Table 1.
Baseline characteristics of the study patients.
| Total population | Survivors | Non‐survivors or transplant recipients | p | |
|---|---|---|---|---|
| N | 180 | 120 | 60 | – |
| Age at CPET (years) | 44.5 ± 0.9 | 43.8 ± 1.0 | 46.6 ± 1.9 | 0.145 |
| Time from PAH diagnosis and CPET (months) | 32.6 (9.1–75.6) | 25.7 (8.0–79.9) | 46.2 (13.3–70.7) | 0.456 |
| Type of PAH | 0.085 | |||
| Idiopathic | 96 (53.3) | 64 (53.3%) | 32 (53.3%) | |
| Heritable | 9 (5.0) | 9 (7.5%) | 0 (0.0%) | |
| Drug‐induced | 17 (9.4) | 7 (5.8%) | 10 (16.7%) | |
| CHD‐PAH | 8 (4.4) | 7 (5.8%) | 1 (1.7%) | |
| CTD‐PAH | 27 (15.0) | 17 (14.2%) | 10 (16.7%) | |
| PVOD | 9 (5.0) | 6 (5.0%) | 3 (5.0%) | |
| Portopulmonary | 4 (2.2) | 2 (1.7%) | 2 (3.3%) | |
| HIV | 10 (5.6) | 8 (6.7%) | 2 (3.3%) | |
| Sex (female) | 125 (69.4%) | 91 (75.8%) | 34 (56.7%) | 0.008 |
| BMI (kg/m2) | 24.0 (21.5–28.0) | 24.4 (22.0–28.7) | 23.4 (20.8–26.0) | 0.040 |
| WHO functional class | 0.011 | |||
| I‐II | 145 (80.6) | 103 (85.8) | 42 (70.0) | |
| III | 35 (19.4) | 17 (14.2) | 18 (30.0) | |
| 6MWT (m) | 475.0 ± 90.8 | 498.7 ± 89.7 | 453.1 ± 85.8 | 0.001 |
| NT‐proBNP (pg/mL) | 253.0 (77.0–895.5) | 124.5 (58.5–528.0) | 756.0 (280.0–1786.5) | <0.001 |
| Pulmonary vasodilator therapy | ||||
| Calcium‐antagonists | 12 (6.7) | 11 (9.2) | 1 (1.7) | 0.048 |
| Monotherapy | 72 (40.0) | 53 (44.2) | 19 (31.7) | 0.033 |
| Dual therapy | 67 (37.2) | 36 (30.0) | 31 (51.7) | |
| Triple therapy | 33 (18.3) | 24 (20.0) | 9 (15.0) | |
| CPET variables | ||||
| Max. power (Watts) | 60.0 (50.0–75.0) | 65.0 (50.0–80.0) | 50.0 (45.0–60.0) | <0.001 |
| Peak VO2 (mL/kg/min) | 16.5 (13.9–20.0) | 17.4 (14.0–21.0) | 15.3 (13.0–18.5) | 0.014 |
| Peak VO2 (predicted%) | 59.5 (48.0–73.0) | 63.0 (51.0–74.0) | 53.0 (45.0–65.0) | 0.002 |
| Peak O2 pulse (mL/beat) | 8.0 (6.0–10.0) | 8.0 (6.0–10.0) | 7.0 (6.0–10.0) | 0.534 |
| VE/VCO2 at AT | 35.4 (31.2–41.4) | 35.0 (30.3–40.1) | 37.3 (32.6–44.7) | 0.021 |
| PetCO2 at AT | 30 (25–35) | 30.0 (25.0–34.8) | 29.6 (23.8–33.2) | 0.155 |
| Max. VE/VCO2 | 45 (36–54) | 43.5 (34.0–54.0) | 45.0 (38.0–58.0) | 0.205 |
| Max. SBP (mmHg) | 149.6 ± 2.1 | 153.1 ± 2.5 | 142.3 ± 3.6 | 0.015 |
Abbreviations: 6MWT, 6‐min walk test; AT, anaerobic threshold; BMI, body mass index; BP, maximum systolic blood pressure; CHD‐PAH, pulmonary arterial hypertension associated with congenital heart diseases; CPET, cardiopulmonary exercise testing; CTD‐PAH, pulmonary arterial hypertension associated with connective tissue disorders; HIV, human immunodeficiency virus; PAH, pulmonary arterial hypertension; PetCO2, end‐tidal carbon dioxide; max; PVOD, pulmonary veno‐occlusive disease; VE/VCO2, ventilatory equivalent for carbon dioxide; VO2, oxygen uptake; WHO, World Health Organization.