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Updated: Sep 14, 2025

Establishment and Validation of a Rat Model of Pulmonary Arterial Hypertension Associated with Pulmonary Fibrosis
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Risk Assessment Models and Event-Free Survival in Pulmonary Arterial Hypertension.

Clara Hjalmarsson1,2, Tanvee Thakur3, Göran Rådegran4

  • 1Department of Cardiology Sahlgrenska University Hospital Gothenburg Sweden.

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|July 21, 2025
PubMed
Summary

Risk assessment models at six months post-diagnosis effectively predict event-free survival (EFS) in pulmonary arterial hypertension (PAH) patients. These models, including MCI, 4SR, and FRS, demonstrate significant prognostic value for PAH management.

Keywords:
hospitalizationoutcomepulmonary arterial hypertensionrisk models

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Area of Science:

  • Cardiology
  • Pulmonology
  • Medical Informatics

Background:

  • Pulmonary arterial hypertension (PAH) management relies on accurate risk stratification for predicting patient outcomes.
  • Limited evidence exists on the predictive accuracy of risk assessment models for event-free survival (EFS) in PAH patients.
  • Early risk assessment is crucial for tailoring treatment strategies and improving survival in PAH.

Purpose of the Study:

  • To evaluate the predictive ability of three risk assessment models—Multicomponent Improvement (MCI), ESC/ERS 4-Strata Risk (4SR), and French PH Registry Score (FRS)—for EFS in PAH patients.
  • To determine the association between risk status at six months post-diagnosis and EFS.
  • To assess the prognostic value of these risk models in a real-world PAH cohort.

Main Methods:

  • Retrospective analysis of 411 incident PAH patients from the Swedish PAH Registry (2008-2021).
  • Risk stratification at six months post-diagnosis using MCI, 4SR, and FRS models.
  • Event-free survival (EFS) defined as survival without hospitalization, significant treatment escalation, or lung transplantation.
  • Kaplan-Meier estimates and Cox proportional regression models were used to evaluate the association between risk status and EFS, adjusted for covariates.

Main Results:

  • All three models (MCI, 4SR, FRS) showed significant predictive ability for EFS.
  • Patients achieving better risk profiles (e.g., two or three MCI criteria, low/intermediate risk by 4SR, low-risk FRS criteria) had significantly higher EFS.
  • Cox regression revealed strong associations: HR 0.58 for MCI, HR 0.16 for 4SR, and HR 0.29 for FRS, all with p < 0.001, indicating reduced risk of events in lower-risk groups.

Conclusions:

  • Risk assessment at six months after PAH diagnosis is a valuable tool for predicting EFS.
  • The MCI, 4SR, and FRS models demonstrate prognostic utility, aiding clinicians in identifying high-risk patients.
  • Implementing these risk assessment strategies can optimize PAH patient management and potentially improve long-term outcomes.