximum heart rate (% of predicted) | 66 (20, 35–104) |
| 2-D echocardiography | |
| Ventricular ejection fraction | 44 (8, 25–50) |
| Moderate to severe ventricular dysfunction | 2 |
| Pulmonary function test | |
| FEV1 (% of predicted) | 77 (14, 53–96) |
| VC (% of predicted) | 78 (19, 48–100) |
| Sustained arrhythmias on ECG: yes/no | 8/4 |
| Laboratory tests | |
| Haemoglobin (g/dl) | 13.4 (2.5, 9.7–15.5) |
| Platelet count (109/l) | 234 (177, 43–645) |
| Total bilirubin (μmol/l) | 24 (20, 7–79) |
| Alanine aminotransferase (IU/l) | 59 (125, 9–454) |
| Alkaline phosphatase (IU/l) | 261 (125, 126–567) |
| Total protein (g/l) | 66 (17, 37–88) |
| Albumin (g/l) | 33 (10, 16–46) |
| Creatinine (μmol/l) | 98 (32, 60–176) |
| INR | 1.2 (0.16, 1–1.5) |
| Factor V (U/dl) | 73 (19, 45–99) |
| α1-Antitrypsin clearance >0.47 mg/g faeces | 5 |
| MELD Score | 9.7 (4.7, 2–18) |
| Child–Turcotte–Pugh Classification Score | 5.7 (1.7, 3–9) |
| Haemodynamics | |
| Aortic saturations in air (%) | 94 (4, 87–100) |
| SVEDP (mm Hg) | 9 (5, 5–17) |
| Systolic aortic pressure (mm Hg) | 86 (16, 55–110) |
| Diastolic aortic pressure (mm Hg) | 51 (12, 35–70) |
| MPA pressure (mm Hg) | 15 (5, 9–28) |
| MPA saturation (%) | 66 (9, 52–81) |
| Fontan pressure (mm Hg) | 15 (5, 9–28) |
| Fontan saturations in air (%) | 68 (10, 47–82) |
| Hepatic vein pressure (mm Hg) | 15 (6, 9–30) |
| Hepatic vein saturation (%) | 52 (17, 14–76) |
| Hepatic vein wedge pressure (mm Hg) | 16 (6, 10–30) |
| Hepatic vein wedge saturation (%) | 59 (12, 38–75) |