TABLE 2.
Evolution of clinical, functional and haemodynamic parameters of patient 1
| Parameter | July 2009 | June 2013 | October 2016 | ||
| NYHA FC | II | II | III/IV | ||
| 6MWD, m | 550 | 506 | NA | ||
| DLCO, %pred | 51 | 35 | 23 | ||
| KCO, %pred | 53 | 39 | 25 | ||
| ABG on room air | |||||
| PaO2, mmHg | 92 | 86 | 46 | ||
| PaCO2, mmHg | 30 | 27 | 27 | ||
| CPET | |||||
| V′O2, mL·min−1·kg−1 (% pred) | 21.4 (69%) | 16 (52%) | Not feasible | ||
| VD/VT | 0.29 | 0.44 | Not feasible | ||
| Peak PaO2, mmHg | 63 | 53 | Not feasible | ||
| PA–aO2, mmHg | 57 | 71 | Not feasible | ||
| BNP, ng·L−1 (<80) | 34 | 34 | – | ||
| NT-proBNP, ng·L−1 (<300) | NA | NA | 160 | ||
| Haemodynamics | Rest | 100 W | Rest | 60 W | Rest |
| RAP, mmHg | 0 | NA | 2 | NA | 3 |
| mPAP, mmHg | 17 | 36 | 25 | 45 | 38 |
| PAWP, mmHg | 1 | 15 | 4 | 14 | 6 |
| CI, L·min−1·m−2 | 3.8 | 7.0 | 3.49 | 6.4 | 2.6 |
| TPR, WU | 2.4 | 2.7 | 3.9 | 3.8 | 7.8 |
| PVR, WU | 2.2 | 1.6 | 3.3 | 2.6 | 6.6 |
6MWD: 6-min walk distance; ABG: arterial blood gases; BNP: brain natriuretic peptide; CI: cardiac index; CPET: cardiopulmonary exercise testing; DLCO: diffusing capacity of the lung for carbon monoxide; FC: functional class; KCO: transfer coefficient of the lung for carbon monoxide; mPAP: mean pulmonary artery pressure; NA: not available; NT-proBNP: N-terminal proBNP; NYHA: New York Heart Association; PA–aO2: alveolar–arterial oxygen tension gradient; PaCO2: arterial carbon dioxide tension; PaO2: arterial oxygen tension; PAWP: pulmonary artery wedge pressure; PVR: pulmonary vascular resistance; RAP: right atrial pressure; TPR: total pulmonary resistance; VʹO2: oxygen uptake; VD/VT: dead space/tidal volume ratio; WU: Wood unit.