Survival Outcomes and Impact of Targeted PAH Therapy in Portopulmonary Hypertension in the PVRI GoDeep Meta‐Registry

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 <i>p</i> < 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/m<sup>2</sup>. 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 <i>p</i> = 0.029), ERA (58% vs. 34%, log-rank <i>p</i> < 0.001), and the combination of PDE5i and/or ERA (51% vs. 22%, log-rank <i>p</i> < 0.001), as well as any PAH-targeting treatment (50% vs. 26%, log-rank <i>p</i> = 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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Publication Details

Journal
Pulmonary Circulation
Published
2025-07-01
DOI
https://doi.org/10.1002/pul2.70121
Citations
3
Primary Topic
Pulmonary Hypertension Research and Treatments
Type
article
Field-Weighted Citation Impact
4.56

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article

Survival Outcomes and Impact of Targeted PAH Therapy in Portopulmonary Hypertension in the PVRI GoDeep Meta‐Registry

Luke Howard, Jean Elwing, Alexandra Arvanitaki, George A. Giannakoulas et al.
3 citations
Pulmonary Circulation
Pulmonary Hypertension Research and Treatments
4.56
article

Survival Outcomes and Impact of Targeted PAH Therapy in Portopulmonary Hypertension in the PVRI GoDeep Meta‐Registry

Luke Howard, Jean Elwing, Alexandra Arvanitaki, George A. Giannakoulas, David Gerard Kiely, Arun Jose, O.O. Torbas, Werner Seeger, Paul M. Hassoun, Raphael W. Majeed, Khaled Saleh, Laura Scelsi, Yu.M. Sіrenko, Héctor Cajigas, Christina A. Eichstaedt, Roham T. Zamanian, Jochen Wilhelm, Aparna Balasubramanian, Sandeep Sahay, Stefano Ghio, Antoine Mouawad, Hani Sabbour, Horst Olschewski, Andrew J. Sweatt, Robert P. Frantz, Khodr Tello, Allan Lawrie, Meike Fünderich, Athiththan Yogeswaran, Hossein Ardeschir Ghofrani, Friedrich Grimminger, Ekkehard Grünig, Martin R. Wilkins, Gábor Kovács
article en
3 citations

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 <i>p</i> < 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/m<sup>2</sup>. 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 <i>p</i> = 0.029), ERA (58% vs. 34%, log-rank <i>p</i> < 0.001), and the combination of PDE5i and/or ERA (51% vs. 22%, log-rank <i>p</i> < 0.001), as well as any PAH-targeting treatment (50% vs. 26%, log-rank <i>p</i> = 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.

Pulmonary CirculationVol. 15(3)
Johns Hopkins University (US), Aristotle University of Thessaloniki (GR), Heidelberg University (DE), University of Pavia (IT), Johns Hopkins Medicine (US), Royal Hallamshire Hospital (GB), Graz University Hospital (AT), Methodist Hospital (US), German Center for Lung Research (DE), Universities of Giessen and Marburg Lung Center (DE), Mayo Clinic in Florida (US), National Scientific Center "M.D. Strazhesko Institute of Cardiology" (UA), Cardio-Pulmonary Institute (DE), Imperial College London (GB), University of Cincinnati (US), RWTH Aachen University (DE), University of Sheffield (GB), Stanford University (US)
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, Janssen Pharmaceuticals
Openalex Percentile: Top 5%
Pulmonary Hypertension Research and Treatments
4.56
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