Novel hepatic function-based prognostic prediction model for pulmonary arterial hypertension
Bingyang Liu, Wenjie Yan, Hanwen Zhang, Qin Luo, Beilan Yang, Yu Chen, Jimin Zhang, Qixian Zeng, Yanru Liang, Zhihui Zhao, Qing Zhao, Tao Yang, Li Deng, Zhihong Liu, Yicheng Yang, Changming XiongBackground
Hepatic dysfunction is increasingly recognized as a contributor to adverse outcomes in patients with pulmonary arterial hypertension (PAH). However, current prognostic models for PAH do not incorporate hepatic function parameters.
Objectives
To develop and validate a novel risk prediction model that integrates hepatic function indicators with established PAH severity markers, and to compare its performance against the COMPERA 2.0 model.
Design
Prospective cohort study.
Methods
Three liver fibrosis scores (LFSs)—FIB-4, NFS, and HUI—were evaluated for their prognostic significance. A total of 715 PAH patients from Fuwai Hospital were used to develop the FW-HP model. The model’s predictive performance was assessed and compared with COMPERA 2.0. Risk stratification of the PAH cohort was subsequently conducted based on model scores.
Results
All three LFSs were independently associated with mortality in PAH. The FW-HP model, incorporating albumin, total bilirubin, N-terminal pro-B-type natriuretic peptide, and World Health Organization functional class, provided superior risk prediction with area under the receiver operating characteristic curve of 0.86, 0.89, and 0.83 at 1-, 2-, and 3-year intervals, respectively—markedly exceeding COMPERA 2.0. The FW-HP score demonstrated higher net clinical benefit and enabled earlier identification of non-low-risk patients requiring more aggressive management.
Conclusions
The FW-HP model offers improved prognostic accuracy by incorporating hepatic function into PAH risk stratification. It represents a practical tool for early risk identification with potential to guide clinical decision-making in PAH patients.