BibTex format
@article{Jose:2025:10.1002/pul2.70121,
author = {Jose, A and Yogeswaran, A and Fuenderich, M and Kiely, D and Sweatt, AJ and Zamanian, RT and Hassoun, PM and Mouawad, A and Balasubramanian, A and Wilkins, M and Lawrie, A and Howard, L and Sahay, S and Olschewski, H and Kovacs, G and Saleh, K and Sabbour, H and Eichstaedt, CA and Grünig, E and Giannakoulas, G and Arvanitaki, A and Sirenko, Y and Torbas, O and Cajigas, H and Frantz, R and Scelsi, L and Ghio, S and Majeed, RW and Wilhelm, J and Ghofrani, HA and Grimminger, F and Tello, K and Elwing, J and Seeger, W},
doi = {10.1002/pul2.70121},
journal = {Pulmonary Circulation},
title = {Survival outcomes and impact of targeted PAH therapy in portopulmonary hypertension in the PVRI GoDeep meta-registry},
url = {http://dx.doi.org/10.1002/pul2.70121},
volume = {15},
year = {2025}
}
RIS format (EndNote, RefMan)
TY - JOUR
AB - 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. P
AU - Jose,A
AU - Yogeswaran,A
AU - Fuenderich,M
AU - Kiely,D
AU - Sweatt,AJ
AU - Zamanian,RT
AU - Hassoun,PM
AU - Mouawad,A
AU - Balasubramanian,A
AU - Wilkins,M
AU - Lawrie,A
AU - Howard,L
AU - Sahay,S
AU - Olschewski,H
AU - Kovacs,G
AU - Saleh,K
AU - Sabbour,H
AU - Eichstaedt,CA
AU - Grünig,E
AU - Giannakoulas,G
AU - Arvanitaki,A
AU - Sirenko,Y
AU - Torbas,O
AU - Cajigas,H
AU - Frantz,R
AU - Scelsi,L
AU - Ghio,S
AU - Majeed,RW
AU - Wilhelm,J
AU - Ghofrani,HA
AU - Grimminger,F
AU - Tello,K
AU - Elwing,J
AU - Seeger,W
DO - 10.1002/pul2.70121
PY - 2025///
SN - 2045-8932
TI - Survival outcomes and impact of targeted PAH therapy in portopulmonary hypertension in the PVRI GoDeep meta-registry
T2 - Pulmonary Circulation
UR - http://dx.doi.org/10.1002/pul2.70121
UR - https://doi.org/10.1002/pul2.70121
VL - 15
ER -