Risk Assessment Models and Event-Free Survival in Pulmonary Arterial Hypertension
(2025) In Pulmonary Circulation 15(3).- Abstract
Evidence on the predictive ability of risk assessment models for event-free survival (EFS) in patients with pulmonary arterial hypertension is scarce. We aimed to investigate the relationship between risk status at 6 months after diagnosis (6 M) and EFS, by three risk models: Multicomponent Improvement (MCI), ESC/ERS 4-Strata Risk (4SR), and noninvasive French PH Registry Score (FRS). Data collected in the Swedish PAH Registry 2008–2021 were used. The study population was risk-stratified at 6 M according to each model. Information on PAH-related hospitalization (HOSP) was collected from the National Patient Register. EFS was defined as survival without occurrence of: (1) HOSP; (2) initiation of parenteral prostacyclin therapy or dose... (More)
Evidence on the predictive ability of risk assessment models for event-free survival (EFS) in patients with pulmonary arterial hypertension is scarce. We aimed to investigate the relationship between risk status at 6 months after diagnosis (6 M) and EFS, by three risk models: Multicomponent Improvement (MCI), ESC/ERS 4-Strata Risk (4SR), and noninvasive French PH Registry Score (FRS). Data collected in the Swedish PAH Registry 2008–2021 were used. The study population was risk-stratified at 6 M according to each model. Information on PAH-related hospitalization (HOSP) was collected from the National Patient Register. EFS was defined as survival without occurrence of: (1) HOSP; (2) initiation of parenteral prostacyclin therapy or dose increase ≥ 10%; (3) lung transplantation. The association between risk and EFS was evaluated by Kaplan–Meier estimates and Cox proportional models. The analysis included 411 incident patients, median age 66 y [50, 73]. Median survival time was 3.5 y [1.7; 5.4], and cumulative EFS was 55%. In a Cox proportional regression adjusted for age, eGFR, obesity, atrial fibrillation, and systemic hypertension, EFS was higher in patients who: (1) achieved two or three MCI criteria compared to one or no MCI criterion (HR 0.58; CI 0.39–0.84, p = 0.005); (2) were assessed as low, intermediate–low, or intermediate–high compared to high risk (HR 0.16; CI 0.09–0.28, p < 0.001); or (3) fulfilled one, two, or three low-risk FRS criteria, compared to no low-risk criterion (HR 0.29; CI 0.19–0.43, p < 0.001). Performing a risk assessment 6 months after diagnosis effectively predicts the likelihood of EFS in the studied population, highlighting its prognostic value.
(Less)
- author
- Hjalmarsson, Clara ; Thakur, Tanvee ; Rådegran, Göran LU ; Björklund, Erik ; Wåhlander, Håkan ; Nisell, Magnus ; Papageorgiou, Joanna Maria ; Söderberg, Stefan ; Lautsch, Dominik and Kjellström, Barbro LU
- organization
- publishing date
- 2025-07
- type
- Contribution to journal
- publication status
- published
- subject
- keywords
- hospitalization, outcome, pulmonary arterial hypertension, risk models
- in
- Pulmonary Circulation
- volume
- 15
- issue
- 3
- article number
- e70132
- publisher
- John Wiley & Sons Inc.
- external identifiers
-
- scopus:105011604289
- pmid:40687338
- ISSN
- 2045-8932
- DOI
- 10.1002/pul2.70132
- language
- English
- LU publication?
- yes
- additional info
- Publisher Copyright: © 2025 Merck Sharp & Dohme LLC and The Author(s). Pulmonary Circulation published by John Wiley & Sons Ltd on behalf of Pulmonary Vascular Research Institute.
- id
- 96853db3-9db9-4029-87c4-748f7318f979
- date added to LUP
- 2025-12-11 15:17:33
- date last changed
- 2025-12-12 03:00:07
@article{96853db3-9db9-4029-87c4-748f7318f979,
abstract = {{<p>Evidence on the predictive ability of risk assessment models for event-free survival (EFS) in patients with pulmonary arterial hypertension is scarce. We aimed to investigate the relationship between risk status at 6 months after diagnosis (6 M) and EFS, by three risk models: Multicomponent Improvement (MCI), ESC/ERS 4-Strata Risk (4SR), and noninvasive French PH Registry Score (FRS). Data collected in the Swedish PAH Registry 2008–2021 were used. The study population was risk-stratified at 6 M according to each model. Information on PAH-related hospitalization (HOSP) was collected from the National Patient Register. EFS was defined as survival without occurrence of: (1) HOSP; (2) initiation of parenteral prostacyclin therapy or dose increase ≥ 10%; (3) lung transplantation. The association between risk and EFS was evaluated by Kaplan–Meier estimates and Cox proportional models. The analysis included 411 incident patients, median age 66 y [50, 73]. Median survival time was 3.5 y [1.7; 5.4], and cumulative EFS was 55%. In a Cox proportional regression adjusted for age, eGFR, obesity, atrial fibrillation, and systemic hypertension, EFS was higher in patients who: (1) achieved two or three MCI criteria compared to one or no MCI criterion (HR 0.58; CI 0.39–0.84, p = 0.005); (2) were assessed as low, intermediate–low, or intermediate–high compared to high risk (HR 0.16; CI 0.09–0.28, p < 0.001); or (3) fulfilled one, two, or three low-risk FRS criteria, compared to no low-risk criterion (HR 0.29; CI 0.19–0.43, p < 0.001). Performing a risk assessment 6 months after diagnosis effectively predicts the likelihood of EFS in the studied population, highlighting its prognostic value.</p>}},
author = {{Hjalmarsson, Clara and Thakur, Tanvee and Rådegran, Göran and Björklund, Erik and Wåhlander, Håkan and Nisell, Magnus and Papageorgiou, Joanna Maria and Söderberg, Stefan and Lautsch, Dominik and Kjellström, Barbro}},
issn = {{2045-8932}},
keywords = {{hospitalization; outcome; pulmonary arterial hypertension; risk models}},
language = {{eng}},
number = {{3}},
publisher = {{John Wiley & Sons Inc.}},
series = {{Pulmonary Circulation}},
title = {{Risk Assessment Models and Event-Free Survival in Pulmonary Arterial Hypertension}},
url = {{http://dx.doi.org/10.1002/pul2.70132}},
doi = {{10.1002/pul2.70132}},
volume = {{15}},
year = {{2025}},
}