At diagnosis | |
Sex male/female n | 7/5 |
Age at diagnosis yrs | 41±12 |
Tobacco exposure pack-yrs | 16±17.3 |
NYHA class at diagnosis | |
Class II | 1 (8.3) |
Class III | 8 (66.7) |
Class IV | 3 (25) |
6-min walk test | |
Distance m | 353±103 |
Nadir Sp,O2 % | 83±6 |
Pa,O2 mmHg | 66±8 |
Pa,CO2 mmHg | 29±4 |
DL,CO % pred | 34±14 |
P̄pa mmHg | 56±10 |
RAP mmHg | 8±6 |
Ppcw mmHg | 7±3 |
CI L·min−1·m−2 | 2.40±0.72 |
SI mL·m−2 | 26±5 |
PVRi WU·m−2 | 23.3±10.6 |
SV,O2 % | 61±12 |
Acute NO responders | 1 (8.3) |
ΔP̄pa with NO % | 10±7 |
patients with ≥2 radiological abnormalities# n | 12 (100) |
Occult alveolar haemorrhage¶ | 3/9 (33.3) |
Data are preented as mean±sd or n (%), unless otherwise indicated. NYHA: New York Heart Association; Sp,O2: pulse arterial oxygen saturation; Pa,O2: arterial partial pressure of oxygen; Pa,CO2: arterial partial pressure of carbon dioxide; DL,CO: diffusing lung capacity of carbon monoxide; % pred: % predicted; P̄pa: mean pulmonary artery pressure; RAP: right atrial pressure; Ppcw: pulmonary capillary wedge pressure; CI: cardiac index; SI: systolic index; PVRi: pulmonary vascular resistance index; WU: Wood units; SV,O2: mixed venous oxygen saturation; NO: nitric oxide; ΔP̄pa: difference in P̄pa. #: radiological abnormalities on high-resolution computed tomography of the chest include lymph node enlargement, centrilobular ground-glass opacities and septal lines; ¶: defined by Golde score >100. n = 12.