Patient | Age at PAH diagnosis (years) | NYHA FC | mPAP (mmHg) | CI (L·min−1·m−2) | PVR (WU) | Acute NO response | ΔPVR (%) | 6MWD (m) | PaO2 (mmHg) | DLCO (% pred) |
I-1 | 26 | II | 79 | 3.00 | 16.4 | Yes | −40 | 580 | 67 | 44 |
I-2 | 13 | III | 68 | 2.65 | 17.3 | No | 391 | 93 | 69 | |
I-3 | 40 | IV | 70 | 1.80 | 23.5 | 70 | ||||
II | 21 | III | 92 | 1.70 | 31.5 | 54 | 50 | |||
III | 35 | IV | 66 | 1.70 | 17.7 | No | −5 | 373 | 57 | 72 |
IV | 6 | III | 81 | 2.90 | 29.6 | No | −28 | 322 | ||
V | 24 | III | 54 | 2.96 | 9.0 | Yes | −55 | 491 | 97 | 74 |
VI | 32 | III | 58 | 2.10 | 12.0 | No | −28 | 460 | 61 | 62 |
VII | 13 | II | 76 | 3.00 | 15.1 | No | −27 | 285 | 49 | 60 |
VIII | 12 | III | 105 | 4.40 | 12.8 | No | −28 | |||
IX | 36 | III | 41 | 3.80 | 4.2 | 79 | 60 | |||
X | 10 | III | 87 | 1.90 | 30.0 | −31 | ||||
XI | 3 | III | 51 | 3.90 | 12.8 | No | −29 | 78 | ||
XII-1 | 8 | II | 49 | 3.71 | 8.2 | No | −20 | 370 | 87 | 97 |
XII-2 | 2 | 38 | 5.38 | 9.6 | No | −9 | ||||
XIII | 18 | III | 80 | 2.30 | 20.3 | No | 460 | 84 | 63 | |
XIV | 27 | II | 37 | 3.00 | 7.5 | No | −10 | 525 | 92 | 66 |
XV | 53 | III | 43 | 2.20 | 10.3 | 0 | 54 | 56 | ||
XVI | 10 | III | 80 | 3.25 | 17.3 | No | −20 | |||
XVII | 16 | II | 33 | 2.50 | 6.2 | No | −24 | 600 | 92 | 81 |
NYHA: New York Heart Association; FC: Functional Class; mPAP: mean pulmonary arterial pressure; CI: cardiac index; PVR: pulmonary vascular resistance; NO: nitric oxide; ΔPVR: percentage of PVR decrease after NO challenge; 6MWD: 6-min walk distance; PaO2: arterial oxygen tension; DLCO: diffusing capacity of the lung for carbon monoxide corrected for haemoglobin concentration.