Evolution of functional and haemodynamic characteristics
| August 2007 | November 2007 | February 2008 | April 2008 | |
| PAH therapy at evaluation | None | Bosentan | Bosentan | Bosentan + sildenafil |
| NYHA FC | III | III | IV | IV |
| Pra mmHg | 3 | 13 | 4 | – |
| P̄pa mmHg | 44 | 45 | 58 | – |
| Ppcw mmHg | 5 | 6 | 4 | – |
| CI L·min−1·m−2 | 4.08 | 3.45 | 3.17 | – |
| TPRi | 864 | 1043 | 1462 | – |
| PVRi | 765 | 904 | 1363 | – |
| Sv,O2 % | 73 | 69 | 68 | – |
| Acute vasodilator testing | Negative | – | – | – |
| 6MWD m | 363 | 390 | 338 | 0 |
| Borg scale 0–10 | 2 | 2 | 9 (malaise) | – |
| DL,CO % | 49 | – | 39 | – |
| DLCO/VA % | 57 | – | 48 | – |
| Pa,O2 mmHg | 67 | – | 71 | 55 (6 L O2) |
| BNP pg·mL−1 | 46 | – | 56 | 715 |
| Modification of specific PAH therapy | Start of bosentan therapy | Bosentan monotherapy | Addition of sildenafil | Stop sildenafil Listed for urgent lung transplantation |
PAH: pulmonary arterial hypertension; NYHA: New York Heart Association; FC: functional class ; Pra: right atrial pressure; P̄pa: mean pulmonary arterial pressure; Ppcw: pulmonary capillary wedge pressure; CI: cardiac index; TPRi: total pulmonary resistance index; PVRi: pulmonary vascular resistance index; Sv,O2: mixed venous oxygen saturation; 6MWD: 6-min walk distance; DL,CO: diffusing capacity of the lung for carbon monoxide; VA: alveolar volume; Pa,O2: arterial oxygen tension; BNP: brain natriuretic peptide.