Hemodynamics and Phosphodiesterase-5 Inhibitor Treatment Associated with Survival in ILD-PH: A PVRI GoDeep Meta-Registry Analysis

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.