von Ashesh Chakraborty ; Marie Zöller ; Aydan Sardogan ; Markus Klotz ; Michal Mastalerz ; Hannah Marchi ; Raphael Meixner ; Rudolf A. Hatz ; Jürgen Behr ; Anne Hilgendorff ; Misako Nakayama ; Claudia Staab-Weijnitz
von Ashesh Anjankumar Chakraborty ; Juliana Giraldo-Arias ; Juliane Merl-Pham ; Elisabeth Dick ; Michal Mastalerz ; Marie Zöller ; Hannah Marchi ; Ronan Le Gleut ; Rudolf A. Hatz ; Jürgen Behr ; Anne Hilgendorff ; Stefanie M. Hauck ; Claudia Staab-Weijnitz
von Steven H. Abman ; Sylvia M. Nikkho ; Rolf M. F. Berger ; Maria Jesus del Cerro ; Eric D. Austin ; Maurice Beghetti ; Dunbar Ivy ; Megan Griffiths ; Anne Hilgendorff ; Steven H. Kawut ; Usha S. Krishnan ; Mary P. Mullen ; Shahin Moledina ; Bernard Thébaud ; Norman Stockbridge
von Saahithi Mallapragada ; Ruqian Lyu ; Arianna L. Williams-Katek ; Brandon K. Fischer ; Annika Vannan ; Niran Hadad ; Evan D. Mee ; Shawyon P. Shirazi ; Christopher S. Jetter ; Nicholas M. Negretti ; Anne Hilgendorff ; Laurie C. Eldredge ; Gail H. Deutsch ; Davis J. McCarthy ; Jonathan A. Kropski ; Jennifer M. S. Sucre ; Nicholas E. Banovich
von Athiththan Yogeswaran ; Paul M. Hassoun ; Khaled Saleh ; Meike Fünderich ; Aparna Balasubramanian ; Ziad Konswa ; David G. Kiely ; Allen Lawrie ; Thenappan Thenappan ; Christina Eichstaedt ; Ekkehard Grünig ; Martin R. Wilkins ; Luke Howard ; Horst Olschewski ; Gabor Kovacs ; Hector R. Cajigas ; Robert Frantz ; Hani Sabbour ; Andrew J. Sweatt ; Roham T. Zamanian ; Alexandra Arvanitaki ; George Giannakoulas ; Jean Elwing ; Arun Jose ; Stephan Beckmann ; Karen M. Olsson ; Stefan Stadler ; Matthias Held ; Michael Halank ; Ralf Ewert ; Jürgen Behr ; Katrin Milger-Kneidinger ; Christine Pausch ; David Pittrow ; Raphael W. Majeed ; Jochen Wilhelm ; Hossein Ardeschir Ghofrani ; Friedrich Grimminger ; Khodr Tello ; Marius M. Hoeper ; Werner Seeger ; Anne Hilgendorff
Pulmonary hypertension (PH) in interstitial lung disease (ILD) lacks approved therapies.The Pulmonary Vascular Research Institute GoDeep metaregistry collects real-world data of patients with PH from international PH referral centers.Patients with ILD-PH and relevant subgroups (idiopathic interstitial pneumonia [IIP], idiopathic pulmonary fibrosis [IPF]) were stratified by pulmonary vascular resistance (PVR). Kaplan-Meier survival analyses and adjusted Cox proportional hazards models were employed, additionally accounting for immortal time bias, sensitivity analyses, Heller explained relative risk statistics, and target trial emulation framework analysis.Among 34,482 patients, 940 with hemodynamically fully characterized incident ILD-PH (median age, 67 [IQR, 59-74] yr) were identified. A total of 62% had severe ILD-PH with PVR >5 Wood units (WU) and poor survival rates (29% and 18% at 3 and 5 yr), significantly worse than patients with ILD-PH with PVR ≤5 WU and patients with pulmonary arterial hypertension. Survival was poorest in severe IPF-PH. A total of 59% of all patients ILD-PH received PH-targeted therapy, predominantly phosphodiesterase-5 inhibitors (PDE5is). PDE5i treatment was consistently associated with significantly improved survival in patients with severe PH (hazard ratios of 0.537 [0.370-0.781], 0.461 [0.233-0.913], and 0.435 [0.215-0.8] for IIP-PH, IPF-PH, and IIP-PH with nintedanib/pirfenidone background therapy), but not in patients with less severe hemodynamic impairment, supported by sensitivity analyses, Heller statistics, and target trial emulation framework analysis. The survival statistics of patients with PDE5i-treated IIP-PH or IPF-PH were validated in the independent COMPERA registry. Combination therapy with PDE5is and inhaled prostacyclin analogues was superior to monotherapy using PDE5is (hazard ratio, 0.341; 0.205-0.566).Prognosis in ILD-PH was generally very poor and was related to PH severity. PDE5i treatment in severe IIP-PH and IPF-PH was associated with improved survival, which is to be further verified in controlled trials.
