Table 2: Baseline and 1-year data on Fontan patients after enrollment.
2a: Clinical events observed in stable Fontan patients within 1 year of serum extraction for biomarkers analysis.
2b: Laboratory and echocardiography profile of stable SV Fontan patients at baseline and 1 year follow up after drawing serum for biomarker levels.
2c: Baseline and 1-year MELD-XI scores in Fontan patients. Corresponding creatinine and bilirubin levels in patients with an increase and stable/unchanged MELD-XI scores are also noted.
| Average Time for Clinic Event | 155 ± 122 days | |
|---|---|---|
| Clinical Events | n | % (out of 44) |
| Hospitalizations | 8 | 18.2% |
| Cardiac Causes | 3 | 6.8% |
| Non-cardiac causes | 5 | 11.4% |
| Increase in MELDXI* score by ≥ 1 | 13 | 29.5% |
| Diagnosis of Hepatocellular Carcinoma | 2 | 4.5% |
| Arrhythmias requiring treatment | 9 | 20.5% |
| Defibrillation | 0 | 0.0% |
| Cardioversion | 6 | 13.6% |
| Ablation | 1 | 2.3% |
| Event rate# | ||
| No Events | 22 | 50.0% |
| Any Events | 22 | 50.0% |
| 1 2 >2 |
10 7 5 |
45.5% 31.8% 22.7% |
| Echocardiography Parameters | |||
|---|---|---|---|
| Parameter | # Patients | Change in # Patients | |
| Systemic Ventricular Function, qualitative (n=35) | |||
| Normal | 24 (68.6%) | +2 | |
| Mildly Depressed | 3 (8.6%) | +1 | |
| Moderate or severely depressed | 8 (22.9%) | 0 | |
| Systemic Valve Regurgitation (n=35) | |||
| None-Trace | 7 (20.0%) | −1 | |
| Mild | 15 (42.9%) | +2 | |
| Moderate | 12 (34.3%) | 0 | |
| Severe | 1 (2.9%) | +1 | |
| Serum Chemistry Laboratory Values | |||
| Parameter | Average at 1 yr (%) | Change from Baseline | P value |
| Creatinine | 0.95±0.66 | +0.014±0.14 | 0.50 |
| Total Bilirubin | 1.1±0.6 | 0.0±0.5 | 0.76 |
| Aspartate Aminotransferase (AST) | 24.6±6.5 | −3.5±8.4 | 0.03 |
| Alanine Aminotransferase (ALT) | 23.3±11.2 | −4.3±11.9 | 0.27 |
| Alkaline Phosphatase | 74.2±23.0 | 4.5±13.9 | 0.31 |
| Albumin | 4.5±0.6 | 0.1±0.3 | 0.27 |
| Platelets | 174.02±65.05 | −8.0±49.51 | 0.76 |
| n | Baseline MELD XI | MELD XI at 1 yr*** | Change in Bilirubin | Change in Creatinine | |
|---|---|---|---|---|---|
| Patients with increase in MELD XI Score | 13 | 8.5 ± 1.2 | 10.0 ± 1.4* | 0.48 ± 0.36# | 0.10 ± 0.10$ |
| Patients with decrease or unchanged MELD XI Score | 31 | 9.8 ± 2.1 | 9.1 ± 2.1** | −0.24 ± 0.38## | −0.03 ± 0.14$ $ |
MELD score is calculated using the equation MELD-XI = 5.11 × ln (serum bilirubin in mg/dL) ± 11.76 × ln (serum creatinine in mg/dL) ± 9.44.
Events include cardiac hospitalization, non-cardiac hospitalization, presence off arrhythmia requiring intervention, increase in MELDXI score of ≥1, diagnosis of hepatocellular carcinoma.
p=0.0002,
p=0.003,
p=0.03,
p=0.04,
p=0.0006,
p=0.21,
MELD XI was remeasured at 428.4 ± 97.0 days.