BACKGROUND: Pulmonary hypertension (PH) in interstitial lung disease (ILD) lacks approved therapies. The PVRI GoDeep meta-registry collects real-world data of PH patients from international PH referral centers. METHODS: ILD-PH patients and relevant subgroups (IIP, IPF) were stratified by pulmonary vascular resistance (PVR). Kaplan-Meier survival analyses, adjusted Cox proportional hazards models, additionally accounting for immortal time bias, sensitivity analyses, Heller explained relative risk statistics and target trial emulation framework analysis were employed. RESULTS: Among 34,482 patients, 940 hemodynamically fully characterized incident ILD-PH patients (median age 67[59,74] years) were identified. 62% had severe ILD-PH with PVR >5 WU and poor survival rates (29% and 18% at 3 and 5 years), significantly worse than ILD-PH patients with PVR
- Yogeswaran, A.
- Hassoun, P. M.
- Saleh, K.
- Fünderich, M.
- Balasubramanian, A.
- Konswa, Z.
- Kiely, D. G.
- Lawrie, A.
- Thenappan, T.
- Eichstaedt, C. A.
- Grünig, E.
- Wilkins, M. R.
- Howard, L.
- Olschewski, H.
- Kovacs, G.
- Cajigas, H. R.
- Frantz, R.
- Sabbour, H.
- Sweatt, A. J.
- Zamanian, R. T.
- Arvanitaki, A.
- Giannakoulas, G.
- Elwing, J.
- Jose, A.
- Beckmann, S.
- Olsson, K. M.
- Stadler, S.
- Held, M.
- Halank, M.
- Ewert, R.
- Behr, J.
- Milger-Kneidinger, K.
- Pausch, C.
- Pittrow, D.
- Majeed, R. W.
- Wilhelm, J.
- Ghofrani, H. A.
- Grimminger, F.
- Tello, K.
- Hoeper, M. M.
- Seeger, W.
Keywords
- PVRI GoDeep
- interstitial lung disease
- pulmonary hypertension
- treatment