American journal of respiratory and critical care medicine New York, NY : American Thoracic Society, 1959 211(2025), 10, Seite 1855-1866 Online-Ressource
von Arun Jose ; Athiththan Yogeswaran ; Meike Fuenderich ; David Kiely ; Andrew J. Sweatt ; Roham T. Zamanian ; Paul M. Hassoun ; Antoine Mouawad ; Aparna Balasubramanian ; Martin Wilkins ; Allan Lawrie ; Luke Howard ; Sandeep Sahay ; Horst Olschewski ; Gabor Kovacs ; Khaled Saleh ; Hani Sabbour ; Christina Eichstaedt ; Ekkehard Grünig ; George Giannakoulas ; Alexandra Arvanitaki ; Yuriy Sirenko ; Olena Torbas ; Hector Cajigas ; Robert Frantz ; Laura Scelsi ; Stefano Ghio ; Raphael W. Majeed ; Jochen Wilhelm ; Hossein Ardeschir Ghofrani ; Friedrich Grimminger ; Khodr Tello ; Jean Elwing ; Werner Seeger ; Anne Hilgendorff
Zuerst veröffentlicht: 08. Juli 2025 ; Gesehen am 06.03.2026
Portopulmonary hypertension (PoPH), a type of pulmonary arterial hypertension (PAH) in patients with liver disease, is associated with high morbidity and mortality. The relationship between cardiopulmonary hemodynamics, PAH therapy, and survival in PoPH remains unclear. We performed a retrospective cohort study of PoPH patients from the international pulmonary hypertension (PH) meta-registry, PVRI GoDeep. PAH was defined by a mean pulmonary arterial pressure > 20 mmHg, pulmonary arterial wedge pressure ≤ 15 mmHg, and a pulmonary vascular resistance (PVR) > 2 Wood Units. PoPH diagnoses were assigned by each center's PH specialist based on international guidelines at the time of enrollment. 246 incident PoPH patients met eligibility criteria and were included in the analysis, equally split between males (51%) and females (49%), with a median age of 54 years. When compared to both patients with IPAH and those with other subtypes of PAH (not classified as PoPH or IPAH), those with PoPH had significantly lower 5-year survival rates (46% vs. 68% vs. 65%, log-rank p < 0.001). Amongst the PoPH patients, however, there was no significant difference in 5-year survival when dichotomized by disease severity, either by a PVR of 5 Wood Units or a CI of 2.5 L/min/m2. Treatment of the PoPH patients with PAH-targeted therapies was associated with significantly higher 5-year survival rates compared to those not receiving such treatments, as shown by Kaplan-Meier analysis. This survival benefit was observed for PDE5i (50% vs. 34%, log-rank p = 0.029), ERA (58% vs. 34%, log-rank p < 0.001), and the combination of PDE5i and/or ERA (51% vs. 22%, log-rank p < 0.001), as well as any PAH-targeting treatment (50% vs. 26%, log-rank p = 0.007). Corresponding survival advantage was noted when including only PoPH patients with MELD Score ≥ 13. PoPH is a disease with significantly worse long-term survival than other PAH subtypes, but targeted PAH therapy is associated with a robust survival benefit. Survival did not differ across high-risk PVR and cardiac index thresholds, suggesting the factors that influence prognosis and survival in PoPH may be unique as compared to other PAH subtypes, and warrant further investigation.
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