Case 10: clinical characteristics
| Treatment at evaluation | None | ERA (for 4 months) | ERA and PDE-5I (for 5 months) | ERA, PDE-5I and i.v. prostacyclin (for 4 months) |
| WHO functional class | III | III | III | II |
| 6MWD m | 519 | 525 | 441 | 601 |
| Pra mmHg</emph> | 7 | 8 | 8 | 3 |
| P̄pa mmHg | 55 | 60 | 65 | 47 |
| CI L·min−1·m−2 | 2.01 | 2.5 | 2.09 | 3.35 |
| PVR dyn·s·cm−5 | 1248 | 1066 | 1368 | 649 |
| BNP pg·mL−1 | 217 | 360 | 62 | |
| Modification of specific PAH therapy | Initiation of ERA | Addition of PDE5-I | Addition of i.v. prostacyclin | No change |
ERA: endothelin receptor agonist; PDE5-I: phosphodiesterase type-5 inhibitor; WHO: World Health Organization; 6MWD: 6-min walk distance; Pra: right atrial pressure; P̄pa: mean pulmonary arterial pressure; CI: cardiac index; PVR: pulmonary vascular resistance; BNP: brain natriuretic peptide; PAH: pulmonary arterial hypertension.