Table 4 Proportion of excess risk mediated by genetic status and clinical variables at diagnosis
Death or transplantation | HR (95% CI) | p value |
|---|---|---|
BMPR2 or biallelic EIF2AK4 variants | 0.114(0.032–0.404) | 0.001* |
Adjusted for DLCO | 0.035(0.001–1.032) | 0.05 |
Adjusted for DLCO, Peak VO2%, 6MWD | 0.001(0.000–0.179) | 0.011* |
DLCO | 0.958(0.919–0.999) | 0.042* |
Adjusted for BMPR2 or biallelic EIF2AK4 variants | 1.024(0.954–1.09) | 0.509 |
Adjusted for Peak VO2%, 6MWD, BMPR2 or EIF2AK4 mutations | 1.158(1.002–1.339) | 0.047* |
Peak VO2% | 0.89(0.819–0.987) | 0.025* |
Adjusted for BMPR2 or biallelic EIF2AK4 variants | 0.883(0.803–0.971) | 0.011* |
Adjusted for DLCO, 6MWD, BMPR2 or EIF2AK4 mutations | 0.725(0.505–1.039) | 0.08 |
6MWD | 0.992(0.984–1) | 0.04* |
Adjusted for BMPR2 or biallelic EIF2AK4 variants | 0.992(0.985–0.999) | 0.018* |
Adjusted for DLCO, Peak VO2%, BMPR2 or biallelic EIF2AK4 variants | 1.00(0.988–1.013) | 0.975 |
Cardiac Index | 0.778(0.285–2.122) | 0.624 |
Pulmonary vascular resistance | 1(0.999–1.001) | 0.429 |
Mean pulmonary arterial pressure | 1.013(0.971–1.057) | 0.55 |
SvO2 | 1.009(0.923–1.1037) | 0.841 |