TABLE 2.
Hazard ratio | 95% CI | p-value | |
Sex (male) | 1.330 | 0.939–1.882 | 0.108 |
Age, per year | 1.032 | 1.018–1.046 | <0.001 |
Body mass index, per kg·m−2 | 1.007 | 0.978–1.036 | 0.646 |
Biallelic EIF2AK4 mutations | 3.176 | 1.177–8.570 | 0.023 |
NYHA FC, per class | 1.697 | 1.303–2.209 | <0.001 |
6MWD, per m | 0.997 | 0.996–0.998 | <0.001 |
BNP, per ng·L−1 | 1.000 | 1.000–1.000 | 0.053 |
NT-proBNP, per ng·L−1 | 1.000 | 1.000–1.000 | 0.019 |
RAP, per mmHg | 1.026 | 0.991–1.062 | 0.146 |
mPAP, per mmHg | 1.001 | 0.987–1.015 | 0.874 |
PAWP, per mmHg | 0.957 | 0.914–1.001 | 0.058 |
Cardiac output, per L·min−1 | 0.856 | 0.753–0.973 | 0.017 |
Cardiac index, per L·min−1·m−2 | 0.673 | 0.524–0.864 | 0.002 |
PVR, per WU | 1.018 | 0.985–1.053 | 0.290 |
S vO2 , per % | 0.970 | 0.948–0.992 | 0.007 |
P aO2 , per mmHg | 0.979 | 0.966–0.993 | 0.003 |
P aCO2 , per mmHg | 1.012 | 0.980–1.044 | 0.472 |
TLC, per % | 0.997 | 0.988–1.007 | 0.599 |
FEV1, per % | 0.999 | 0.991–1.007 | 0.781 |
D LCO , per % | 0.980 | 0.967–0.993 | 0.002 |
D LCO /AV, per % | 0.990 | 0.980–1.000 | 0.054 |
Number of PAH therapies | 0.793 | 0.597–1.053 | 0.108 |
EIF2AK4: Eukaryotic translation initiation factor 2-α kinase 4; NYHA FC: New York Heart Association functional class; 6MWD: 6-min walk distance; BNP: brain natriuretic peptide; NT-proBNP: N-terminal pro-brain natriuretic peptide; RAP: right atrial pressure; mPAP: mean pulmonary artery pressure; PAWP: pulmonary artery wedge pressure; PVR: pulmonary vascular resistance; SvO2: mixed venous oxygen saturation; PaO2: partial pressure of oxygen; PaCO2: partial pressure of carbon dioxide; TLC: total lung capacity; FEV1: forced expiratory volume in 1 s; DLCO: diffusing capacity for carbon monoxide; DLCO/AV: diffusing capacity for carbon monoxide to alveolar volume ratio; PAH: pulmonary arterial hypertension.