Patient's data before and 1 year after treatment with bosentan
| . | Baseline . | 1 y follow-up . |
|---|---|---|
| Hemoglobin, g/dL | 10 | 9.8 |
| Hematoctit (%) | 31 | 30.5 |
| Fetal hemoglobin (%) | 5.8 | N/A |
| Transfusions, units of PRBC/mo | 2 | 2 |
| Lactate dehydrogenase, U/L | 302 | 259 |
| Aspartate aminotransferase, U/L | 14 | 31 |
| Alanine aminotransferase, U/L | 15 | 21 |
| Alkaline phosphatase, U/L | 47 | 62 |
| Total Bilirubin, mg/dL | 2 | 1.9 |
| Direct Bilirubin, mg/dL | 0.5 | 0.5 |
| Ferritin, ng/mL | 927 | 808 |
| NT-pro BNP, pg/mL | 235 | 225 |
| NYHA class | III | II |
| 6 MWT, m | 480 | 625 |
| RAP, mmHg | 1 | 1 |
| PAP(S/D/M), mmHg | 45/24/31 | 37/14/22 |
| PCWP, mmHg | 6 | 3 |
| CO, L/min | 6.9 | 6 |
| CI, L/min/m2 | 4.4 | 3.8 |
| PVR, wood units | 3.6 | 3.2 |
| SvO2 % | 61 | 60 |
| . | Baseline . | 1 y follow-up . |
|---|---|---|
| Hemoglobin, g/dL | 10 | 9.8 |
| Hematoctit (%) | 31 | 30.5 |
| Fetal hemoglobin (%) | 5.8 | N/A |
| Transfusions, units of PRBC/mo | 2 | 2 |
| Lactate dehydrogenase, U/L | 302 | 259 |
| Aspartate aminotransferase, U/L | 14 | 31 |
| Alanine aminotransferase, U/L | 15 | 21 |
| Alkaline phosphatase, U/L | 47 | 62 |
| Total Bilirubin, mg/dL | 2 | 1.9 |
| Direct Bilirubin, mg/dL | 0.5 | 0.5 |
| Ferritin, ng/mL | 927 | 808 |
| NT-pro BNP, pg/mL | 235 | 225 |
| NYHA class | III | II |
| 6 MWT, m | 480 | 625 |
| RAP, mmHg | 1 | 1 |
| PAP(S/D/M), mmHg | 45/24/31 | 37/14/22 |
| PCWP, mmHg | 6 | 3 |
| CO, L/min | 6.9 | 6 |
| CI, L/min/m2 | 4.4 | 3.8 |
| PVR, wood units | 3.6 | 3.2 |
| SvO2 % | 61 | 60 |
The starting dose of bosentan was 62.5 mg twice daily and increased after 4 weeks to 125 mg twice daily.
PRBC indicates packed red blood cells; NT-pro BNP, N-terminal fragment of the brain-natriuretic peptide; NYHA, New York Heart Association; 6 MWT, 6 minute walk test; RAP, right atrium pressure; PAP(S/D/M), pulmonary artery pressure (systolic, diastolic, mean); PCWP, pulmonary capillary wedge pressure; CO, cardiac output; CI, cardiac index; PVR, pulmonary vascular resistance; SvO2, mixed venous oxygen saturation; and N/A, not available.