Survival Outcomes and Impact of Targeted PAH Therapy in Portopulmonary Hypertension in the PVRI GoDeep Meta‐Registry
Year: 2025
Type: article
Study design: Observational Study
Abstract: ABSTRACT 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.
Source: Pulmonary Circulation
DOI: 10.1002/pul2.70121
Authors: 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 A. 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
Institutions: University of Cincinnati, German Center for Lung Research, Universities of Giessen and Marburg Lung Center, Cardio-Pulmonary Institute, Royal Hallamshire Hospital, National Institute for Health and Care Research, NIHR Sheffield Biomedical Research Centre, University of Sheffield, Stanford Medicine, Stanford University, Johns Hopkins University, Johns Hopkins Medicine, Imperial College London, Houston Methodist, Graz University Hospital, Cleveland Clinic Abu Dhabi, Heidelberg University, Aristotle University of Thessaloniki, National Scientific Center "M.D. Strazhesko Institute of Cardiology", Mayo Clinic, University of Pavia, RWTH Aachen University
Language: English
Subfield: Pulmonary and Respiratory Medicine
Field: Medicine
Domain: Health Sciences
Open Access status: gold
Estimated APC paid: $2,310
Funders: Pfizer, AstraZeneca, Vanderbilt University, United Therapeutics Corporation, Johns Hopkins University, University of Pittsburgh, Children’s Hospital of Wisconsin Research Institute, Vanderbilt University Medical Center, Murdoch Children's Research Institute, Acceleron, Cardiovascular Medical Research and Education Fund, Insmed, National Institute for Health and Care Research, British Heart Foundation, Deutsche Forschungsgemeinschaft, Monash University, Universidad Nacional de La Plata, Universidad de Córdoba, Deutsches Zentrum für Lungenforschung, AOP Orphan, Cardio-Pulmonary Institute, National Institutes of Health, National Heart, Lung, and Blood Institute, Janssen Pharmaceuticals
OpenAlex ID: W4412113971