Table 1.
Characteristics of the patients
| Variables | All patients | Hyper- bilirubinemia |
Normo- bilirubinemia |
p Value |
|---|---|---|---|---|
| n = 37 | n = 6 | n = 31 | ||
| Age, yrs | 49 ± 18 | 53 ± 15 | 48 ± 19 | 0.62 |
| Women | 29 (78) | 1 | 7 | 0.75 |
| Height, cm | 153 ± 20 | 152 ± 7 | 153 ± 19 | 0.36 |
| Body weight, kg | 50 ± 10 | 43 ± 10 | 51 ± 9 | 0.04 |
| Systolic blood pressure, mm Hg | 113 ± 20 | 104 ± 12 | 114 ± 22 | 0.26 |
| Diastolic blood pressure, mm Hg | 70 ± 14 | 61 ± 11 | 71 ± 14 | 0.10 |
| Heart rate, beats per minute | 88 ± 14 | 95 ± 8 | 86 ± 15 | 0.18 |
| WHO Functional class II/III/IV | 15/12/10 (41/32/27) | 1/0/5 | 14/12/5 | 0.003 |
| Idiopathic/CTD-associated | 18/19 (49/51) | 4/2 | 14/17 | 0.34 |
| Treatment at baseline | ||||
| Epoprostenol alone | 11 (30) | 4 | 7 | |
| Sildenafil alone | 4 (11) | 0 | 4 | |
| Bosentan alone | 6 (16) | 0 | 6 | |
| Beraprost alone | 13 (35) | 2 | 11 | |
| Epoprostenol + Sildenafil | 1 (3) | 0 | 1 | |
| Sildenafil + Beraprost | 1 (3) | 0 | 1 | |
| Sildenafil + Bosentan + Beraprost | 1 (3) | 0 | 1 | |
| Treatment at the end of follow-up | ||||
| Epoprostenol alone | 17 (46) | 5 | 12 | |
| Sildenafil alone | 5 (14) | 0 | 5 | |
| Bosentan alone | 5 (14) | 0 | 5 | |
| Beraprost alone | 6 (16) | 0 | 6 | |
| Epoprostenol + Sildenafil | 3 (8) | 1 | 2 | |
| Sildenafil + Beraprost | 1 (3) | 0 | 1 | |
| Mean right atrial pressure, mm Hg* | 8 ± 4 | 14 ± 2 | 7 ± 3 | <0.001 |
| Mean pulmonary arterial pressure, mm Hg* | 52 ± 20 | 71 ± 4 | 50 ± 19 | 0.002 |
| Cardiac index, l/min/m2 | 2.87 ± 0.80 | 2.61 ± 0.90 | 2.90 ± 0.81 | 0.46 |
| Doppler RV index | 0.64 ± 0.30 | 0.86 ± 0.23 | 0.58 ± 0.29 | 0.04 |
| Plasma BNP, pg/ml† | 170 [70, 796] | 1062 [833, 1210] | 337 [141, 959] | 0.003 |
| AST, U/L | 34 ± 38 | 58 ± 87 | 29 ± 18 | 0.46 |
| ALT, U/L | 36 ± 55 | 67 ± 129 | 29 ± 23 | 0.51 |
| LDH, U/L | 247 ± 82 | 315 ± 167 | 234 ± 48 | 0.29 |
| Bilirubin, mg/dL | 0.9 ± 0.9 | 2.4 ± 1.5 | 0.6 ± 0.3 | N/A |
| Uric acid, mg/dL | 6.8 ± 2.5 | 7.6 ± 4.7 | 6.6 ± 1.8 | 0.64 |
Values are shown as a number (%) or as a mean ± SD as appropriate; ALT = alanine aminotransferase, AST = aspartate aminotransferase, BNP = brain natriuretic peptide; CTD = connective tissue disorder; LDH = lactate dehydrogenase, PAH = pulmonary arterial hypertension, RV = right ventricle, WHO = World Health Organization. *n = 26, † Data are given as a median [25th, 75th percentile].